McGraw-Hill Specialty Board Review Pediatrics, 2nd Edition

Chapter 7. GENERAL PEDIATRICS

CASE 53: A 12-YEAR-OLD WITH EAR DISCHARGE

A 12-year-old child comes to your office during the summer because of left-sided ear pain for 2 days followed by “smelly stuff” coming out of the same ear for 2 days. This has never happened before. The ear pain does not respond to acetaminophen. It hurts his jaw to chew food. He has had no fever and no upper respiratory tract symptoms.

SELECT THE ONE BEST ANSWER

1. Which of the following are possible diagnoses from this history?

(A) otitis externa

(B) otitis media

(C) otitis media with effusion

(D) tuberculous otitis media

(E) A and B

2. On examination, you note thick, yellow-white foulsmelling discharge in the ear canal with underlying erythema. After flushing with water, you see that the tympanic membrane (TM) is intact and appears slightly erythematous. The examination also reveals tenderness when you gently tug on the pinna of the left ear and some small (<1 cm), tender, mobile anterior cervical and preauricular lymph nodes on the left side.

Of the following, which is the least likely pathogen that has caused this problem?

(A) Candida

(B) Staphylococcus aureus

(C) Pseudomonas aeruginosa

(D) group A streptococci

(E) C and D are both unlikely

3. Which of the following is not a risk factor for otitis externa?

(A) otitis media

(B) swimming

(C) cotton swab use

(D) foreign body

(E) all are risk factors

4. Which of the following is/are treatment(s) for otitis externa?

(A) antipyrine with benzocaine

(B) polymyxin B/neomycin sulfate otic drops

(C) acetic acid or boric acid solutions

(D) B and C

(E) all of the above

5. Which of the following is a cause of bloody otorrhea?

(A) acute otitis media (AOM)

(B) Langerhans cell histiocytosis

(C) chronic otitis externa

(D) bullous myringitis

(E) all of the above

6. What is the most important test to perform on clear fluid associated with otorrhea?

(A) bacterial culture

(B) glucose level

(C) pH

(D) potassium hydroxide (KOH) prep

(E) none of the above need to be done

7. Which of the following is a potential cause of clear otorrhea?

(A) bullous myringitis

(B) AOM

(C) fungal otitis externa

(D) perforated TM

(E) A and D

8. A 3-year-old boy is brought to the emergency department by his mother because of ear pain that started the day before and is progressing in intensity. He has had minimal relief from acetaminophen given at home. He has not been ill in the last 2 weeks but was at a birthday party 3 days earlier, where his mother is “sure he caught something from one of the other kids.” On examination he has normal vital signs, profuse white, slightly foul smelling discharge from the right ear, clear nares, normal oropharynx, and no lymphadenopathy. What is the best course of action?

(A) placement of an ear wick

(B) ciprofloxacin/hydrocortisone otic drops

(C) water lavage

(D) topical lidocaine

(E) oral amoxicillin

9. On further otoscopy, you find what you believe to be a small disk-shaped battery, presumably from a toy. You attempt a gentle removal, but it is unsuccessful. You should next

(A) observe for 24 hours

(B) send him to the emergency department for ear, nose, and throat (ENT) consultation

(C) apply topical lidocaine into the ear

(D) start preventive ciprofloxacin/hydrocortisone drops

(E) start oral amoxicillin

10. A 6-year-old is brought to your office for a routine checkup. Her mother says that she “failed” her hearing screen performed at her school 3 months ago and was instructed to get further hearing testing. She reminds you that her daughter has had multiple ear infections in the last year. On examination you note no conjunctival injection, clear nares, normal oropharynx, no lymphadenopathy, but clear effusion behind a mobile TM on the left. What is the most appropriate next step?

(A) reevaluate her effusion in 3 months

(B) automated auditory brainstem response testing

(C) otoacoustic emissions testing

(D) pure tone audiometry testing

(E) tympanometry

11. What is the most common type of hearing loss in children?

(A) sensorineural

(B) conductive

(C) combined

(D) attention deficit disorder

(E) A and B occur with equal frequency

12. What is the most common cause of conductive hearing loss in the United States?

(A) cholesteatoma

(B) middle ear effusion

(C) impacted cerumen

(D) foreign body

(E) perforated TM

13. What is the most common infectious cause of sensorineural hearing loss in the United States?

(A) rubella

(B) syphilis

(C) cytomegalovirus

(D) toxoplasmosis

(E) herpesvirus

Match the following syndromes with the type of hearing loss associated with it.

14. Down (A) conductive

15. Treacher-Collins     (B) sensorineural

16. Pierre-Robin

17. Hunter-Hurler

18. Alport

19. Crouzon

20. Which of the following does not indicate a need for hearing screening if newborn hearing screening was performed?

(A) recognition of developmental delay

(B) meningitis

(C) post-natal exposure to cytomegalovirus (CMV)

(D) otitis media with effusion for 4 months

(E) skull fracture

ANSWERS

1. (E) Both otitis externa and otitis media with a perforated TM can cause purulent discharge and cannot always be differentiated by history alone. The term otitis media with effusion is used to refer to an effusion behind the TM without signs of acute inflammation. The differential diagnosis for ear discharge also must include foreign body in the external auditory canal, chronic suppurative otitis, and acquired cholesteatoma. Tuberculous otitis media should also be considered but is unlikely a process of 2 days.

2. (A) This scenario depicts otitis externa, which presents with the main complaint of ear pain, especially with movement of the pinna or tragus, and thick white discharge from the external auditory canal. P. aeruginosa is the most common isolate when the onset of symptoms follows swimming (80%), but S aureusStreptococcus pyogenes (group A Streptococcus), gram negatives such as Proteus or Klebsiella, and fungi such as Candida or Aspergillus are also possibilities. The last is rare and presents with a more insidious ear discharge that is white and fluffy, sometimes with small black spores visible along the ear canal.

3. (E) Anything that causes trauma to the external canal (cotton swabs, foreign body) or changes the microbial environment (swimming, and thus the designation “swimmer’s ear” for otitis externa) predisposes to bacterial infection of the external canal. Otitis media can cause otitis externa when the TM ruptures and secondarily infects the ear canal.

4. (D) Topical therapy is the first line treatment for uncomplicated otitis externa. Oral antibiotics are reserved for those with chronic otitis externa, concomitant otitis media, or a patient with an underlying immunodeficiency. Topical antibiotics should have activity against P. aeruginosa and S. aureus. Acidifying solutions such as acetic or boric acid lower the pH, thereby making the environment less favorable to the causative organisms. Topical steroids may decrease the associated inflammation and pruritus with otitis externa but are not necessary for a mild to moderate otitis externa.

5. (E) Both otitis media and otitis externa can present with bloody otic discharge. Likewise, rupture of a bulla when bullous myringitis is present may produce bloody discharge. Langerhans cell histiocytosis is a spectrum of disorders, the most systemic of which is Letterer-Siwe disease, often associated with chronic otitis externa.

6. (B) The most important condition to rule out in the presence of clear otorrhea is a leakage of cerebrospinal fluid (CSF). Measurement of glucose (CSF glucose concentration is 45-70 mg/100 mL), protein (<200 mg/mL), and beta2-transferrin levels may be of assistance because very low concentrations of these should be present in inflammatory fluid. In addition, imaging of the area is warranted to rule out congenital or malignant causes of CSF leak.

7. (E) Infections of the inner (media) or outer (externa) ear typically cause purulent discharge or otorrhea. In bullous myringitis, the middle ear is filled with clear fluid, so if the bulla ruptures, clear otorrhea is possible. In addition, a CSF leak through a perforated TM produces clear otorrhea.

8. (C) Because the main diagnostic possibilities include AOM with TM perforation, otitis externa, and the presence of a foreign body, and because the discharge is prohibiting you from making a diagnosis, attempting gentle water lavage is the best next step. The foul-smelling discharge should alert you to the possibility of a foreign body, which should be removed in as timely a manner as possible to prevent secondary infection and puncture of the TM. Sometimes small foreign bodies can be removed simply by gentle warm water lavage.

9. (B) Of the foreign bodies that 3-year-olds place in their ear, batteries are the most urgent. As they degrade, they leak an ototoxic fluid that can be damaging to the ear canal. They must be removed as promptly as possible. Both topical lidocaine and topical antibiotics are often used for treatment. Lidocaine can numb the ear canal for removal of the foreign body, and antibiotics may be useful for infection of the canal associated with the foreign body. Neither should be prescribed until the procedure for battery removal is imminent.

10. (D) Pure tone audiometry is most appropriate for evaluation of hearing in children older than 4-5 years. Although this child may have otitis media with effusion, the larger problem is that even 3 months ago she failed a screening hearing test, suggesting there may be a more chronic problem involved. Automated brainstem response (ABR) and otoacoustic emission (OAE) tests depend less on patient cooperation and are therefore more useful in infants. Tympanometry may support your finding of middle ear effusion but will not assess hearing.

11. (B)

12. (B) A middle ear effusion is the most common cause of conductive hearing loss. In addition to those listed above, other potential causes include congenital anomalies of the external auditory canal, tympanosclerosis, trauma, and masses in the inner ear (histiocytosis, rhabdomyosarcoma).

13. (C) Sensorineural hearing loss (involves inner ear, cochlea or auditory nerve) is more often caused by systemic infections as opposed to the localized middle or outer ear infections that lead to conductive hearing loss. The incidence of congenital CMV in the United States ranges from 0.2% to 2.0% of newborns. Of those, 10% have clinical manifestations at birth. Seventy-five percent of symptomatic infants and 5-15% of asymptomatic infants have sensorineural hearing loss that often may be progressive and present at 4-5 years old.

14. (B) Down syndrome is one of the most common and well-studied trisomies. The incidence is 1 in 700 births and increases in women older than 35 years. Major associations include congenital heart disease, gastrointestinal (GI) disorders (most commonly celiac disease), thyroid disease, leukemia, and mental retardation. Associated anomalies are numerous and include delayed puberty, short stature, sensorineural hearing loss, rapid aging, and sterility in males.

15. (A) Treacher-Collins, or mandibulofacial dysostosis, is an autosomal dominant syndrome characterized by hypoplasia of the facial bones. The clinical findings vary but often include microtia, conductive hearing loss, cleft palate, colobomas, and facial deformities.

16. (A) Pierre-Robin syndrome is an autosomal recessive disorder characterized by severe mandibular hypoplasia, leading to cleft palate, conductive hearing loss, congenital heart disease, and other oral deformities (see Figure 53-1).

art

FIGURE 53-1. A child with Pierre-Robin syndrome. Note the extremely retruded chin in this child, who is being prepared for surgical tongue-lip adhesion. (Reproduced, with permission, from Lalwani AK. Current Diagnosis & Treatment in Otolaryngology: Head & Neck Surgery, 2nd ed. New York: McGraw-Hill; 2008: Fig. 19-7.)


17. (B) Hunter-Hurler syndrome is an autosomal recessive lysosomal disorder characterized by dwarfism, hunchback, coarse facies, mental retardation, sensorineural hearing loss, and cardiac abnormalities. Affected children may appear normal until 1 year of age.

18. (B) Alport syndrome is an X-linked dominant or recessive disease that causes hearing sensorineural loss, hematuria, and renal failure.

19. (A) In general, syndromes that involve craniofacial abnormalities are more likely to display conductive hearing loss. Both Crouzon (1/25,000 births) and Apert (1/100,000 births) syndromes have craniosynostosis as their most prominent clinical feature, associated with multiple other facial dysmorphisms. Crouzon syndrome is an autosomal dominant disorder with variable clinical presentation.

20. (C) Congenital CMV is associated with sensorineural hearing loss, although acquired CMV infection is not.


SUGGESTED READING

American Academy of Pediatrics Subcommittee on Management of Acute Otitis Media. Diagnosis and management of acute otitis media. Pediatrics. 2004;113:1451-1465. http://aappolicy.aappublications.org/cgi/content/full/pediatrics;113/5/1451. Accessed June 2010.

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

Gürtler N. Etiology of syndromic and nonsyndromic sensorineural hearing loss. Otolaryngol Clin North Am. 2002;35(4):891-908.

Hone SW. Medical evaluation of pediatric hearing loss. laboratory, radiographic, and genetic testing. Otolaryngol Clin North Am. 2002;35(4):751-764.

Sander R. Otitis externa: a practical guide to treatment and prevention. Am Fam Physician. 2001;63(5):927-942.

CASE 54: A 2-YEAR-OLD WITH EYE REDNESS

A 30-month-old child is brought to see you by his mother, who has noticed redness of his left eye for the last few weeks. She reports very little discharge and no surrounding swelling or redness. He attends daycare 5 days a week and has had several colds in the last 3 months, most recently 3 weeks prior. He currently has no associated upper respiratory symptoms. His mother does not think that he is bothered by the eye problem.

On examination you find a friendly, interactive toddler. He points to animals on the wallpaper, smiles at his mother, and plays interactively with his toys. He is afebrile. You note unilateral moderate conjunctival injection but no discharge. There are no other findings, including on your fundoscopic examination, during which he is quite fussy.

SELECT THE ONE BEST ANSWER

1. What is your best next action?

(A) begin antibiotic eye drops

(B) begin steroid eye drops

(C) ask them to return for a follow-up visit in a few days if the redness is not resolved

(D) begin anti-allergy eye drops

(E) begin an oral antihistamine

2. The mother misses the follow-up appointment and comes in 3 months later because she now has the sense that the left eye is “growing” faster than the other. She says that the redness is still present and he also has some tears that leak out of that eye. On examination you note the same unilateral moderate conjunctival injection but now also tearing and an enlarged globe. You cannot perform a fundoscopic examination because of lack of cooperation of the child. What is your working diagnosis?

(A) corneal abrasion

(B) viral conjunctivitis

(C) allergic conjunctivitis

(D) bacterial conjunctivitis

(E) glaucoma

3. Which test that can be conducted in the office of a general pediatrician is most indicated during the evaluation of the suspected condition for the patient in this case?

(A) Snellen chart testing

(B) fundoscopic examination

(C) corneal diameter

(D) pupillary reaction to light

(E) B and C

4. Which of the following is the best treatment for a 2-year-old with glaucoma?

(A) beta-blocker

(B) alpha-agonist

(C) trabeculotomy

(D) A and B

(E) none of the above

5. All of the following are associated with glaucoma in childhood except

(A) congenital rubella

(B) congenital syphilis

(C) Sturge-Weber syndrome

(D) Marfan syndrome

(E) neurofibromatosis

6. A 12-year-old who wears contact lenses comes to see you because of several days of right eye redness, pain, and now light sensitivity and blurry vision. She has no other symptoms, does not wear glasses, and recalls no trauma to the eye. What is the most likely diagnosis?

(A) glaucoma

(B) viral conjunctivitis

(C) allergic conjunctivitis

(D) bacterial conjunctivitis

(E) corneal abrasion

7. What is the best way to diagnose this condition?

(A) slit lamp examination

(B) fundoscopic examination

(C) visual acuity testing

(D) fluorescein examination

(E) pupillary reaction to light

8. In addition to discontinuing contact lens use, which additional treatment(s) would be best to treat this condition?

(A) topical antibiotics

(B) oral antibiotics

(C) eye patch

(D) topical steroids

(E) B and D

9. You see a 3-day-old healthy full-term infant in the hospital for her newborn examination and on your fundoscopic examination, you note the absence of a red reflex on one side. Instead you see a white discoloration. If you suspect congenital cataracts, which of the following tests would be part of your workup?

(A) thyroid-stimulating hormone (TSH) and free thyroxine (T4)

(B) rubella antibody titers

(C) galactose-1-phosphate

(D) glucose-6-phosphate dehydrogenase (G6PD)

(E) B and C

10. Approximately what percentage of unilateral congenital cataracts are hereditary?

(A) 5%

(B) 25%

(C) 50%

(D) 75%

(E) 90%

11. Approximately what percentage of bilateral congenital cataracts are hereditary?

(A) 5%

(B) 25%

(C) 50%

(D) 75%

(E) 90%

12. You see a 6-month-old girl in your office for a health prevention visit and on your fundoscopic examination notice a unilateral white discoloration in place of her red reflex. Which of the following is not associated with this finding?

(A) retinoblastoma

(B) Down syndrome

(C) retinopathy of prematurity

(D) incontinentia pigmenti

(E) retinal detachment

13. After referral to an ophthalmologist, you are told that she has a cataract. Which of the following are associated with infantile cataracts?

(A) trisomy of chromosome 13

(B) trisomy of chromosome 18

(C) deficiency of galactose-1-uridyltransferase

(D) chromosome 5 short arm deletion

(E) all of the above

14. You see a 2-week-old in your office for a newborn checkup and on eye examination find that he has a very small amount of iris tissue. You diagnose aniridia. His mother and father say that no one else in the family has anything similar. What other test does this infant most need?

(A) echocardiogram

(B) renal ultrasound

(C) spinal ultrasound

(D) electroencephalogram (EEG)

(E) complete blood count (CBC) with differential

15. What is the mode of inheritance for most children with this disease?

(A) sporadic

(B) autosomal dominant

(C) autosomal recessive

(D) X-linked

(E) not known

16. Which of the following is not associated with a coloboma?

(A) trisomy 13

(B) trisomy 18

(C) VATER

(D) CHARGE

(E) sebaceous nevus

17. The mother of a 4-year-old brings her daughter in after she was hit in the eye with a small toy. She has been very irritable since it happened and says that her eye hurts. On examination you note frank red blood filling the lower third of the anterior chamber. You cannot perform a fundoscopic examination because she is uncooperative. What is her most likely diagnosis?

(A) intraorbital tumor

(B) hyphema

(C) keratitis

(D) coagulopathy

(E) foreign body

18. Which of the following is not a recommended treatment for this disorder?

(A) bed rest

(B) oral steroids

(C) head elevation

(D) surgical drainage

(E) topical steroids

19. What is the most common complication of this disorder?

(A) glaucoma

(B) blood loss

(C) rebleeding

(D) loss of vision

(E) corneal abrasion

ANSWERS

1. (C) Because the diagnosis at this point is unclear, and the diagnostic possibilities include viral infections, bacterial infections, allergic disorders, and other ophthalmologic diseases, the best action is reassurance, with a follow-up visit if it does not resolve spontaneously.

2. (E) Infantile glaucoma (2 months to 3 years of age) is diagnosed mainly by signs and symptoms. The triad of tearing, photophobia, and blepharospasm is typical but only occurs in 30% of children with glaucoma. Findings on examination may include increased corneal diameter (>12 mm), cloudy cornea, conjunctival injection, and ocular enlargement.

3. (E) Fundoscopic examination needs to be performed both to look for orbital nerve cupping (secondary to the increase in pressure) and to rule out intraorbital pathology. You should attempt to measure a corneal diameter in this case with your ophthalmoscope. Children’s corneas reach adult size (12.0 mm) by 2-3 years old. An increase of even 1 mm over the average is considered abnormal and should prompt an immediate referral to an ophthalmologist. A 2-year-old is too young to cooperate with a Snellen chart. The pupillary reaction to light should be maintained in glaucoma. An ophthalmologist may be able to measure intraocular pressure, which will aid in diagnosis.

4. (C) Surgical management is the mainstay of congenital and pediatric glaucoma. Side effects from medical therapies and the difficulty of instilling drops into children’s eyes each day keep these medications from being as useful as they are in adults.

5. (B)

6. (E) These are the classic symptoms of a corneal abrasion: an acute onset with no other symptoms except photophobia and loss of visual acuity.

7. (A) Although the other answers listed are a standard part of the eye examination when considering corneal abrasion, slit lamp examination is considered the gold standard. After installation of fluorescein dye, inspection of the cornea with a blue-filtered light is necessary (available on a slit lamp, ophthalmoscope, or Wood lamp).

8. (A) Prophylactic antibiotics should be applied until the epithelium is completely healed, usually 7-10 days. Several recent studies have suggested no benefit from the use of an eye patch. Steroids are not necessary in this situation. Contact lenses should not be used during the healing process.

9. (E) The most common cause of leukocoria in infants (Figure 54-1) is a congenital cataract. The workup includes rubella titers for congenital infection, galactose-1-phosphate for galactosemia, glucose for hypoglycemia, Venereal Disease Research Laboratory test (VDRL) for syphilis, urine protein for Alport syndrome, urine amino acids for Lowe syndrome, homocysteine for homocystinuria, copper level for Wilson disease, and a karyotype to detect a possible trisomy. Thyroid abnormalities and G6PD are unrelated.

art

FIGURE 54-1. Leukocoria of the left eye caused by retrolental membrane (persistent hyperplastic primary vitreous or persistent fetal vasculature). (Reproduced, with permission, from Hay Jr WW, Levin MJ, Sondheimer JM, et al. Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill; 2009: Fig. 15-1.)


10. (A)

11. (C)

12. (B) Patients with Down syndrome can have epicanthal folds and Brushfield spots but not leukocoria. Cataracts are the most common cause of leukocoria in children, and all the other answers represent the differential diagnosis. About 55% of infants with congenital cataracts have a positive family history.

13. (E) Trisomy 13 (Patau syndrome), trisomy 18 (Edward syndrome), deficiency of galactose-1- uridyltransferase (galactosemia), and cri-du-chat syndrome (deletion of the short arm of chromosome 5) are all associated with cataracts in infancy. Children with these genetic diseases require regular ophthalmologic evaluation. The cataracts associated with galactosemia can be prevented by appropriate diet.

14. (B) Of patients with sporadic aniridia (see next answer), one-fifth will develop Wilms tumor. Thus these patients require renal ultrasound as a screening tool every 3-6 months until they are 5 years old. Also, aniridia leads to glaucoma in 75% of patients.

15. (B) Two-thirds of cases are autosomal dominant; one-third are sporadic. The gene for aniridia has been localized to 11p13. Aniridia is bilateral in 98% (see Figure 54-2).

art

FIGURE 54-2. Bilateral aniridia. Iris remnants present temporally in each eye. (Reproduced, with permission, from Hay Jr WW, Levin MJ, Sondheimer JM, et al. Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill; 2009: Fig. 15-21.)


16. (C) CHARGE syndrome (coloboma, heart disease, atresia choanae, retarded growth and development, genital anomalies, and ear anomalies), the trisomies, and nevus sebaceous are all associated with colobomas. VATER association (vertebral defects, anal atresia, tracheoesophageal fistula, radial dysplasia, renal anomaly) is not (see Figure 54-3).

art

FIGURE 54-3. Iris coloboma located inferiorly. (Reproduced, with permission, from Hay Jr WW, Levin MJ, Sondheimer JM, et al. Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill; 2009: Fig. 15-20.)


17. (B) Blood in the anterior chamber almost always represents a hyphema in children. Tumors and coagulopathies can present similarly but are rare (see Figure 54-4).

art

FIGURE 54-4. Hyphema filling approximately 20% of the anterior chamber. (Reproduced, with permission, from Hay Jr WW, Levin MJ, Sondheimer JM, et al. Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill; 2009: Fig. 15-10.)


18. (D) Treatment for hyphema is supportive with bed rest and head elevation to 30-45 degrees to promote resorption. Steroids, both topical and oral, have been used as have topical mydriatics. Surgical drainage is not a recommended treatment (see Figure 54-5).

art

FIGURE 54-5. Hyphema. This hyphema is just beginning to layer out reflecting its acute nature. (Reproduced, with permission, from Knoop KJ, Stack LB, Storrow AS, et al. Atlas of Emergency Medicine, 3rd ed. New York: McGraw-Hill; 2010:82. Photo contributor: Lawrence B. Stack, MD.)


19. (C) Rebleeding most often occurs 3-5 days after the initial bleed and can lead to other complications. The treatments discussed above are aimed at preventing rebleeding.


SUGGESTED READING

American Academy of Ophthalmology Web site. www.aao.org. Accessed June 2010.

Beck AD. Diagnosis and management of pediatric glaucoma. Ophthalmol Clin North Am. 2001;14(3):501-512.

Fallaha N. Pediatric cataracts. Ophthalmol Clin North Am. 2001;14(3):479-492.

Michael JG. Management of corneal abrasion in children: a randomized clinical trial. Ann Emerg Med. 2002;40(1):67-72.

Wilson SA, Last A. Management of corneal abrasions. Am Fam Physician. 2004;70(1):123-8.

CASE 55: A 5-YEAR-OLD BOY WITH A PAINLESS LIMP

A 5-year-old boy has come to your office today because his mother has noticed him limping on the right for 3-4 days. He does not recall any trauma to the area and has not been ill in the last month. He has had no fevers and denies any pain in the back, hip, knee, or ankle.

On examination he is afebrile. His weight is 18 kg (50%), height is 39.5 inches (<5%), and body mass index (BMI) is 18.3 (>95%). Upon walking across the examination room, you note an obvious limp on the right. Examination of the leg reveals no swelling, erythema, or warmth. You note that he is holding the leg in external rotation and has discomfort and decreased range of motion on internal rotation and abduction. His knee and ankle examinations are normal.

SELECT THE ONE BEST ANSWER

1. What is the most likely diagnosis?

(A) transient synovitis

(B) SCFE (slipped capital femoral epiphysis)

(C) Legg-Calvé-Perthes disease

(D) septic arthritis

(E) growing pains

2. What is the cause of this disorder?

(A) rapid growth

(B) dislocation of the femoral head

(C) bacterial invasion of the femoral head capsule

(D) poor blood supply to the femoral head

(E) immature bone development

3. What is the best test to order first?

(A) CBC with differential

(B) ultrasound

(C) radiograph

(D) CT scan

(E) magnetic resonance imaging (MRI)

4. If a radiograph of the hips is normal, what is the best course of action at this time for this 5-year-old boy?

(A) brace

(B) serial casting

(C) surgical correction

(D) observation

(E) physical therapy

5. A 6-year-old boy is brought in for a painful limp of 2 days’ duration on the left side. He recalls no trauma to the affected leg and has only been getting mild relief with nonsteroidal anti-inflammatory compounds. His recent medical history includes a “viral illness” several weeks ago and a temperature of 100°F (37.7°C) each day. On examination you note that he holds the left hip in a flexed, externally rotated position, and he has pain on internal rotation. There is no erythema or warmth over the area. What is the most likely diagnosis?

(A) transient synovitis or septic arthritis

(B) SCFE

(C) Legg-Calvé-Perthes disease

(D) growing pains

(E) none of the above

6. Which of the following tests would be most helpful to make a diagnosis for the patient in question 5?

(A) radiographs

(B) CBC

(C) erythrocyte sedimentation rate (ESR)

(D) synovial fluid aspirate with gram stain and culture of the fluid obtained

(E) MRI

7. A 14-year-old boy is in your office for a sports physical examination for football and on questioning admits that he has had a limp for about 1 month that he states is a result of a “pulled muscle” that occurred during a workout session. He complains of intermittent pain in his right knee and thigh. He has been afebrile and has been able to play sports, although there is pain with activity.

On examination his BMI is 31 kg/m(>95%). His musculoskeletal examination is significant for his right leg that is held in external rotation. He has pain in the right hip on internal rotation. He is able to bear weight but walks with a limp on the right. What is the most likely diagnosis?

(A) inguinal hernia

(B) SCFE

(C) Legg-Calvé-Perthes disease

(D) septic arthritis

(E) growing pains

8. Which of the following is true of the disorder described in question 7?

(A) it has equal incidence in males and females

(B) it is more common in overweight or obese adolescents

(C) it is more common in rapidly growing adolescents

(D) it is almost always bilateral

(E) B and C

9. What is the most appropriate way to treat the disorder described in question 7?

(A) rest and ice compresses

(B) nonsteroidal anti-inflammatory drugs (NSAIDs)

(C) surgical pinning

(D) serial casting

(E) observation

10. In a sports physical examination performed on a 14-year-old soccer-playing boy, you are told about pain below the knee that worsens without other symptoms when he is playing soccer. On physical examination he has a hard prominence over the tibial tubercle that is tender on palpation. The rest of his examination is normal. What is your diagnosis?

(A) osteosarcoma

(B) Osgood-Schlatter disease

(C) septic arthritis

(D) patellofemoral stress syndrome

(E) stress fracture

11. What other physical examination findings might there be in question 7?

(A) knee effusion

(B) tight quadriceps and/or hamstring muscle(s)

(C) diminished deep tendon reflexes

(D) B and C

(E) none of the above

12. What is the best treatment for the patient’s problem in question 10?

(A) brace

(B) surgical correction

(C) rest and NSAIDs

(D) corticosteroid injection

(E) no treatment is indicated

13. During a sports physical examination of a 10-yearold girl, you note mild lateral asymmetry of her thoracic spine on standing, and moderate to severe asymmetry of her posterior chest on forward bending. She is Tanner 2 breast, Tanner 1 pubic hair. What is the next appropriate action?

(A) observation and reexamination next year

(B) surgical consultation for back brace placement

(C) physical therapy

(D) MRI of the thoracolumbar spine

(E) posteroanterior (PA) radiographs

14. Which of the following is associated with congenital scoliosis?

(A) horseshoe kidney

(B) hip dysplasia

(C) spinal dysraphism

(D) congenital heart disease

(E) all of the above

15. At what age should girls begin to have scoliosis screening examinations?

(A) 2 years

(B) 5 years

(C) 7 years

(D) 12 years

(E) 15 years

16. What is the Cobb angle cutoff whereby a patient with a greater angle is likely to develop pulmonary problems because of their scoliosis?

(A) 10 degrees

(B) 20 degrees

(C) 40 degrees

(D) 60 degrees

(E) 80 degrees

17. Which of the following is true of most cases of kyphosis in adolescents?

(A) it is usually postural

(B) therapy is exercise to strengthen abdominal muscles

(C) kyphosis corrects with hyperextension

(D) patients have normal radiographs

(E) all of the above

18. What distinguishes postural kyphosis from Scheuermann kyphosis in adolescents?

(A) etiology

(B) examination

(C) radiographic findings

(D) treatment

(E) all of the above

ANSWERS

1. (C) This is the classic presentation of Legg-Calvé-Perthes disease, a disorder of the femoral head that causes a painless limp in prepubertal, immature children, 2-12 years old (mean: 7 years old). There is a predilection for boys (4 to 5:1). It is sometimes associated with delayed bone age, disproportionate growth, and short stature, as in this case (see Figure 55-1).

art

FIGURE 55-1. X-ray of Legg Perthes disease with significant deformity of right femoral head. (Reproduced, with permission, from Doherty G. Current Diagnosis & Treatment: Surgery, 13th ed. New York: McGraw-Hill; 2010: Fig. 40-28.)


2. (D) A poor blood supply to the femoral head causes avascular necrosis, whose cause is unknown.

3. (C) Anteroposterior (AP) and lateral (frog) views are the gold standard for diagnosis. Often the radiographs are normal, although several groups have developed diagnostic criteria that depend on the degree of growth cessation of the capital femoral epiphysis, presence of any subchondral fracture, and degree of resorption and reossification.

4. (D) Observation is the rule in children younger than 6 years old (possibly <5 years for girls), unless there is significant deformity of the capital femoral epiphysis on the radiographs. If pain is present, temporary physical therapy and bed rest may be in order.

5. (A) Transient synovitis and septic arthritis can be difficult to differentiate without further testing. Both usually present with a unilateral painful limp and often with fever. Transient synovitis is the most common cause of nontraumatic hip pain in children. It is an inflammatory process that often follows a viral illness, although no specific virus has been implicated. The painful limp can be acute or gradual in onset and is usually unilateral.

6. (D) A positive culture from a joint aspirate or blood are gold standards for diagnosing septic arthritis. Hematologic abnormalities (increased leukocyte count and band count) are similar in both septic joint and transient synovitis. ESR and C-reactive protein (CRP) are more often elevated with septic arthritis but are nonspecific. Serum CRP rises more quickly than ESR in a patient with a septic joint, and it is the most useful test during the early stages. Radiographs may show effusion in either illness, and MRI is not indicated although ultrasonography may be helpful to detect an effusion.

7. (B) This is the classic presentation of SCFE (ie, a painful limp in an obese pubertal boy). The patient may complain of pain in the hip, thigh, or knee but on examination holds the leg in external rotation (see Figure 55-2).

art

FIGURE 55-2. Slipped capital femoral epiphysis. Anteroposterior (AP) and Frog-leg views of a slipped epiphysis. The dotted lines show the normal position of the femoral head. (Reproduced, with permission, from Skinner HB. Current Diagnosis & Treatment in Orthopedics, 4th ed. New York: McGraw-Hill; 2006: Fig. 11-13.)


8. (E) SCFE is more common in obese or rapidly growing pubertal boys. It is bilateral 20-50% of the time. Each side usually presents at different times.

art

FIGURE 55-3. Radiographic images of a young patient with a slipped capital femoral epiphysis, before (A) and after (B) screw fixation. (Reproduced, with permission, from Brunicardi FC, Andersen DK, Billiar TR, et al. Schwartz’s Principles of Surgery, 9th ed. New York: McGraw-Hill; 2010: Fig. 43-57A,B.)


9. (C) Because further slippage of the capital femoral epiphysis will occur without treatment, surgical correction is necessary. The most popular technique is pinning through the femoral head and neck to stabilize the area (see Figure 55-3).

10. (B) Osgood-Schlatter disease is a common cause of knee pain in athletic adolescents. Males are affected more than females. It manifests as pain and sometimes swelling over the tibial tubercle that is made worse by activities that involve pressure with bending of the knee: squatting, jumping, and kneeling (see Figure 55-4).

11. (B)

12. (C) NSAIDs, ice, and rest as needed are the preferred and common treatments of Osgood-Schlatter disease. Steroid injections are not advised, and treatment is almost always nonsurgical. Braces offer minimal support.

13. (E) When noting more than a mildly abnormal Adams test (forward bending to assess for posterior chest asymmetry, the screening tool for scoliosis) in a premenarchal girl, radiographic evaluation is an appropriate first step to assess the degree of severity of the scoliosis. The risk for progression of scoliosis is much greater in premenarchal girls and should be pursued more aggressively. Posterior to anterior radiographs of the thorax should be ordered to determine the Cobb angle of deviation (the angle made by the intersection of 2 lines drawn parallel to the uppermost and lowermost vertebrae involved in the curve) (see Figure 55-5). Incidentally, PA radiographs subject the breast tissue to less radiation than anterior to posterior films.

14. (E) Twenty percent of patients with congenital scoliosis have genitourinary defects; 15% have congenital heart disease. Other skeletal malformations are also common.

art

FIGURE 55-4. Osgood-Schlatter disease. There is pain and swelling over the tibial tubercle. The radiographs would show characteristic fragmentation of the tibial tubercle apophysis, similar to diagram. (Reproduced, with permission, from Skinner HB. Current Diagnosis & Treatment in Orthopedics, 4th ed. New York: McGraw-Hill; 2006: Fig. 11-29.)


art

FIGURE 55-5. Cobb method. Use of Cobb method to measure the scoliotic curve. First lines are drawn along the endplates of the upper and lower vertebrae that are maximally tilted into the concavity of the curve. Next, a perpendicular line is drawn to each of the earlier-drawn lines. The angle of intersection is the Cobb angle.


15. (C) Screening examinations should start at 6-7 years old when girls are premenarchal because the risk of progression is highest at this time. Although in 2004 the USPSTF (United States Preventive Services Task Force) released a statement recommending against routine screening for idiopathic scoliosis in adolescents, the AAP (American Academy of Pediatrics) and AAOS (American Academy of Orthopedic Surgeons) take the opposite view, recommending routine screening for all prepubertal children and adolescents (see Figure 55-6).

art

FIGURE 55-6. Structural scoliosis. Inspection of the flexed spine from behind shows the unequal elevation of the two erector spinae muscle masses. (Reproduced, with permission, from LeBlond RF, DeGowin RL, Brown DD. DeGowin’s Diagnostic Examination, 9th ed. New York: McGraw-Hill; 2009: Fig. 13-13.)


16. (D) An angle of 60 degrees or more is severe scoliosis and often leads to cardiopulmonary sequelae. When the angle is 25 degrees or more, observation is recommended. Between 25 and 45 degrees, close follow-up, physical therapy, and occasionally a brace are sufficient treatment. When 45 degrees or more, surgical treatment is often required.

17. (E)

18. (E) Scheuermann kyphosis is a clinical entity distinct from postural kyphosis, which has a clearly understood cause and can be corrected by the patient during the examination. On radiographs of the back, Scheuermann kyphosis has typical findings of wedging of 3 or more thoracic vertebrae and loss of the anterior height of the affected vertebrae. Patients with this disorder, when mild, require no treatment. But unlike postural kyphosis, many require brace and exercise programs (see Figure 55-7).

art

FIGURE 55-7. Scheuermann kyphosis is characterized by vertebral wedging endplate changes and kyphosis. (Reproduced, with permission, from Skinner HB. Current Diagnosis & Treatmen)t in Orthopedics, 4th ed. New York: McGraw-Hill; 2006: Fig. 11-34.



SUGGESTED READING

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

DeLee JC, Drez D Jr. DeLee & Drez’s Orthopaedic Sports Medicine Principles and Practice. Philadelphia, PA: WB Saunders; 2003:1831-1835.

Kim MK. The limping child. Clin Pediatr Emerg Med. 2002; 3(2):129-137.

Richards BS, Vitale MG. Screening for idiopathic scoliosis in adolescents. J Bone Joint Surg. 2008;90-A(1):195-198.

Sassmannshausen G. Back pain in the young athlete. Clin Sports Med. 2002;21(1):121-132.

CASE 56: A 4-YEAR-OLD WITH EAR PAIN

A 4-year-old boy comes to your office because of ear pain. It began the night before and woke him from sleep several times. Appropriate doses of analgesics for the pain did not help much. His mother reports temperatures as high as 102°F (38.8°C). He had a cold approximately 1 week ago that has been improving the last several days.

On examination he is afebrile. His nares have scant, clear rhinorrhea; the oropharynx is normal. His right TM and ear canal appear normal. His left ear canal is normal, but the TM is bulging outward and is light yellow with erythema around the rim. His lungs are clear to auscultation.

SELECT THE ONE BEST ANSWER

1. Which of the following is the least likely pathogen associated with his ear pain?

(A) Haemophilus influenzae

(B) Streptococcus pneumoniae

(C) influenza virus

(D) Moraxella catarrhalis

(E) chlamydia

2. Which of the following is/are risk factors for this problem?

(A) craniofacial abnormality

(B) exposure to cigarette smoke

(C) formula use

(D) gastroesophageal reflux

(E) all of the above

3. Besides direct observation of the TM, which of the following methods is/are helpful to diagnose otitis media?

(A) tympanogram

(B) radiographs of the skull

(C) pure tone audiometry

(D) pneumatic otoscopy

(E) A and D

4. Which of the following tympanograms would you expect with this patient?

(A) completely flat; high on the y-axis (high volume)

(B) rounded or somewhat flattened peak

(C) sharp peak at 0 daPa

(D) completely flat; low on the y-axis

(E) none of the above

5. All of the following are complications of otitis media except

(A) mastoiditis

(B) pneumonia

(C) brain abscess

(D) meningitis

(E) facial palsy

6. If you choose to put him on an antibiotic, which is your first choice?

(A) azithromycin

(B) amoxicillin-clavulanic acid

(C) ciprofloxacin ear drops

(D) ceftriaxone

(E) amoxicillin

7. If he returned 3 days later because of no change in his symptoms and his examination was exactly the same, what would your next step be?

(A) tympanocentesis

(B) tympanogram

(C) change the oral antibiotic

(D) add oral steroids

(E) add topical ear drops

8. In which of the following scenarios would you choose observation over antibiotic treatment?

(A) a 4-year-old with purulent effusion, limited TM mobility, minimal pain, no fever or other systemic symptoms

(B) a 6-week-old with same findings as above

(C) a 3-year-old with purulent effusion, limited TM mobility, and severe otalgia

(D) all of the above

(E) none of the above

9. If you had begun a patient with unilateral AOM on antibiotic therapy and he returned 1 week later, still on medication, with a new otitis media on the other side, which is your next course of action?

(A) tympanocentesis

(B) tympanogram

(C) change antibiotic

(D) add oral steroids

(E) add topical ear drops

10. His mother calls you worried 2 days later because he has a very high fever, 103°F (39.4°C) axillary, and it appears that his “ear is bulging outward.” What is his likely diagnosis?

(A) otitis media with perforation

(B) subdural abscess

(C) acute mastoiditis

(D) meningitis

(E) foreign body

11. Of the following choices, what is the next best action for the case in question 10?

(A) admission for additional evaluation and intravenous (IV) antibiotics

(B) change to a stronger oral antibiotic

(C) tympanostomy tubes

(D) biopsy of mastoid

(E) course of oral steroids

12. Which of the following is not an indication for tympanocentesis?

(A) otitis media in an immune-compromised patient

(B) AOM and temperature of 102.6°F (39.2°C) in a 3-week-old

(C) mastoiditis

(D) a 2-month history of clear effusion with some conductive hearing loss

(E) failure of second-line treatment of AOM

13. You see a 2-year-old child in your office for a checkup and note on examination a clear effusion with air-fluid levels behind his left TM, which is mobile on pneumatic otoscopy. He has no ear pain, fever, or other symptoms. What is your diagnosis?

(A) otitis media

(B) recurrent otitis media

(C) resistant otitis media

(D) otitis media with effusion

(E) chronic serous otitis media

14. You see the 2-year-old from question 13 back in your office 3 months later with the same physical findings and still no symptoms. His parents note that he seems to say “what?” a lot when they are talking to him. What is your next action?

(A) no testing

(B) audiology referral for hearing test

(C) antibiotics

(D) speech therapy

(E) tympanostomy tubes

15. You see an 8-year-old for ear pain that she has had for 4 days. She had a cold last week, but most of the congestion and cough has resolved. She has had no fevers, no drainage from the ear, and analgesics help the pain minimally. On examination her TM appears retracted, and your in-office tympanogram shows a flattened peak between 200 and 0 daPa. What is her diagnosis?

(A) otitis externa

(B) otitis media

(C) otitis media with effusion

(D) eustachian tube dysfunction

(E) cholesteatoma

16. A 3-year-old girl with bilateral tympanostomy tube placement 6 months ago comes to you with purulent discharge from her left ear for 1 day. She has had a temperature of 102°F (38.8°C) in the last day and is eating less than her usual amount. She has no other symptoms. What is the best management of this patient?

(A) amoxicillin

(B) amoxicillin/clavulanic acid

(C) topical fluoroquinolone drops

(D) topical steroid and fluoroquinolone drops

(E) oral steroids

17. What is the least likely pathogen involved in chronic suppurative otitis?

(A) P aeruginosa

(B) S aureus

(C) Escherichia coli

(D) Proteus mirabilis

(E) S pneumoniae

18. All of the following are possible treatments and managements for chronic suppurative otitis media except

(A) surgical intervention

(B) topical antibiotics

(C) close observation

(D) oral antibiotics

(E) hearing evaluation

ANSWERS

1. (E) Tympanocentesis studies have revealed the etiology of otitis media to be S pneumoniae in 20-35% of cases, H influenzae in 20-30%, Moraxella catarrhalis in 20%, no isolate in 20-30%, and a virus in 17-44%. Chlamydia species are not believed to be common causes of AOM, although in several studies Chlamydia species have been identified by tympanocentesis culture in children with otitis media with effusion.

2. (E) Smoking, reflux, and craniofacial abnormalities, especially cleft lip, put children at increased risk for AOM (children with cleft lip have recurrent otitis media). Multiple studies have shown that infants fed formula have a higher rate of otitis media than breast-fed infants. This difference likely reflects both the protective effect of breast milk and the predisposition to otitis media when bottles of formula milk are “propped up.”

3. (E) Radiographs have no role in diagnosing otitis media, and a hearing test may indeed be abnormal but will not help the practitioner distinguish among AOM, otitis media with effusion, or other forms of conductive hearing loss. Pneumatic otoscopy is the best single sign correlating with post-TM effusion.

4. (B) Tympanograms estimate air pressure and volume of the middle ear space. The normal pressure, or compliance, of a healthy TM fluctuates from −200 to +200 daPa when a tone, sound energy, is applied to it. A graph is produced with air pressure on the x-axis and volume on the y-axis. A tympanogram of a normal middle ear space should have a high peak at atmospheric pressure (0 on the graph) (see Figure 56-1). With middle ear effusion of any cause, there is some mobility of the TM, but it is dampened, producing a rounded curve instead of a sharp peak and often a more negative middle ear pressure.

With eustachian tube dysfunction (most commonly secondary to a cold or sinusitis), there is negative pressure in the middle ear space compared with the nasopharynx, and so middle ear pressures are in the more extreme negative range, −400 to −200 daPa. With a perforated TM, the ear canal and the middle ear are at the same pressure, so that applying a force to the ear canal produces a flat tracing, often with high volume (higher on the curve).

5. (B) Mastoiditis, brain abscess, and meningitis are all possible complications of AOM, either through direct extension or hematogenous spread. Facial nerve palsy occurs because of the proximity of the facial nerve to the middle ear.

art

FIGURE 56-1. Tympanogram of otitis media. The compliance tracing of an ear with otitis media. The line is flat (arrow). (Reproduced, with permission, from Knoop KJ, Stack LB, Storrow AS, et al. Atlas of Emergency Medicine, 3rd ed. New York: McGraw-Hill; 2010:98. Photo contributor: Lawrence B. Stack, MD.)


6. (E) Despite recognized resistance of the major otitis pathogens to penicillin, amoxicillin is still the first choice for treatment. Low-dose amoxicillin (45 mg/kg per day) is no longer preferred and a higher dose (90 mg/kg per day) should be instituted. Amoxicillin-clavulanic acid should be reserved for resistant or recurrent otitis media that has been clinically refractory to high-dose amoxicillin. Cephalosporins, either ceftriaxone intramuscularly (IM) for 3 days or an oral second- or third-generation drug, should be reserved for penicillin-allergic patients, as should azithromycin. Antimicrobial ear drops play no role in the management of AOM, unless there is a perforation in the TM, in which case recent studies have shown similar benefit with antibiotic- and steroid-containing drops versus oral amoxicillin alone.

7. (C) After beginning antibiotic therapy for AOM, resolution of symptoms should begin within 24-48 hours. In this case, it is appropriate to try a different antibiotic because one-fourth of S pneumoniae isolates are resistant to penicillin, and one-fourth to one-third of H influenzae isolates are ampicillin resistant (beta-lactamase positive). All Moraxella species are resistant because of beta-lactamase production. Tympanocentesis may be helpful in guiding a change in therapy but is not the first step. A tympanogram will not provide any extra knowledge. Steroids have no role in the management of otitis media.

8. (A) The most recent guidelines for management of AOM recommend observation for children with uncomplicated otitis media—the absence of high fever or severe pain. Otitis media occurring in a 6-week-old child always requires treatment, followup, and, sometimes, further workup.

9. (C) In this case after asking about compliance, it is also appropriate to try a different antibiotic regimen with a broader antimicrobial spectrum, for the same reasons as in answer 7.

10. (C) This is the clinical picture of acute mastoiditis, which can be a complication of otitis media. Causative organisms are usually S pneumoniaeS pyogenesS aureusPseudomonas species, and H influenzae. Sixty percent of mastoiditis occurs in children younger than 2 years. Clinical findings include fever, point tenderness, and fluctuance over the mastoid, prominence of the pinna, and usually otitis media. Incidentally, all cases of uncomplicated AOM by virtue of proximity show some inflammation of the mastoid air cells. Beyond that, when the infection spreads to the overlying periosteum but not the underlying bone, the diagnosis is acute mastoiditis (see Figure 56-2). Spread to the underlying bone constitutes acute mastoid osteitis, a case that may require medical and surgical management.

art

FIGURE 56-2. Acute mastoiditis. Axial CT image shows an acute fluid collection within the mastoid air cells on the left. (Reproduced, with permission, from Fauci AS, Kasper DL, Braunwald E, et al. Harrison’s Principles of Internal Medicine, 17th ed. New York: McGraw-Hill; 2008: Fig. 31-1.)


11. (A) All cases of suspected mastoiditis should be confirmed by CT of the temporal bone to distinguish acute mastoiditis from mastoid osteitis. For acute mastoiditis, myringotomy and parenteral antibiotics are the next steps.

12. (D) Otitis media in young infants who have systemic symptoms should warrant tympanocentesis as part of the sepsis workup. Two months of clear effusion is otitis media with effusion and does not require tympanocentesis in this time frame.

13. (D) Because of the lack of purulent fluid, outward bulging of the TM, and the presence of a freely mobile TM, all other possible answers are excluded.

14. (B) Otitis media with effusion is considered “persistent” when it has been present for 3 months or more. By that time, if no resolution or improvement is seen, a hearing test is the next appropriate step, especially if subjective hearing loss is still evident and not resolving. A conductive hearing loss would likely be found.

15. (D) Eustachian tube dysfunction is common in the weeks following an upper respiratory infection (URI). The only finding on examination is a retracted TM, and symptomatically the child may complain of frequent popping, pain, or pressure. Various over-the-counter (OTC) remedies have been employed to treat this, including pseudoephedrine and nasal saline sprays.

16. (A) Recent studies have proven that topical otic drops that are a combination of antibiotics and steroids are equivalent if not superior in eradicating otitis media with tympanostomy tubes. However, use of quinolones in this fashion encourages antimicrobial resistance and should be therefore avoided.

17. (A) P aeruginosa in the absence of a perforated eardrum and otorrhea is a rare cause of otitis media.

18. (C) Surgery may be required if there is an anatomic defect that is acting as a continued bacterial medium (ie, cholesteatoma). Observation is not an acceptable treatment plan because hearing loss often ensues with chronic otitis media and worsens with time.


SUGGESTED READING

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

Hendley JO. Otitis media. N Engl J Med. 2002;347:1169-1174.

CASE 57: A 14-YEAR-OLD BOY WITH A SORE THROAT AND ENLARGED SPLEEN

A 14-year-old boy comes into your office complaining of sore throat of 1 week’s duration. It is getting worse by the day, and this morning he felt as if he could not swallow his breakfast, both because of the pain on swallowing and because it felt as if something was “blocking it down there.” He has been feeling hot and cold but has not measured his temperature. He is feeling fatigued, but the rest of his review of systems is negative. He has had multiple episodes of “tonsillitis” this year.

On examination, he is cooperative but appears quite tired. His temperature is 101.5°F (38.6°C) orally; the other vital signs are normal. His conjunctivae are clear, TMs are normal, and oropharynx is quite erythematous with enlarged erythematous tonsils covered by diffuse white exudates bilaterally. No petechiae are seen. His lymphatic examination reveals anterior and posterior cervical lymphadenopathy, all 1-2.5 cm in diameter, mobile, and moderately tender. His lungs are clear. The cardiovascular examination is normal, and the abdominal examination reveals a palpable spleen tip 4 cm under the rib cage. There is no rash.

SELECT THE ONE BEST ANSWER

1. What is his most likely diagnosis?

(A) streptococcal pharyngitis

(B) viral upper respiratory tract infection

(C) adenoviral pharyngotonsillitis

(D) mononucleosis

(E) peritonsillar abscess

2. What kind of microorganism most likely caused this infection?

(A) gram-positive coccus

(B) enterovirus

(C) herpesvirus

(D) echovirus

(E) Coxsackie virus

3. Which of the following is not a clinical association of this patient’s likely diagnosis?

(A) Guillain-Barré syndrome

(B) maculopapular dermatitis

(C) pharyngoconjunctival fever

(D) transient thrombocytopenic purpura

(E) bilateral orchitis

4. During what time of year is this illness most common?

(A) spring

(B) summer

(C) fall

(D) winter

(E) no seasonal association

5. What is the incubation period of this illness?

(A) 1-4 days

(B) 4-7 days

(C) 7-14 days

(D) 14-28 days

(E) 30-50 days

6. What is the best specimen to diagnose this disease?

(A) sputum

(B) oropharyngeal secretions

(C) blood

(D) urine

(E) stool

7. If this is infectious mononucleosis caused by Epstein-Barr virus (EBV), what is the simplest way to support the diagnosis?

(A) EBV titers

(B) CBC

(C) throat culture

(D) monospot test

(E) nasal swab

8. If you found that this patient had EBV mononucleosis, what is your treatment?

(A) no treatment

(B) oral steroids

(C) amoxicillin

(D) ceftriaxone

(E) azithromycin

9. Under which age does the monospot test to detect heterophil antibody decrease significantly in sensitivity?

(A) 1 year

(B) 2 years

(C) 4 years

(D) 8 years

(E) 10 years

10. Another child who was thought to have mononucleosis had the following test results:

Immunoglobulin (Ig)G to viral capsid antigen

(VCA): positive

IgM to VCA: negative

Early antigen (EA): negative

Antibody against EBNA (EBV nuclear antigen): positive

What can be deduced from these results?

(A) no present or past EBV infection

(B) definite past infection

(C) acutely infected

(D) either past infection or recent infection

(E) none of the above

11. How long does infection with EBV last?

(A) 1 month

(B) 6 months

(C) 1 year

(D) 2 years

(E) lifelong

12. The most reasonable indication for treating mononucleosis with steroids is

(A) severe splenomegaly

(B) severe pain from sore throat

(C) impending airway obstruction

(D) hemolytic anemia

(E) myocarditis

13. A patient with mononucleosis and splenomegaly should be advised to avoid contact sports for at least how long?

(A) 1-2 weeks

(B) 3 weeks-6 months

(C) 1 year

(D) life

(E) none of the above; no recommendation to avoid contact sports

14. The 14-year-old boy from the vignette calls you 10 days after you saw him in your office and diagnosed him with mononucleosis. He now has a fever of 103.2°F (39.5°C), cannot swallow, and feels that he cannot open his mouth easily. You see him hours later in the office and you note that his voice is quite muffled. On examination, his temperature is 100.6°F (38.1°C) (he took acetaminophen 2 hours prior). The blood pressure (BP) is 110/65. He cannot open his mouth wide, but on minimal opening you note white exudates covering both tonsils, marked tonsillar enlargement, right greater than left, and a visible bulge at the superior aspect of the right tonsil. What is his diagnosis?

(A) secondary strep pharyngitis

(B) peritonsillar abscess

(C) retropharyngeal abscess

(D) Mycoplasma pneumoniae cellulitis

(E) Corynebacterium diphtheriae infection

15. Which of the following should be part of his management?

(A) IV clindamycin

(B) surgical incision and drainage

(C) IV ampicillin

(D) tonsillectomy

(E) A, B, and D

16. Which of the following is not a relative indication for tonsillectomy?

(A) more than 8 episodes of viral pharyngitis in 1 year

(B) more than 6 episodes of group A streptococcal pharyngitis in 1 year

(C) acute airway obstruction concurrent with peritonsillar abscess

(D) peritonsillar abscess following recurrent tonsillitis

(E) obstructive sleep apnea

17. A patient’s mother is worried about the complications of tonsillectomy. Which of the following is not a known complication?

(A) velopharyngeal insufficiency

(B) hemorrhage

(C) sinusitis

(D) eustachian tube injury

(E) dehydration

18. Adenoidectomy is potentially beneficial in all of the following except

(A) chronic recurrent sinusitis

(B) hyponasal speech

(C) hypernasal speech

(D) submucous cleft palate

(E) excessive snoring

ANSWERS

1. (D) The most likely diagnosis for this boy is EBV mononucleosis because of his age, presenting symptoms, and examination. Mononucleosis can present with a wide range of severity, but most patients have fever that averages 6 days’ duration, exudative pharyngitis, lymphadenopathy that is most often anterior and posterior cervical but can be generalized, fatigue, and hepatosplenomegaly in 50% of patients.

2. (C) Seventy-nine percent of infectious mononucleosis syndromes are caused by EBV, a herpesvirus. The other 21% are caused by CMV. Sore throat with exudative tonsillitis is more common with EBV than CMV mononucleosis; fever is a predominant feature of CMV mononucleosis. With CMV, the heterophil agglutinin test (monospot test) is negative.

3. (C) Pharyngoconjunctival fever is associated with adenovirus.

4. (E)

5. (E)

6. (C) Despite the physical findings of mononucleosis, often centered in the oropharynx, blood is the most useful specimen because serologic testing will produce a more timely diagnosis. Viral culture is possible by pharyngeal swab but is performed in only a few laboratories and may be positive for many years after the acute infection.

7. (D) The monospot test is relatively sensitive and specific in this age group. A positive test may be considered diagnostic. Even though a single EBV serology determination might give you helpful information, repeat testing for convalescent serology may be necessary, and thus this serologic approach may not be simplest. A CBC with an absolute lymphocytosis in the second week of illness is supportive of the diagnosis but not specific. A throat cultures for viral isolation does not necessarily indicate acute infection in any disease where viral persistence is common. EBV viral isolation is not routinely available.

8. (A) Because mononucleosis has a viral etiology (EBV), no antibacterial therapy is warranted. A high percentage of patients with mononucleosis who are exposed to ampicillin or amoxicillin develop a nonallergic morbilliform rash. Penicillin is an acceptable alternative if antibacterial therapy is indicated. About 10-30% of patients with mononucleosis are concomitantly group A strep positive; the treatment of the latter can complicate the diagnosis of the former. Steroids should only be considered in severe cases of mononucleosis.

9. (C) Children younger than 4 years of age may have EBV infectious mononucleosis but lack heterophil antibodies. The reason is not known. If infectious mononucleosis is suspected in these young children, EBV serology should be performed.

10. (D) The VCA IgM and IgG occur in high titers early after infection begins. The IgM response lasts for 3-5 months, and IgG can be detected indefinitely. The EA likewise is positive in the first 2 weeks of infection and peaks at about 3 months. It is important to note that if tested too early, the EA and the IgM VCA may not yet be positive. EBNA antibodies are not present until several weeks to months after onset of infection. So although a positive EBNA result excludes acute infection, it cannot differentiate between recent or past infection.

11. (E) EBV, like other herpesviruses, establishes lifelong latent infection. As opposed to the other herpesviruses, reinfection with EBV is usually asymptomatic.

12. (C) There are few objective data that document the effectiveness of corticosteroids in infectious mononucleosis. Many clinicians use them in the management of impending or actual airway obstructions. They should otherwise be avoided as unnecessary therapy that may even increase the rate of occurrence of certain complications like encephalitis. Severe pain may be treated adequately with other medications.

13. (B) Splenic enlargement is common in mononucleosis, but splenic rupture is rare (0.1-0.2%). Rupture most commonly occurs within the first 3 weeks following diagnosis. Athletes are sometimes counseled to wait up to 6 months before resuming contact sports. At the time of activity resumption, neither the spleen nor the liver should be enlarged.

14. (B) Although a peritonsillar abscess is most often a complication of group A streptococcal pharyngitis, EBV infectious mononucleosis and group A streptococci are commonly present simultaneously. This patient has fever, trismus, and an obvious tonsillar bulge, the triad of which strongly suggests the diagnosis.

15. (E) In addition to standard treatment of peritonsillar abscess, parenteral antibiotics, and surgical drainage, this patient is a candidate for tonsillectomy because of his history of recurrent tonsillitis. This would be performed after the acute episode resolves. Ampicillin is an unwise choice because of the known increase in the incidence of rash when patients with mononucleosis are given ampicillin or derivatives. This rash does not represent ampicillin allergy. It presents as a maculopapular pruritic confluent rash mainly over the trunk and includes the palms and soles (see Figure 57-1).

art

FIGURE 57-1. When needle aspiration is used to manage peritonsillar abscess, aspiration should be attempted at each of these three anatomic locations. (Reproduced, with permission, from Stone CK, Humphries RL. Current Diagnosis & Treatment: Emergency Medicine, 6th ed. New York: McGraw-Hill, 2008. Fig. 30-9.)


16. (A) Viral pharyngitis is never an indication for tonsillectomy. Repeated bacterial infection of the tonsils may be, although the number of infections per year that warrant tonsillectomy is controversial. Some recommend it when there are more than 3 episodes per year, others wait for more than 6 per year, and yet others await more than 3 episodes a year for 3 consecutive years. Other reasons for performing tonsillectomy are obstructive sleep apnea or, rarely, acute airway obstruction.

17. (C) In addition to those listed, complications of tonsillectomy include risks of anesthesia, infection, and atlantoaxial subluxation. Velopharyngeal insufficiency may occur because tonsillectomy increases the space in the nasopharynx leading to inability of the nasopharyngeal sphincter to close properly, causing hypernasal speech and regurgitation. The incidence of these complications is increased in children with craniofacial abnormalities or orofacial problems, such as those with Down, Treacher-Collins, and Pierre-Robin syndromes.

18. (C) Hyponasal speech is an indication for adenoidectomy, not hypernasal. Children with cleft palate and submucous cleft palate should have preventive adenoidectomy performed. Excessive snoring is a likely sign of chronic adenoid hypertrophy. A patient with excessive snoring should undergo further evaluation to determine whether adenoidectomy may be beneficial.


SUGGESTED READING

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

Discolo CM. Infectious indications for tonsillectomy. Pediatr Clin North Am. 2003;50(2):445-458.

Gross CW, Harrison SE. Tonsils and adenoids. Pediatr Rev. 2000;21(3):75-78.

Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009.

CASE 58: A 5-YEAR-OLD WITH NOSEBLEEDS

A 5-year-old boy is brought for evaluation during winter by his mother because of recurrent nosebleeds. They happen 1-3 times a week, last 10 minutes, and are difficult to stop. This has been going on for about 6 weeks.

SELECT THE ONE BEST ANSWER

1. All of the following would prompt you to explore the etiology of this boy’s nosebleeds except

(A) duration of 10 minutes or longer

(B) family history of bleeding disorder

(C) age younger than 2 years

(D) low hematocrit on screening

(E) gum bleeding

2. The differential diagnosis for recurrent epistaxis in an adolescent includes which of the following malignancies?

(A) squamous cell carcinoma

(B) adenocarcinoma

(C) nasopharyngeal angiofibroma

(D) malignant melanoma

(E) nasal lymphoma

3. An 8-year-old girl with a history of seasonal allergic rhinitis comes in during autumn because she is “always congested.” Her regular OTC allergy medicine is not working. She feels the congestion is getting worse each week and is worse on the left side.

On examination you note allergic shiners, an allergic salute sign, mild conjunctival injection bilaterally, and a 0.75-cm soft gray bulge in the left nare, adjacent to the septum. There is some clear rhinorrhea, no foul smell, and no bleeding. What is her most likely diagnosis?

(A) foreign body

(B) nasal polyp

(C) sinusitis

(D) juvenile nasopharyngeal angiofibroma

(E) hematoma

4. With which chronic disease(s) is/are the problem(s) in question 3 associated?

(A) chronic sinusitis

(B) asthma

(C) cystic fibrosis

(D) aspirin sensitivity

(E) all of the above

5. What are possible treatments used for nasal polyps?

(A) intranasal steroids

(B) antihistamines and decongestants

(C) surgical removal

(D) oral steroids

(E) A, C, and D

6. A 6-year-old boy comes to your office in August with a “runny nose.” His mother states that it has been going on for approximately 3 weeks and is not improving. He has been afebrile but has a nonproductive cough each night. What finding is not consistent with a diagnosis of allergic rhinitis?

(A) cobblestoning of the posterior pharynx

(B) dark circles under both eyes

(C) edematous, erythematous nasal turbinates

(D) a horizontal indentation across the bridge of the nose

(E) nighttime cough

7. Which of the following is not a comorbid disorder of allergic rhinitis?

(A) speech impairment

(B) eczema

(C) failure to thrive

(D) otitis externa

(E) pharyngitis

8. Which of the following is false regarding allergic rhinitis?

(A) prevalence peaks in adolescents

(B) its incidence is increasing

(C) higher socioeconomic status is a risk factor

(D) serum IgE more than 100 IU/mL in childhood is a risk factor

(E) early introduction to solid foods in infancy is a risk factor

9. Which treatment for allergic rhinitis most successfully diminishes nasal symptoms?

(A) leukotriene inhibitor

(B) antihistamine

(C) allergen injection immunotherapy

(D) intranasal steroid

(E) decongestant

10. You see a new patient in your office who is 1 week old. He is there for his preventive 1-week visit. His medical history is significant for a cleft lip and palate, and no other known conditions. All of the following are true regarding cleft lip and palate except

(A) cleft lip is more common in males

(B) cleft lip alone is more common than cleft palate alone

(C) cleft lip and palate can be inherited in an autosomal dominant pattern

(D) sensorineural hearing loss is common with cleft palate

(E) no exceptions; all of the above are true

11. Which of the following statements is false?

(A) the most immediate problem facing a child with an uncomplicated cleft lip is feeding difficulty

(B) surgical closure of a cleft lip is usually performed in the first 3 months of life

(C) surgical closure of a cleft palate is usually performed by age 1 year

(D) children with uncomplicated cleft lip do not require subspecialist involvement with the exception of the surgical repair

(E) children with cleft palate often require prophylactic tympanostomy tubes

12. Which of the following syndromes are associated with cleft lip and/or palate?

(A) histiocytosis

(B) thrombocytopenia and absent radius (TAR) syndrome

(C) cardiac abnormality, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia (CATCH-22) syndrome

(D) Waardenburg syndrome

(E) all of the above

13. You see a 1-month-old child in your office for a preventive visit. You note on examination that the infant is at the 80th percentile for height and weight and developing well. She is breast-fed and her mother reports that it is going “well.” You note, on examination, that she has a very short lingual frenulum. What will you recommend to her mother be done?

(A) nothing

(B) surgical correction

(C) cease breastfeeding

(D) early speech therapy

(E) oral motor physical therapy

14. The mother from question 13 asks you about when her 1-month-old might start to develop teeth. What can you tell her?

(A) most often by 2 months

(B) most often by 3 months

(C) most often by 4 months

(D) most often between 4 and 5 months

(E) most often between 6 and 12 months

15. At a child’s 2-year routine visit, the mother asks how she can help prevent dental cavities in her child. You recommend

(A) using fluoride-containing toothpaste

(B) flossing regularly and a low-sugar diet

(C) discontinuing sleeping with the bottle

(D) B and C

(E) A, B, and C

16. Which of the following is the main etiology of dental caries?

(A) Streptococcus pyogenes

(B) Streptococcus viridans

(C) Streptococcus mutans

(D) Streptococcus pneumoniae

(E) Enterococcus species

ANSWERS

1. (A) The duration of epistaxis that should prompt further workup is 30 minutes. Most nosebleeds can be stopped sooner, if proper technique is used: blowing the nose first, tilting the head forward, followed by firm and constant pressure just below the bridge of the nose for a minimum of 5 minutes. Children younger than 2 years should be evaluated further because epistaxis in this age group is rare, and because most epistaxis in children is secondary to self-inflicted trauma (vigorous nose rubbing or picking).

2. (C) Nasopharyngeal angiofibromas occur in children and 30- to 40-year-old adults but have a peak incidence in adolescent boys. They present with nasal obstruction and recurrent epistaxis. A CT of the relevant structures can define the extent of the tumor. Surgical excision is the usual treatment.

3. (B) Nasal polyps are benign pedunculated tumors of the nasal mucosa that are associated with multiple chronic diseases. Presentation includes nasal obstruction, hyponasal speech, rhinorrhea, and mouth breathing (see Figure 58-1).

art

FIGURE 58-1. Endoscopic view of nasal polyp obstructing the posterior nasal airway. A small residual air passage (A) is seen between the polyp and the nasal septum. (Reproduced, with permission, from Brunicardi FC, Andersen DK, Billiar TR, et al. Schwartz’s Principles of Surgery, 9th ed. New York: McGraw-Hill; 2010: Fig. 18-3.)


4. (E) Approximately 30% of children with cystic fibrosis (CF) develop nasal polyps. In any child younger than 12 years old in whom nasal polyps are diagnosed, regardless of other risk factors, sweat testing for CF should be performed. All the other diseases listed are associated with nasal polyps, including the Samter triad of aspirin sensitivity, nasal polyps, and asthma.

5. (E) Besides surgical removal, steroids are the most successful treatment for nasal polyps. Intranasal steroids should be tried first, and systemic steroids are an alternative depending on the severity of disease. Neither antihistamines nor decongestants will shrink a polyp, although they may provide symptomatic relief from associated illnesses, such as allergic rhinitis.

6. (C) The nasal turbinates in a patient with allergic rhinitis are usually pale or blue and edematous; erythematous turbinates are more commonly seen with infectious causes of nasal discharge. The “allergic salute” (rubbing the nose upward) causes a longterm crease across the bridge, which is indicative of chronic allergic rhinitis. “Allergic shiners” are dark circles under the eyes, caused by chronic lid edema and resolution, in addition to subcutaneous venodilation. Postnasal drip eventually leads to a cobblestoned appearance in the posterior pharynx and often a nighttime cough.

7. (D) Allergic rhinitis is associated with otitis media with effusion, not otitis externa.

8. (A) The prevalence of allergic rhinitis peaks in late childhood. The incidence of allergic rhinitis is increasing, especially in affluent societies. Risk factors include high socioeconomic status, high IgE in childhood, family history of atopy, heavy indoor allergen concentrations, mothers who smoke near their children in the first year of life, and early introduction of solids in infancy. This last risk factor has led to the recommendation by the American Academy of Pediatrics that solid food not be started until infants are 6 months old.

9. (D) Intranasal steroids have the greatest effect against nasal congestion with allergic rhinitis. First-generation antihistamines are sedating and have limited use for daytime control of symptoms in children. Second-generation antihistamines, leukotriene inhibitors, and avoidance of allergens will provide good control of symptoms, whereas decongestants are short term and will give no lasting benefit. Allergen injections are considered the last resort for symptom control in children.

10. (D) Cleft lip with or without associated cleft palate is present in 1 in 750 white births; cleft palate alone is present in 1 in 2500 white births. It is more common in males and can be inherited dominantly in Van der Woude syndrome. It has the highest incidence ethnically in Asians and the lowest in African Americans. Conductive hearing loss is commonly associated with cleft palate but not sensorineural hearing loss (see Figure 58-2).

11. (D) From birth, a team of services should be alerted and develop a management plan for the child with the family. These would likely include, but are not limited to, a pediatrician, an otolaryngologist, a pediatric dentist, a speech therapist, a geneticist, a social worker, and/or a psychologist.

12. (C) CATCH-22 syndrome includes cardiac, abnormalities, abnormal facies, thymic hypoplasia, cleft palate, and hypocalcemia. TAR syndrome is thrombocytopenia and absent radii. Waardenburg syndrome, or partial albinism, includes acrocephaly, facial dysmorphism (not cleft lip or palate), ocular problems, deafness, and abnormal dentition.

13. (A) Ankyloglossia, or “tongue tie,” is an abnormally short lingual frenulum (see Figure 58-3). In most children, the frenulum grows with them and does not cause any difficulties. In some, feeding trouble ensues in early infancy because of restricted extension of the tongue. These children should see a general pediatric surgeon early on and be counseled regarding surgical correction, especially if they are failing to gain weight appropriately. In others, speech difficulties arise later on. Most speechdelayed children still do not require surgery but will benefit from speech therapy.

art

FIGURE 58-2. Anatomy of cleft palate. (A) Normal anatomy; note the sling formed by the two sides of the levator palatini muscle. (B) Cleft palate; the levator muscle is oriented longitudinally, somewhat parallel with the cleft margin. (Reproduced, with permission, from Lalwani AK. Current Diagnosis & Treatment in Otolaryngology: Head & Neck Surgery, 2nd ed. New York: McGraw-Hill; 2008: Fig. 19-7.)


art

FIGURE 58-3. Ankyloglossia (tongue-tie). (Reproduced, with permission, from Hay Jr WW, Levin MJ, Sondheimer JM, et al. Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill; 2009: Fig. 16-4.)


14. (E) Most often children start eruption of their primary teeth at 6 months and continue until 22 months. Primary eruption can be as early as 4 months but more often occurs after 6 months.

15. (E) Dental caries are common chronic diseases of childhood (see Figure 58-4). Caused by bacterial overgrowth and stagnation in the oral cavity, caries can be prevented by several means, including all of the given choices. Fluoride-containing toothpaste is generally recommended starting at 2 years of age, at a time when children can begin to spit out the paste after use (the danger is an overdose of fluoride if too much is used at an early age and the child swallows it all). Sucrose is the most likely carbohydrate involved in caries formation and is contained in sweetened drinks that children often start drinking at a young age.

art

FIGURE 58-4. Dental caries. (Reproduced, with permission, from South-Paul JE, Matheny SC, Lewis EL. Current Diagnosis & Treatment in Family Medicine, 2nd ed. New York: McGraw-Hill; 2008: Fig. 45-5.)


16. (C)


SUGGESTED READING

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

Kirschner RE. Cleft lip and palate. Otolaryngol Clin North Am. 2000;33(6):1191-1215.

Lack G. Pediatric allergic rhinitis and comorbid disorders. J Allergy Clin Immunol. 2001;108:S9-S15.

Martof A. Consultation with the specialist: dental care. Pediatr Rev. 2001;22(1):13-15.

Scadding G. Optimal management of nasal congestion caused by allergic rhinitis in children: safety and efficacy of medical treatments. Pediatric Drugs. 2008;10(3):151-162.

CASE 59: A 4-YEAR-OLD GIRL WITH “PINK EYE”

A 4-year-old girl comes in with eye swelling of 3 days’ duration. She is a new patient and has brought her old records with her. Her mother confirms that she has had no immunizations, based on parental refusal. The girl is rubbing at her eye a lot, and it is red and puffy. Her mother says that she thinks her daughter “has pink eye” and saw a doctor at another office several weeks ago for it. At that time she was congested and had a cough, and she was sent home with no medication.

On examination you note that she is afebrile and slightly fussy. She has 2 cm of erythema and edema surrounding the right eye and it is very tender. She is able to follow your finger in all directions and has no proptosis. Her nose, throat, and lung examinations are normal.

SELECT THE ONE BEST ANSWER

1. What is her most likely diagnosis?

(A) Pott puffy tumor

(B) acute bacterial sinusitis

(C) preseptal cellulitis

(D) orbital cellulitis

(E) type 1 hypersensitivity reaction

2. In this case, what is the most helpful information to make the diagnosis?

(A) physical examination and history

(B) CBC with differential leukocyte count

(C) blood culture

(D) culture of eye discharge

(E) CT of orbits

3. How would you treat this child?

(A) oral cephalexin

(B) oral dicloxacillin

(C) oral prednisone

(D) IV ceftriaxone and clindamycin

(E) IV ceftriaxone alone

4. A 6-year-old boy is brought to your office by his mother because he has had some increasing swelling around his eye for the last several days that seems worse today. This morning he was having difficulty “looking around” with that eye and is complaining that it hurts. On examination he is afebrile, has right-sided lid edema and erythema, proptosis, and lateral gaze is decreased. What is his diagnosis?

(A) Pott puffy tumor

(B) acute bacterial sinusitis

(C) preseptal cellulitis

(D) orbital cellulitis

(E) type 1 hypersensitivity reaction

5. What is the most common associated finding of this disease?

(A) paranasal sinusitis

(B) frontal sinusitis

(C) conjunctivitis

(D) bacteremia with hematogenous spread

(E) trauma to the orbit

6. Which organism(s) is (are) the least likely to cause this disease in young children?

(A) group A streptococci

(B) S pneumoniae (C) H influenzae type b

(D) S aureus (E) A and D

7. Which of the following tests is the gold standard in diagnosing an orbital abscess?

(A) testing of visual acuity and extraocular movements

(B) ultrasound

(C) radiographs of the sinus and orbits

(D) CT of orbit

(E) MRI of orbit

8. For how long should he receive antibiotics for his disease?

(A) 7 days

(B) 10 days

(C) 14 days

(D) 21 days

(E) 30 days

9. Preseptal cellulitis occurs in an older age group than orbital cellulitis

(A) true

(B) false

10. Sinusitis more commonly is an associated finding of orbital cellulitis than preseptal cellulitis

(A) true

(B) false

11. A 12-year-old boy is seen in your office with redness in both eyes of 3 days’ duration. They do not itch. He also has had nasal congestion and a cough for 5 days. On examination he is afebrile. He has audible congestion and scant clear rhinorrhea. His turbinates are red and swollen. His eyes are bilaterally mildly injected and watery, with no discharge currently. There is no surrounding erythema or edema, and his fundoscopic examination is normal bilaterally. His lungs are clear bilaterally. What is the most likely diagnosis?

(A) bacterial conjunctivitis

(B) viral conjunctivitis

(C) allergic conjunctivitis

(D) herpes simplex conjunctivitis

(E) dacryocystitis

12. The child from question 11 returns several days later with the same eye complaints but has developed a sore throat. He has been intermittently febrile (101.2°F [38.4°C] orally), and his eye examination has only changed in that the conjunctival erythema has become more intense. His posterior pharynx is slightly red, there are no exudates or petechiae, and the oral cavity is normal. He has a few tender cervical and preauricular lymph nodes, all smaller than 2 cm. What is the most likely pathogen causing this disease?

(A) EBV

(B) Coxsackie virus

(C) herpes simplex virus

(D) adenovirus

(E) influenza virus

13. You see a 13-year-old boy in your office because of redness in one eye that is persisting for 3 days. It does not itch, but he is concerned because this morning there was thick, yellow discharge from the eye. Upon waking, his eyelids were stuck together. He had a “cold” last week but that has resolved. On examination, he is afebrile, and his left eye is severely injected, with dried and crusted yellowish discharge. His extraocular movements are intact. There is no preseptal erythema or edema. The rest of his examination is normal. What is his diagnosis?

(A) bacterial conjunctivitis

(B) viral conjunctivitis

(C) allergic conjunctivitis

(D) herpes simplex conjunctivitis

(E) dacryocystitis

14. What will you prescribe for the boy from question 13 to treat this condition?

(A) steroid and antibiotic eye drops

(B) oral amoxicillin

(C) oral doxycycline

(D) antibiotic eye drops

(E) warm compress only

15. You see a 6-month-old in the office because of “stuff coming out of his eye.” His father describes a yellowish watery discharge that makes his left eye stuck closed in the morning. This has been going on for 3 days. On examination, the baby smiles while you examine him. You note unilateral watery discharge and some scant yellow discharge. The conjunctiva is white. The infant fixes and follows on your hand 180 degrees. What is his diagnosis?

(A) dacryocystitis

(B) nasolacrimal duct obstruction

(C) viral conjunctivitis

(D) Chlamydia trachomatis conjunctivitis

(E) Neisseria gonorrhoeae conjunctivitis

16. You see a 5-year-old boy in your office because of a “lump” in his eye. It has been there for several weeks, is not red, does not hurt, but he does play with it frequently, which bothers his parents. On examination, he is afebrile and cooperative. You note a firm, nontender nodule in the lower left lid. There is no overlying erythema. His extraocular movements and gross visual acuity are intact. Which gland does this involve?

(A) gland of Zeis

(B) lacrimal gland

(C) gland of Moll

(D) meibomian gland

(E) none of the above

17. You get a call at night from a mother worried about her 8-year-old daughter, who has a “red lump” in her eye. It developed 2 days ago and looks worse to her mother today. She describes a red, tender lump about 0.5 cm across that is just at the edge of the lower eyelid. From her description, what is the most likely diagnosis?

(A) internal hordeolum

(B) external hordeolum

(C) chalazion

(D) dacryocystitis

(E) blepharitis

18. Which of the following is not associated with childhood ptosis?

(A) botulism

(B) myasthenia gravis

(C) trisomy 13

(D) amblyopia

(E) muscular dystrophy

ANSWERS

1. (C) This is most likely preseptal cellulitis based on the physical findings (see Figure 59-1). Preseptal (or periorbital) is differentiated from orbital cellulitis anatomically, symptomatically, and by physical findings (see below for further discussion).

art

FIGURE 59-1. Preseptal Cellulitis. Left preseptal cellulitis with edema and erythema of the eyelids. Note that the conjunctiva is clear and not injected. (Reproduced, with permission, from Knoop KJ, Stack LB, Storrow AS, et al. Atlas of Emergency Medicine, 3rd ed. New York: McGraw-Hill; 2010:431. Photo contributor: Kevin J. Knoop, MD, MS.)


2. (A) In cases where orbital cellulitis is not suspected, history and physical examination should lead to the correct diagnosis (see Figure 59-2). A CBC may be somewhat helpful if a leukocytosis or “left shift” is present, but such a change is nonspecific. A culture of the eye discharge, if present, takes too long to be helpful in immediate decision making. A CT is not indicated for preseptal cellulitis unless orbital involvement cannot be excluded.

art

FIGURE 59-2. Left orbital cellulitis with decreased range of motion secondary to edema. Note the injected conjunctiva. (Reproduced, with permission, from Knoop KJ, Stack LB, Storrow AS, et al. Atlas of Emergency Medicine, 3rd ed. New York: McGraw-Hill; 2010:431. Photo contributor: Javier A. Gonzalez del Rey, MD.)


3. (D) Because of the lack of vaccination receipt in this child, H influenzae type b should probably still be considered even though it is unlikely. Once a major pathogen causing preseptal cellulitis, it is now quite rare because of the vaccination program against it in the United States. The patient is also 4 years old, on the tail end of what was the H influenzae type b agespecific curve. H influenzae type b causes a rapidly progressive preseptal cellulitis and therefore it is appropriate to treat presumptively with parenteral antibiotics that cover it as well as the other common causes, S aureusS pneumoniae, and S pyogenes. Clindamycin is now an agent of choice to cover these gram-positive species in the many areas where methicillin-resistant S aureus (MRSA) isolates are prevalent.

4. (D) Orbital cellulitis is differentiated from preseptal cellulitis based on anatomy (anterior vs. posterior to the orbital septum), symptomatically (pain on eye motion, decreased vision), and by physical findings (proptosis, decreased extraocular movement).

5. (A) Paranasal sinusitis is the most common associated finding with orbital cellulitis in children. Direct extension of infection or venous spread can also occur from the lid, conjunctiva, globe, lacrimal gland, or nasolacrimal sac. Frontal sinusitis also can cause contiguous spread of infection although less frequently than paranasal sinusitis. In addition, because of the age at which sinuses develop anatomically, paranasal sinusitis occurs in a younger group (frontal sinuses begin to develop at approximately 7 years old).

6. (C) Although all of the above are implicated, the incidence of H influenzae infection has decreased dramatically since widespread vaccination began in 1985. Members of the species other than type b may still found in older children with frontal sinusitis extending to the orbit with resultant cellulitis.

7. (D) Although physical examination can often lead to the differentiation between orbital and preseptal cellulitis, a CT of the orbits makes the definitive diagnosis. Orbital CT changes may be minimal, however, in the absence of abscess. CT is superior to MRI in its ability to distinguish the orbital soft tissues from the surrounding bone. Some experts believe that known orbital abscesses can be followed with ultrasound to limit radiation exposure, but this modality is not useful as an initial evaluation.

8. (D) He should receive parenteral antibiotics for 7-10 days, depending on the rapidity of improvement. Oral antibiotics may then be used to complete a 21-day course.

9. (B) Preseptal cellulitis occurs most often in children younger than 5 years old, whereas orbital cellulitis occurs in older children and, less commonly, adults.

10. (A) Whereas orbital cellulitis is commonly an associated finding of sinusitis, preseptal cellulitis is commonly an associated finding of trauma, upper respiratory tract infections, or dacryocystitis.

11. (B) Viral conjunctivitis is common during an upper respiratory tract infection. It is more commonly bilateral, watery, and mild to moderate conjunctival injection.

12. (D) This is pharyngoconjunctival fever, caused by adenovirus. The diagnostic triad is fever, conjunctivitis, and pharyngitis.

13. (A) As opposed to viral conjunctivitis, bacterial conjunctivitis is more often unilateral. There is severe injection and thick and sometimes copious yellow discharge. It often follows a viral upper respiratory tract infection.

14. (D) Topical antibiotic drops are the best way to treat bacterial conjunctivitis. The fluoroquinolones are heavily promoted for the therapy of conjunctivitis but select for resistance frequently. Sulfacetamide drops are almost always effective and still constitute relatively inexpensive front-line therapy.

15. (B) This is typical nasolacrimal duct (NLD) obstruction that can occur in the newborn period and can be relapsing. It most often resolves by 9-12 months of age. Symptoms include watering and yellow discharge. In the newborn period the conjunctivae are sometimes mildly erythematous, making the differentiation between NLD obstruction and chlamydia or gonococcal infection difficult. In most cases, treatment for NLD obstruction is not provided unless it persists past 9-12 months, at which time therapeutic probing of the NLD can be performed by an ophthalmologist.

16. (D) This is a chalazion, a granulomatous inflammation in the meibomian gland (see Figure 59-3). These can be chronic, and inflammatory signs are typically absent. Treatment most often is not warranted, although surgical excision may be needed in instances of a large growth obstructing vision.

art

FIGURE 59-3. Chalazion. This nontender lowerlid chalazion was seen with lid eversion. (Reproduced, with permission, from Knoop KJ, Stack LB, Storrow AS, et al. Atlas of Emergency Medicine, 3rd ed. New York: McGraw-Hill; 2010:37. Photo contributor: James Dahle, MD.)


17. (B) This is most likely an external hordeolum, or stye. Internal hordeolums are on the inner lid and appear to be bulging from underneath. Dacryocystitis, or infection of the lacrimal gland, is located inferiorly and medially to the medial tear duct. Blepharitis is inflammation of the lid margins and typically has erythema and crusting or scaling.

18. (C) Amblyopia may occur as a result of ptosis, either from the lid covering the visual axis or astigmatism secondary to the weight of the lid on the globe.


SUGGESTED READING

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

Greenberg MF. The red eye in childhood. Pediatr Clin North Am. 2003;50(1):105-124.

Mawn LA. Preseptal and orbital cellulitis. Ophthalmol Clin North Am. 2000;13(4):633-641.

CASE 60: A 9-MONTH-OLD IN A CAR ACCIDENT

You see a 9-month-old infant in the emergency department with his mother, who was rear-ended in a car accident 1 hour prior. At the time of the accident, he was in his infant carrier car seat, in the backseat, facing forward. Since that time he has been acting his usual self, smiling and laughing, playful, and hungry.

On examination, he weighs 22 pounds, is alert, and interactive. His neurologic examination is normal, as is the rest of his examination.

SELECT THE ONE BEST ANSWER

1. What can you tell his mother about his car seat?

(A) it should be forward-facing in the front seat

(B) it should be rear-facing in the front seat

(C) it should be forward-facing in the backseat

(D) it should be rear-facing in the backseat

(E) he should be in a booster seat

2. She is wondering if the airbags in her 4-door car are a danger to him while he is restrained in his car seat. What will you tell her?

(A) airbags in the front seat should be disabled

(B) she should place her infant in the center of the backseat to avoid side airbags

(C) she should place her infant in the front to avoid backseat airbags

(D) she should place her infant next to the side airbag for protection

(E) none of the above are true

3. Approximately what percentage of caregivers have installed their infant car seat correctly?

(A) 10%

(B) 30%

(C) 50%

(D) 70%

(E) 90%

4. Which of the following is the safest choice in car seats for a 13-month-old 23-pound infant?

(A) rear-facing convertible car seat

(B) rear-facing infant car seat

(C) front-facing convertible car seat

(D) belt-positioning booster seat

(E) shield booster seat

5. At what age is it acceptable for children to ride in the front seat with a passenger belt?

(A) 2 years

(B) 5 years

(C) 8 years

(D) 10 years

(E) 13 years

6. In the event of an accidental ingestion, what is the best first recommended action that parents should take?

(A) go the emergency department

(B) administer syrup of ipecac

(C) administer activated charcoal

(D) call the poison control center

(E) perform the Heimlich maneuver

7. At what age does the American Academy of Pediatrics generally recommend starting the regular use of sunscreen?

(A) 1 week

(B) 1 month

(C) 2 months

(D) 6 months

(E) 1 year

8. A parent calls you from their vacation on the East Coast. This morning, their 7-year-old son found a tick on his leg that is still embedded. They are wondering how to get the tick off.

(A) put rubbing alcohol on it

(B) smother it with a cloth

(C) try to burn it off with a match

(D) pull it out with tweezers

(E) break the body off at the skin level

9. At what temperature should household hot water heaters be set at or below to prevent accidental burns?

(A) 90°F

(B) 100°F

(C) 110°F

(D) 120°F

(E) 130°F

10. What is the leading cause of death for all childhood age groups (1-21 years old) in the United States?

(A) suicide

(B) unintentional injuries

(C) malignancies

(D) homicide

(E) heart disease

11. What is the leading cause of death among children younger than 1 year of age?

(A) congenital anomalies

(B) sudden infant death syndrome (SIDS)

(C) unintentional injury

(D) malignancies

(E) sepsis

12. Who is the most likely person to be involved in a motor vehicle accident (MVA)?

(A) 17-year-old boy

(B) 17-year-old girl

(C) 25-year-old man

(D) 25-year-old woman

(E) none of the above

13. By what percentage do helmets reduce the risk of bicycle-related traumatic brain injuries?

(A) 10%

(B) 25%

(C) 50%

(D) 75%

(E) 95%

14. Which of the following would you not include in counseling a family on fire safety and prevention?

(A) replace their fire detector batteries every 6 months

(B) place portable heaters 1 foot or more from bedding

(C) store matches and other fire-starting materials out of reach

(D) inform them that smoke is more dangerous than the fire

(E) discontinue smoking

15. Of these common myths regarding water safety and drowning, which is true?

(A) children don’t need life vests after they have learned to swim

(B) fences that surround pools won’t prevent a child from drowning

(C) drowning children make lots of noise like splashing and will be heard easily

(D) continuous supervision at pools is not necessary when life vests are used

(E) children can drown in several inches of water

16. Which of the following will you counsel parents on regarding safety with lawn mowers?

(A) children should be at least 10 years old to use sit-down riding mowers

(B) use eye protection during mowing

(C) children must be at least 7 years old to use push-type mowers

(D) children older than 10 years can ride as passengers on sit-down mowers

(E) A, B, and C

17. You get a call from a mother who is taking her child to the northern Midwest during late summer and is worried about her 1-year-old and 3-year-old getting West Nile virus. Which of the following statements would you use to counsel her on this disease?

(A) approximately 20% of those infected develop mild disease

(B) the incubation period is 3-14 days

(C) 1 in 150 who are infected develop severe neurologic disease

(D) encephalitis and meningitis are the most common complications of disease

(E) all of the above

18. Which of the following statements regarding the prevention of West Nile Virus is(are) true?

(A) DEET (N,N-diethyl-3-methylbenzamide) should not be used for infants under 2 months of age

(B) the maximum acceptable concentration of DEET for children is 30%

(C) DEET should be applied before clothing is put on

(D) A and B

(E) A, B, and C

ANSWERS

1. (D) The AAP and the National Highway Traffic Safety Administration (NHTSA) recommend that infants remain in their rear-facing car seat in the backseat until they are 1 year of age and heavier than 20-22 pounds, and then for as long as possible thereafter. This is so the deceleration forces are distributed over the infant’s entire trunk during a crash. It is also to protect their incompletely ossified vertebrae and corresponding weak connecting ligaments from injury. For infants who reach 20 pounds before 1 year of age it is acceptable to change from an infant carrier seat (which typically only can fit infants <20-22 pounds) to a “convertible”-type car seat, which has the ability to face forward or backward as the age of the child dictates.

2. (B) In vehicles that have a front passenger airbag, infants must be in the backseat because riding in the front seat puts them in a direct path of the front airbag and can lead to fatality. When children start riding in the front seat (see answer to question 5), or for cars in which there is no backseat, several rules should be put into effect. Older children should be seated as far back as possible from the airbag. If a child is in a car seat of any type, the passenger airbag should be disabled. If an infant is rear facing in the front seat (in a 2-seater car, for example), the passenger air bag should be disabled. For the backseat, some cars come with side airbags. Because they are difficult to disable, it is best to center infant car seats in the back to avoid the side airbags.

3. (B) A study conducted for the NHTSA in the mid-1990s found that approximately 72% of child restraints were being used incorrectly. A recently completed NHTSA-sponsored study updates that research. Interestingly, rear-facing seats (infant and convertible) had the highest rate of misuse, approximately 84% for both. The NHTSA passed new regulations that require all passenger vehicles made after 2002 to be equipped with the LATCH system (Lower Anchors and Tethers for Children), which significantly reduces the complexity of installing a car seat.

4. (A) In general, as stated above, infants should be 20-22 pounds and 1 year of age to face forward. Convertible car seats are made to face forward or backward and vary in their weight limits. It is safest to keep infants facing backward for as long as possible, up to the rear-facing maximum weight limit indicated on the convertible car seat. Almost every convertible car seat on the market today has a rearfacing weight limit of 30 pounds. Booster seats are made for toddlers, and almost all have a minimum weight limit of 30-40 pounds. Information about any age, length, or weight parameters for a specific car seat should be affixed to the car seat.

5. (E) The NHTSA suggests all children 12 years and younger should ride in the back seat. Pediatricians should check the law in their state.

6. (D) Parents should be advised at preventive visits to always have the poison control center number nearby and to call there in case of accidental ingestions. The number is 800-222-1222. In 2003, the AAP reversed their position on the use of syrup of ipecac in the home. Because multiple studies did not show a difference in outcome in children on whom ipecac was used, and because activated charcoal is considered to be superior, most physicians prefer to treat ingestions in an emergency department setting with charcoal.

7. (D) In 1999 the AAP changed its policy from recommending against the use of sunscreen in infants under 6 months to not using it for that age group unless adequate clothing and/or shade are not available. In those instances, applying small amounts to areas like the face and hands is acceptable. Sunscreen should always be SPF 15 or greater and be reapplied after water exposure.

8. (D) Ticks must be removed as completely as possible. Grasping the part of the tick sticking out of the skin as close to the surface as possible and slowly pulling directly out most likely will aid in getting both the body and the head, which is buried underneath the skin. Old methods considered useful, including smothering with petroleum jelly or other substances, burning the tick off, or using rubbing alcohol, have not been proven to be as effective.

9. (D) At 160°F (71.1°C), a full-thickness scald burn can occur in less than 1 second. At 120°F (48.8°C), many minutes of exposure are required for the same injury.

10. (B) For the past 5 years, unintentional injuries (including MVAs, poisoning, drowning, falls, fire/burns, firearm injuries) have ranked as the number-one cause of mortality for all children older than 1 year of age.

11. (A) For children younger than 1 year of age, congenital anomalies are the number-one cause of death.

12. (A) Male teen drivers are the most likely group to be involved in a MVA. In addition, teens are 5-10 times more likely to be involved in a fatal MVA while driving at night versus driving during the day.

13. (D)

14. (B) Portable heaters should always be placed 3 feet or more from any material that could be flammable (bedding, curtains, etc). Children younger than 5 years and the elderly are at highest risk of injury from residential fires. Smoke detectors decrease the risk of fatality in a house fire by approximately 60-70%, but among smoke detectors that failed to alarm in a fire, 59% had been disconnected because of false or battery-related alarms. Smoke inhalation kills more people than direct fire-related injuries.

15. (E) Infants and children can drown in as little as 2-3 inches of water in a bucket or bathtub. Children often do not splash or make noise when they are drowning. Reports from survivors have suggested that they either are too young to maneuver out of the water, or if older, simply cannot think of what to do at that moment, and quietly slip underneath the water. Full 5-feet surround fences are the only proven way to prevent pool drownings because children can too easily climb over chain-link and picket fences. Children should wear properly fitting life vests or use personal flotation devices for a minimum of 2 years after they learn to swim, at which time constant supervision is still necessary.

16. (B) The AAP recommends that children younger than 12 years of age not use push-type mowers, that children younger than 16 years not use sit-down mowers, and that no children ride on a sit-down mowers as a passenger. Sturdy shoes and eye protection should always be worn during mowing.

17. (E)

18. (D) DEET-containing products are the most effective insect repellents. They should never be applied under clothing and cannot be combined with other insect repellants. The concentration of DEET ranges from 5% to 30%, at which point the maximum efficacy is obtained. The percentage directly correlates with the number of hours of protection given (eg, 10% gives about 2 hours of protection; 24% gives about 5 hours).


SUGGESTED READING

American Academy of Pediatrics Web site. www.aap.org. Accessed June 2010.

Injury Prevention & Control: Data and Statistics. Ten leading causes of death and injury. CDC Web site. http://www.cdc.gov/ injury/wisqars/LeadingCauses.html. Accessed June 2010.

McKay MP, Curtis L. Children in cars: keeping them safe at every age. Contemp Pediatr. 2003;20(9):65-78, 81.

Johnston B, Rivara F. Injury control: new challenges. Pediatr Rev. 2003;24:111-117.

National Highway Traffic Safety Administration Web site. www.nhtsa.gov. Accessed June 2010.

CASE 61: AN 8-YEAR-OLD WITH CONGESTION

An 8-year-old boy comes to your office with his mother because of congestion for 5 days. In addition he has sneezing, rhinorrhea, and a mild cough. He has no fever and his symptoms are worse at night. He has no previous medical conditions.

On examination, he has mild bilateral conjunctival injection, clear rhinorrhea, red swollen turbinates, and a mildly erythematous pharynx.

SELECT THE ONE BEST ANSWER

1. What is his most likely diagnosis at this time?

(A) allergic rhinitis

(B) rhinovirus

(C) influenza

(D) sinusitis

(E) Coxsackie virus

2. How many typical upper respiratory tract infections per year does a healthy child have?

(A) 2-3

(B) 3-4

(C) 4-5

(D) 5-6

(E) more than 6

3. He returns 14 days later with continued congestion. He no longer has a “runny” nose or sneezing, although his cough is still present and worse at night. His mother is concerned because he has never had a “cold” for this long before. On examination, he is afebrile, has mild bilateral preseptal edema, thick purulent nasal discharge, swollen turbinates, and a mildly erythematous pharynx. What is his most likely diagnosis at this time?

(A) allergic rhinitis

(B) rhinovirus

(C) influenza

(D) sinusitis

(E) immotile cilia syndrome

4. Which of the following is not implicated in causing acute bacterial sinusitis?

(A) Streptococcus pneumoniae

(B) Moraxella catarrhalis

(C) Haemophilus influenzae

(D) adenovirus

(E) Coxsackie virus

5. At what time would you diagnose his condition as “chronic”?

(A) 4 weeks

(B) 6 weeks

(C) 8 weeks

(D) 12 weeks

(E) 16 weeks

6. By what age could this child develop frontal sinusitis?

(A) 1 year

(B) 2 years

(C) 3 years

(D) 4 years

(E) 5 years

7. A 9-year-old girl comes in to see you with fever. She had a “runny” nose several days ago, which has now changed to a thick yellow discharge. Three days ago she developed a fever of 103°F (39.4°C), which has remained until today. She also has a mild headache and a nighttime cough but no other symptoms. What is her diagnosis?

(A) allergic rhinitis

(B) rhinovirus

(C) influenza

(D) sinusitis

(E) immotile cilia syndrome

8. What should your next course of action be with the patient in question 7?

(A) plain films of the sinuses

(B) intranasal steroid spray

(C) antibiotics

(D) CT of sinuses

(E) prescription antihistamine not associated with drowsiness

9. If you chose to use antibiotics, which would be your first choice?

(A) amoxicillin at 45 mg/kg per day

(B) amoxicillin at 90 mg/kg per day

(C) amoxicillin (600 mg/5 mL)–clavulanate (42.9 mg/5 mL) at 90 mg/kg per day of amoxicillin

(D) azithromycin

(E) trimethoprim-sulfamethoxazole

10. How long will you treat her?

(A) 7 days

(B) 10-14 days

(C) 3 weeks

(D) 1 month

(E) 2 months

11. After beginning antibiotics, when would you expect clinical improvement (decrease in symptoms and increase in well-being)?

(A) 1 day

(B) 3 days

(C) 7 days

(D) 10 days

(E) 14 days

12. Which of the following might prompt you to change your medical therapy?

(A) day-care attendance

(B) recent treatment with amoxicillin

(C) presence of frontal or sphenoidal sinusitis

(D) otitis media

(E) B and C

13. She returns 10 days later still on your original prescription with eye pain. On examination she has left-sided preseptal edema, erythema, proptosis, and a fever of 103°F (39.4°C). What is your next step?

(A) change to a different oral antibiotic

(B) start IV

(C) ophthalmology referral

(D) sinus aspiration

(E) oral prednisone

14. You see a 12-year-old boy who has had a month and a half of congestion and cough, day and night. He does not have fever but has a mild sore throat every day. On examination you confirm nasal congestion. His pharynx is mildly erythematous. He has normal-appearing tonsils, no lymphadenopathy, and no wheezing. What is his most likely diagnosis?

(A) allergic rhinitis

(B) rhinovirus

(C) influenza

(D) sinusitis

(E) immotile cilia syndrome

15. Which of the following can predispose an individual to develop sinusitis?

(A) viral upper respiratory tract syndrome

(B) dental infections

(C) allergic rhinitis

(D) cystic fibrosis

(E) all of the above

16. What should your next course of action be with the patient just described?

(A) radiographs of the sinuses

(B) intranasal steroid spray

(C) oral antibiotics

(D) CT of sinuses

(E) prescription antihistamine not associated with drowsiness

17. How long should this patient receive antibiotics if clinical improvement is apparent?

(A) 7 days

(B) 10-14 days

(C) 21 days

(D) 30 days

(E) 45 days

18. Under which condition would a maxillary sinus aspiration not be indicated?

(A) failure to respond to multiple courses of antibiotics

(B) severe facial pain

(C) an orbital or intracranial complication

(D) symptoms for 30 days

(E) evaluation of sinusitis in an immunocompromised patient

ANSWERS

1. (B) The vignette is a typical story for the common cold, most often caused by rhinovirus.

2. (E) Most healthy children have 6-8 upper respiratory tract infections per year.

3. (D) The definition of acute bacterial sinusitis is persistent symptoms lasting more than 10 and less than 30 days. Nasal discharge of any quality, cough that is present daytime and nighttime (although usually worse at night), foul breath, and facial pain and/or headache are the most common presenting symptoms. On examination, preseptal swelling, facial pain or tenderness over the sinuses, and findings of an upper respiratory tract infection are often present. Immotile cilia syndrome, or primary ciliary dyskinesia, is an inherited disease affecting the respiratory cilia. Most patients with this disease have chronic respiratory illnesses (including URIs, chronic sinusitis, chronic otitis media) throughout their childhood.

4. (E)

5. (D) Subacute sinusitis is defined as persistent symptoms from 30 to 90 days. Chronic sinusitis lasts longer than 90 days.

6. (E) The frontal sinuses develop from the anterior ethmoid cells and move into their position by 5-6 years old. The maxillary and ethmoid sinuses form in utero and are present at birth.

7. (D) This is another, less common, presentation of acute bacterial sinusitis in children: high fever (>102°F [38.8°C]) and purulent nasal discharge for at least 3-4 consecutive days. Viral upper respiratory tract infections can also present with fever and thick nasal discharge, although the timing is different. With a viral URI, the quality of the nasal discharge may change several times throughout the course of the illness, from clear to thick and back to clear. Fever is usually present at the outset and resolves after several days; other constitutional symptoms are usually present.

8. (C) When children meet the criteria for acute bacterial sinusitis, whether in the case above or the case from question 3, antibiotic therapy is warranted. See the next question and answer for a discussion on antibiotic choices. Although amoxicillin is still considered the first choice, penicillin resistance to S pneumoniae should be considered. The rates of resistance vary by location. Routine radiographic examination of uncomplicated acute bacterial sinusitis is not helpful. A CT scan of a patient with suspected sinuitis may be helpful, but changes consistent with mild sinusitis are often found during uncomplicated upper respiratory tract infections (ie, mucosal changes within the sinuses that are indistinguishable from acute bacterial sinusitis). Recent studies investigating the use of intranasal steroids in patients with acute bacterial sinusitis are promising but not conclusive.

9. (B) Despite penicillin resistance of S pneumoniae (approximately half of the isolates obtained by sinus aspiration are intermediately resistant to penicillin and a small proportion of these will be fully resistant), amoxicillin is still the first choice for uncomplicated acute bacterial sinusitis, although at the higher dose of 90 mg/kg per day. Both azithromycin and trimethoprim-sulfamethoxazole have less efficacy against the primary agents of sinusitis and should not be used as first line therapy.

10. (B) Most patients should be treated for 10-14 days. Some require longer courses if symptoms persist. An alternative treatment approach is to treat for 7 days beyond resolution of symptoms. Treatment beyond a few weeks is not recommended or supported by clinical studies.

11. (B) Most patients who are treated with an appropriate antibiotic respond promptly, within 48-72 hours.

12. (E) Recent amoxicillin therapy and the presence of frontal or sphenoid sinusitis are situations in which an alternative to amoxicillin may be appropriate. Symptomatology for more than 30 days is another indicator. Reasons include the higher likelihood of a resistant organism and the need for higher drug levels than oral amoxicillin can provide. Clinically important frontal or sphenoid sinusitis may require parenteral therapy.

13. (B) This is likely to be an orbital process, either cellulitis or abscess, which are both complications of acute bacterial sinusitis. They require parenteral antibiotics and inpatient observation. Other complications of sinusitis include subperiosteal intraorbital abscess, sinus-associated osteomyelitis (frontal bone osteomyelitis, also known as Pott puffy tumor), epidural abscess, meningitis, and brain abscess.

14. (D) This is consistent with subacute bacterial sinusitis, with protracted respiratory symptoms (congestion and cough are most common). At this point it is important to consider other possibilities with your examination such as CF (poor growth, clubbing, barrel chest, nasal polyps, respiratory findings), allergic rhinitis (dark circles under the eyes, horizontal crease across the nose, Morgan-Dennie lines characterized by skin folds under the lower eyelid), adenoidal hypertrophy, or immunodeficiency.

15. (E) All of the other illnesses or situations listed predispose patients to sinusitis, acute and bacterial. Immune disorders, immotile cilia syndrome, facial trauma, choanal atresia, and foreign bodies have also been implicated.

16. (C) Because the microbial etiologies of subacute sinusitis are somewhat different from that of acute bacterial sinusitis, the treatment also is different, although antibiotics remain the first step in therapy. In addition to S pneumoniae, H influenzaeS aureus, and M catarrhalis, anaerobes may play a role. Therapy should be directed against all of these. One reasonable choice is amoxicillin-clavulanate except in areas where MRSA isolates are prevalent. If oral antibiotic therapy is not successful, surgical drainage and parenteral antibiotics are often required.

17. (C)

18. (D) Maxillary sinus aspiration can be performed by an otolaryngologist on an outpatient basis, but this should be reserved for the other situations previously listed. Sinus symptoms for 30 days are still considered subacute, and an initial trial of oral antibiotics is appropriate.


SUGGESTED READING

Brook I. Microbiology and antimicrobial management of sinusitis. Otolaryngol Clin North Am. 2004;37(2):253-266.

Clinical practice guideline: management of sinusitis. American Academy of Pediatrics Web site. http://aappolicy.aappublications.org/. Accessed June 2010.

Nash D, Wald E. Sinusitis. Pediatr Rev. 2001;22(4):111-116.

CASE 62: AN 8-YEAR-OLD GIRL WITH SORE THROAT

An 8-year-old girl comes to your office during winter complaining of worsening throat pain for 2 days, tactile temperatures at home, and abdominal pain. She has not vomited, has no diarrhea, and she has noticed no rashes. She was barely able to eat food this morning because of the pain. No one else is ill at home, but several of her friends at school have the “same thing.”

On examination you note a temperature of 101.5°F (38.6°C), clear nares, white conjunctivae, oropharynx with palatal petechiae, a swollen and erythematous uvula, enlarged erythematous tonsils, and no tonsillar exudates. She has tender anterior cervical lymphadenopathy, left greater than right, all smaller than 1 cm in diameter. Her lungs are clear bilaterally; she has mild periumbilical tenderness, no hepatosplenomegaly, and no rash.

SELECT THE ONE BEST ANSWER

1. What is the most likely diagnosis?

(A) viral pharyngitis

(B) mononucleosis

(C) group A streptococcal pharyngitis

(D) Coxsackie virus

(E) group B streptococcal pharyngitis

2. What is the most appropriate next step?

(A) in-office monospot test

(B) CBC and EBV titers

(C) rapid latex test for group A streptococcus

(D) reassurance

(E) start antibiotics

3. Which of these is not a complication of this illness?

(A) retropharyngeal abscess

(B) splenic rupture

(C) glomerulonephritis

(D) peritonsillar abscess

(E) rheumatic fever

4. If this patient’s rapid strep test was positive, what is your first choice of medication?

(A) cephalexin

(B) penicillin

(C) azithromycin

(D) ceftriaxone

(E) prednisone

5. If she came back after taking the prescribed antibiotic for 10 days, still had a sore throat, and similar findings on examination, what would you do next?

(A) reculture for group A streptococcus

(B) monospot test

(C) diagnose her as a group A streptococcus carrier

(D) admit for IV antibiotics

(E) A and B

6. If her repeat throat culture grew group A streptococcus, what medication would you start her on?

(A) acetaminophen or ibuprofen

(B) penicillin

(C) azithromycin

(D) ceftriaxone

(E) prednisone

7. Her mother wants to know if her 6-year-old brother, who is at home, should also be tested for group A streptococcus. He has no symptoms and is otherwise healthy. Which is the next appropriate step for you to take for her brother?

(A) rapid strep test

(B) throat culture for strep

(C) no tests

(D) prophylactic oral antibiotics

(E) prophylactic IM ceftriaxone

8. One week later, her 2-year-old brother developed a fever of 101°F (38.3°C) axillary, serous rhinitis, and mild cough. On examination, he was found to have a temperature of 100.5°F (38°C) rectally, a normal respiratory rate, nasal congestion, and yellowish discharge in both nares. The oropharynx was moist and normal appearing, and the lungs were clear. What test(s), if any, would you do?

(A) rapid strep test

(B) monospot

(C) rapid flu test

(D) CBC with differential leukocyte count

(E) no tests

9. A 6-month-old male infant presents to your office with a diaper rash for 8 days. His parents have tried OTC diaper creams but none seem to be helping. He is eating normally, has regular soft bowel movements, and has had no other symptoms except irritability for 2 days when he has a bowel movement and when they are cleaning him afterward. On examination you note bright erythema perianally extending about 3 cm outward but no other finding. His genitals appear normal, and the rest of his examination is normal. What is the likely etiology of this rash?

(A) Candida albicans

(B) group A streptococci

(C) group B streptococci

(D) seborrheic dermatitis

(E) pinworm infestation

10. What is the most worrisome complication of the disease from question 9?

(A) rheumatic fever

(B) abscess

(C) impetigo

(D) glomerulonephritis

(E) severe diarrhea

11. What lab test can you do to confirm your suspicion raised in question 9?

(A) KOH prep

(B) skin culture

(C) throat culture

(D) urinalysis

(E) stool studies

12. What do you recommend to the parents for treatment in question 9?

(A) watchful waiting

(B) continue OTC diaper creams only

(C) topical antibiotics

(D) topical steroids

(E) penicillin orally

13. A 15-year-old girl whom you saw in your office last week and diagnosed with group A streptococcus pharyngitis has returned. She stopped taking her antibiotics after 3 days because her sore throat resolved. It has now returned, and she feels more pain than before. She reports a fever of 104°F (40°C) last night and this morning is having difficulty opening her mouth to eat and severe pain on swallowing water. On examination she is ill appearing. Her temperature is 102°F (38.8°C) and she has very tender anterior and posterior cervical lymph nodes bilaterally. She cannot open her mouth for you to examine her oropharynx. Her abdominal examination is normal. What is the likely diagnosis?

(A) group A streptococcal pharyngitis

(B) EBV mononucleosis

(C) retropharyngeal abscess

(D) peritonsillar abscess

(E) gonococcal pharyngitis

14. What is the study to best diagnose the problem from question 13?

(A) radiograph of lateral neck

(B) CT

(C) MRI

(D) throat culture

(E) fine-needle aspiration

15. All of the following are useful treatment measures for the diagnosis in question 13 except

(A) parenteral antibiotics

(B) oral steroids

(C) surgical drainage

(D) patient-controlled analgesia (PCA) pump

(E) all are appropriate treatment measures

16. A 21-month-old girl is brought in by her mother because of fever (101°F-103°F [38.3°C-39.4°C]) and anorexia for 2 days. She has not urinated in 12 hours. She has no other symptoms, specifically no cough, rhinorrhea, rash, vomiting, or diarrhea. On examination, she appears quite ill, has an axillary temperature of 102.5°F (39.1°C), heart rate of 140, and a capillary refill of 3 seconds. She holds her head hyperextended and resists examination of her oral cavity. She has tender bilateral cervical lymphadenopathy and has mild stridor. The rest of her examination is normal. What is the most likely diagnosis?

(A) group A streptococcal pharyngitis

(B) EBV mononucleosis

(C) retropharyngeal abscess

(D) peritonsillar abscess

(E) gonococcal pharyngitis

17. What is the most concerning possible complication in this child from question 16?

(A) febrile seizure

(B) airway occlusion

(C) dehydration

(D) sepsis

(E) aspiration pneumonia

18. On arrival in the emergency department, the child from question 16 is examined by an otolaryngologist who notes a right-sided anterior bulge in the posterior oropharynx. Which of the following is the first step of your management?

(A) airway securing and monitoring

(B) placement of IV and nothing by mouth (NPO) order

(C) surgery consult

(D) radiographs

(E) IM dexamethasone

ANSWERS

1. (C) Although bacteria are the etiology in only 5-10% of cases of pharyngitis, this presentation is most likely group A beta-hemolytic streptococcal pharyngitis. It is most common among school-age children and causes rapid-onset pharyngitis with associated symptoms of fever, headache, neck tenderness, abdominal pain, and emesis. Viral pharyngitis is more common in conjunction with other upper respiratory tract symptoms (congestion, rhinorrhea, cough, ear pain). Coxsackie virus is an enterovirus that can cause typical symptoms of the common cold, ulcerative pharyngitis, and handfoot-mouth disease. EBV mononucleosis can cause exudative pharyngitis, and often splenomegaly is found on examination. Group B streptococcus does not cause pharyngitis; it is a major cause of perinatal infections and urinary tract infections among pregnant women.

2. (C) If you are suspicious of streptococcal pharyngitis, a rapid latex test can be performed in your office. Several rapid tests are available, and all require vigorous swabbing of the palate and tonsils, and/or the posterior pharynx. Sensitivities and specificities are similar and approximately 80-90% and 95%, respectively. Because of the high specificity, a positive latex test does not require a confirmatory throat culture. Conversely, a negative rapid test should prompt a throat culture to screen for group A streptococci. Starting antibiotics without documenting group A streptococci by latex or throat culture is not appropriate.

3. (B) All of these are possible complications of group A streptococcal pharyngitis except splenic rupture, which is associated with trauma to the enlarged and friable spleen associated with EBV mononucleosis. A retropharyngeal abscess presents as high fever, drooling, trismus (inability to open jaw), painful pharyngitis, and, sometimes, a toxic appearance. Rheumatic fever in children younger than 3 years old is uncommon. With improved testing and treatment, rheumatic fever in the United States today is uncommon (0.3% in nonepidemics, 3% in epidemics), although it may still occur in association with group A streptococcal outbreaks. All the complications previously listed are usually preventable by timely testing and antibiotic treatment.

4. (B) Penicillin is still the antibiotic of choice for group A streptococcal pharyngitis. Oral (phenoxymethyl) penicillin or IM benzathine penicillin are acceptable. Other beta-lactams such as amoxicillin are also acceptable. Azithromycin or erythromycin have activity against group A streptococci (although rare resistance is reported) but should be reserved for penicillin-allergic patients. Firstgeneration oral cephalosporins (cephalexin, cefadroxil) can be used against group A streptococci but are more expensive and have wider antimicrobial spectra. They should also be reserved for allergic patients. IV or IM cephalosporins are not indicated. Steroids have no role in treating group A streptococci pharyngitis.

5. (E) Reculturing this patient for group A streptococci is an appropriate next step. Posttreatment throat cultures should be reserved only for those patients who are still symptomatic or for those patients who are at very high risk for rheumatic fever. Those who are asymptomatic at the end of treatment but still culture positive should not receive additional antimicrobial treatment because carriage of group A streptococci in the pharynx can continue for several weeks after active infection. One might also consider the possibility of concomitant EBV pharyngitis because approximately 10% of patients with mononucleosis are group A streptococcus positive. This patient may also be a group A streptococcal carrier and have prolonged viral pharyngitis, although a second round of treatment for another positive throat culture is warranted before entertaining the carrier diagnosis. Treating with a different oral or IV antibiotic is unnecessary even if the patient is still positive for group A streptococci.

6. (B) For a persistent symptomatic patient with a positive group A streptococcal culture, the same antibiotic is recommended (penicillin). In this case, continued infection should be ruled out with a second course of penicillin. A persistently positive test for group A streptococci after a second course of penicillin suggests that the patient is a group A streptococci carrier and has another etiology for her pharyngitis.

7. (C) Any testing of children exposed to group A streptococci is unwarranted because asymptomatic carriage during an outbreak can be as high as 25-40%. Only symptomatic children should be examined, and a rapid group A streptococci test and culture should be considered.

8. (E) In toddlers younger than 3 years, group A streptococcal infection may present with serous rhinitis, fever, irritability, and anorexia, instead of the pharyngitis typically seen in older children. However, because the risk of rheumatic fever in this age group is extremely low in developed countries, culturing for group A streptococci is not recommended in this age group unless the patient is known to be at high risk for rheumatic fever or the illness occurs during a rheumatic fever outbreak. In this case, (E) (no test) therefore is the correct answer. Testing for mononucleosis or influenza is not indicated based on the symptoms. The monospot test is insensitive in a 2-year-old child.

9. (B) This is the classic presentation of perianal group A streptococci dermatitis (see Figure 62-1). It occurs more often in boys between the ages of 6 months and 1 year. Presenting symptoms and findings are itching, pain on defecation, blood-streaked stool, and a well-circumscribed erythematous rash from the anus extending outward. Recurrence rates are 40-50%. The differential diagnosis includes A, D, and E, psoriasis, and sexual abuse.

10. (D) Glomerulonephritis is the most serious complication associated with group A strep skin infections (and pharyngeal infections); rheumatic fever is not associated with skin infections caused by group A streptococci.

art

FIGURE 62-1. Group A streptococcus intertrigo—perianal streptococcal cellulitis. Well-demarcated erosive erythema in the perianal region and perineum in an 8-year-old boy who complained of soreness. (Reproduced, with permission, from Wolff K, Goldsmith LA, Katz SI, et al. Fitzpatrick’s Dermatology in General Medicine, 7th ed. New York: McGraw-Hill; 2008: Fig. 177-15.)


11. (B)

12. (E) Group A streptococci dermatitis, especially perianal disease, requires oral antibiotics for treatment. Topical antibiotics will be ineffective.

13. (D) This is a classic story for peritonsillar abscess, most likely caused by group A streptococci (see Figure 62-2). She is experiencing trismus, the inability to open the jaw secondary to peritonsillar and lymphatic edema. If she were to open her mouth, you would find an asymmetric tonsillar bulge, perhaps with uvular and/or palatal displacement. Generally, retropharyngeal abscesses are less common in children older than 5 years because the retropharyngeal nodes that fill the potential retropharyngeal space involute before that age; thus the pathophysiology of this disease differs in adolescents and adults, in whom it is rare. Gonococcal pharyngitis should be considered among sexually active persons and can present with fever, sore throat, and greenish pharyngeal/tonsillar exudates.

art

FIGURE 62-2. Open mouth view with the retractor on the tongue in a patient demonstrating medial right tonsillar displacement, palatal edema, and uvular deviation consistent with a peritonsillar abscess. (Reproduced, with permission, from Tintinalli, JE, Kelen GD, Stapczynski JS. Tintinalli’s Emergency Medicine: A Comprehensive Study Guide, 6th ed. New York: McGraw-Hill; 2004: Fig. 243-1.)


14. (B) A CT of the neck with IV contrast will most clearly and rapidly assess for a ring-enhancing abscess or possibly a phlegmon. Fine-needle aspiration is one approach to surgical treatment but is seldom performed for diagnosis.

15. (B) IV antibiotics and incision and drainage are the mainstays of treatment of peritonsillar abscess (see Figure 62-3). Pain control is usually needed and can be supplied in oral (liquid) or IV form. Oral steroids may be useful in severe mononucleosis but not for peritonsillar abscesses.

art

FIGURE 62-3. Peritonsillar abscess. Acute peritonsillar abscess showing medial displacement of the uvula, palatine tonsil, and anterior pillar. Some trismus is present, as demonstrated by patient’s inability to open the mouth maximally. (Reproduced, with permission, from Knoop KJ, Stack LB, Storrow AS, et al. Atlas of Emergency Medicine, 3rd ed. New York: McGraw-Hill; 2010:118. Photo contributor: Kevin J. Knoop, MD, MS.)


16. (C) Retropharyngeal abscess is a disease of younger children, as stated earlier, whereby infection of the lymph nodes located in the retropharyngeal space is associated with fever, sore throat, trismus, drooling, stridor, and change in voice (cri du canard: cry of the duck [a duck-quacking sound]) (see Figure 62-4).

art

FIGURE 62-4. Retropharyngeal abscess. Lateral soft tissue neck X-ray demonstrating prevertebral soft tissue density constant with retropharyngeal abscess. (Reproduced, with permission, from Widmaier EP, Raff H, Strang KT: Vander’s Human Physiology, 11th ed. McGraw-Hill, 2008:211.)


17. (B) Airway occlusion in this case is a most urgent concern because it appears that the patient has a pharyngeal process. The difficulty in examining a child like this further complicates the situation, but the combination of fever, ill appearance, hyperextension of the neck, and stridor should prompt the physician to be prepared immediately for airway management. The other complications listed are all concerns with this patient but relatively less urgent. Aspiration pneumonia is a known and dangerous complication of retropharyngeal abscess.

18. (A) Securing the airway is the most urgent first step in this case. Radiographic investigation most often begins with lateral neck films (with neck in full extension during deep inspiration), which are evaluated for the width of the retropharyngeal space. The width of the prevertebral soft tissue should be no more than 7 mm at C2 and 20 mm at C 6. If more urgent evaluation is required, or if proper positioning of the neck is not possible, a CT of the neck with contrast is an alternative option.


SUGGESTED READING

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

Long SS, Pickering LK, Prober CC, eds. Principles and Practice of Pediatric Infectious Diseases. Philadelphia, PA: Elsevier; 2008.

Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book 2009: Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009.

CASE 63: A 7-MONTH-OLD WITH CROSSED EYES

A mother brings her 7-month-old full-term twins in to see you for a routine check-up. During the visit she mentions that she is concerned because they both seem to have “crossed eyes” a lot of the time, especially when they are tired or at nighttime.

On examination, of the first child you note that her eyes appear asymmetric. Her left eye appears to deviate inward. She has an asymmetric corneal light reflex, with the left corneal light reflection displaced temporally.

SELECT THE ONE BEST ANSWER

1. All of the following cranial nerves are involved in alignment of the eyes except

(A) II

(B) III

(C) IV

(D) VI

(E) no exception; all of the above are involved

2. Which type of strabismus does she have?

(A) esotropia

(B) esophoria

(C) exotropia

(D) exophoria

(E) pseudostrabismus

3. What is the most appropriate next step for this patient?

(A) observation with reexamination in 3 months

(B) ophthalmology referral

(C) Snellen visual acuity test

(D) cover test

(E) eye patch

4. If you send her to the ophthalmologist, who confirms your diagnosis, what is the most likely treatment of this disorder?

(A) surgical correction

(B) eyeglasses

(C) eye patch

(D) observation

(E) prism glasses

5. What percentage of untreated children with strabismus develop amblyopia?

(A) 10-30%

(B) 20-40%

(C) 30-50%

(D) 50-80%

(E) 90-95%

6. You examine her twin sister and note that her eyes also appear asymmetric. Her right eye appears deviated inward throughout the examination. Her corneal light reflex is normal. Which type of strabismus does she have?

(A) esotropia

(B) esophoria

(C) exotropia

(D) exophoria

(E) pseudostrabismus

7. What is the next recommended action for the patient in question 6?

(A) reassurance

(B) ophthalmology referral

(C) prescription for glasses

(D) eye patch

(E) surgical correction

8. A father brings his 3-year-old son to your office one day because of a “lazy eye” they have been noticing for a few months. His father does not recall any trauma to the eye and tells you that both he and the patient’s mother wear glasses. His son rides a tricycle, helps dress himself, can copy a circle, and uses 3-word sentences. The “lazy eye” doesn’t seem to bother him. On examination, you note a left esotropia, an asymmetric corneal light reflex, and an abnormal cover test. What will the treatment be for his most likely diagnosis?

(A) eye patch

(B) prism glasses

(C) prescription eye glasses

(D) surgical correction

(E) observation

9. Which of the following children does not require a referral to a pediatric ophthalmologist?

(A) a 6-month-old with fetal alcohol syndrome

(B) a 2-week-old with nystagmus

(C) a 5-year-old with Down syndrome

(D) a 4-month-old with intermittent strabismus

(E) a former 26-week premature infant now 9 months old

10. You see a 3-day-old for a well-baby visit in your office and her mother asks you what she is able to see. What can you tell her mother that her vision would be, approximately, if she were able to read off a Snellen chart?

(A) 400/20

(B) 200/20

(C) 20/20

(D) 20/200

(E) 20/400

11. Which of the following children needs to be evaluated further for their vision?

(A) a 6-week-old who cannot smile

(B) a 1-month-old who does not fixate and follow parents’ faces across midline

(C) a 5-month-old with intermittently “crossed eyes”

(D) a 15-month-old who cannot put a peg into a round hole

(E) a 2-year-old with mild hyperopia

Questions 12 through 15. Match the following ages with the most appropriate vision tests and screening tools:

12. 6 months    (A) Allen cards

13. 2.5 years    (B) HOTV or tumbling E’s

14. 4 years       (C) Fix and follow

15. 7 years       (D) Snellen charts

16. An 8-year-old girl is in your office for a routine checkup. On your questioning, she tells you that she has some difficulty seeing the blackboard in class. Her mother states that she does seem to be holding her books closer to her face lately. Sometimes she squints when they are in the car. What will you most likely find on your fundoscopic examination?

(A) +1.00

(B) +4.00

(C) −1.00

(D) −4.00

art

FIGURE 63-1. Ocular muscles. The oculomotor, trochlear, and abducens nerves; and then cranial nerves (II) innervation. (Reproduced, with permission, from Waxman SG. Clinical Neuroanatomy, 26th ed. New York: McGraw-Hill; 2010: Fig. 8-4.)


17. Which of the following statements regarding myopia is false?

(A) most infants’ eyes are slightly myopic

(B) it requires a concave lens to correct

(C) it is often hereditary

(D) most myopia is physiologic

(E) myopia is more common in preterm compared with full-term infants

18. At what age should children begin to have routine screening visual acuity examinations?

(A) 6 months

(B) 1 year

(C) 2 years

(D) 3 years

(E) 4 years

ANSWERS

1. (A) Cranial nerve (CN) III (oculomotor) is responsible for levator muscle constriction, pupillary constriction, and lens accommodation. CN IV (trochlear) innervates the superior oblique muscle and CN VI (abducens) innervates the lateral rectus muscle. CN II (optic) is not involved in aligning the eyes, only in the transmission of visual signals (see Figure 63-1).

2. (A) This is esotropia because her eye is deviated medially (eso-) and is constant (-tropia) as opposed to periodic or latent (-phoria). The corneal light reflex test is performed by the examiner shining a light onto both corneas simultaneously and watching where on the cornea the reflection occurs. In eyes that are aligned, the reflection appears symmetric. If one eye is deviated, the normal eye is centered and the reflex in the deviated eye appears offcenter. Infantile, or congenital esotropia is the most common esodeviation in children (see Figure 63-2).

3. (B) Referrals to ophthalmology for strabismus are not generally made before 6 months of age because intermittent asymmetry of pursuit can be present in healthy infants and resolves by 3-6 months (see Figure 63-3). Observation is not acceptable because delay in treatment increases the likelihood of amblyopia. The Snellen charts are visual acuity tests for vision screening for older children. The cover test is best used to detect visual axis deviations. Each eye is covered in turn while the uncovered eye is examined for tropias. It is an appropriate test to perform when trying to confirm the presence of strabismus as suspected by abnormal corneal light reflex, but it requires patient cooperation to perform and would likely be difficult in an infant this age.

art

FIGURE 63-2. Accommodative esotropia. A. Without glasses, esotropic; B. with glasses, well-aligned at distance; C. and wellaligned at near with bifocal correction. (Reproduced, with permission, from Hay Jr WW, Levin MJ, Sondheimer JM, et al. Current Diagnosis & Treatment: Pediatrics, 19th ed. New York: McGraw-Hill; 2009: Fig. 15-26A-C.)


4. (A) It is widely agreed that surgical correction, namely medial rectus recession, is the treatment most likely to be corrective. The timing of the surgery is controversial, some arguing for as early as 3-4 months, some as late as 1 year.

5. (C) The amblyopia that commonly accompanies strabismus requires separate treatment.

6. (E) This is pseudostrabismus, when one or both eyes appear deviated medially because of prominent epicanthal folds or an especially broad nasal bridge. The corneal light reflex is normal because the eyes are actually aligned and can be confirmed by a cover test in older children.

art

FIGURE 63-3. Strabismus (squint). This term refers to disorders in which the optic axes are not parallel. The diagrams illustrate positions of the patient’s eyes as they appear to the observer. The unbroken circles connected by the unbroken lines show pairs in the primary position with the normal or fixing eye represented in heavier lines. Pairs with broken lines are in secondary positions with the heavier lines for the fixing eye. A. Comitant strabismus: the squint angle between the two optic axes is constant in all positions regardless of which eye fixates. B. Right lateral rectus paralysis: the right eye is unable to move laterally. C. Right medial rectus paralysis: the right eye is lateral in the primary position; it fails to move medially. D. Right superior rectus paralysis: the right eye is slightly depressed in the primary position and fails to move farther upward. E. Right inferior rectus paralysis: the right eye is elevated slightly in the primary position; it cannot move downward. (Reproduced, with permission, from LeBlond RF, DeGowin RL, Brown DD. DeGowin’s Diagnostic Examination, 9th ed. New York: McGraw-Hill; 2009: Fig. 7-35.)


7. (A)

8. (C) He most likely has an accommodative esotropia, which most commonly presents between 2 and 3 years old as a new-onset esotropia. Family histories of amblyopia are common. The great majority of these children have an associated hyperopia, and their esotropia is because of overaccommodation in response to the hyperopia. Treatment of the hyperopia is indicated first (with prescription eyeglasses) (see Figure 63-4).

art

FIGURE 63-4. Common defects of the optical system of the eye. In hyperopia (farsightedness) the eyeball is too short and light rays come to a focus behind the retina. A biconvex lens corrects this by adding to the refractive power of the lens of the eye. In myopia (near-sightedness) the eyeball is too long and light rays focus in front of the retina. Placing a biconcave lens in front of the eye causes light rays to diverge slightly before striking the eye so that they are brought to a focus on the retina. (Reproduced, with permission, from Widmaier EP, Raff H, Strang KT: Vander’s Human Physiology, 11th ed. McGraw-Hill, 2008:211.)


9. (D) Strabismus can be found in healthy infants until age 3-6 months, at which time the inability to follow a target symmetrically resolves. Ophthalmology referrals are needed for children with genetic syndromes, in utero drug or alcohol exposure, infants with retinopathy of prematurity (a 26-week gestation premature infant would most likely have this), children who have evidence of ocular pathology on examination, and children with a strong family histories of vision difficulties.

10. (E) When measured by visual evoked potential, newborns have visual acuity of approximately 20/400.

11. (D) Peg-in-hole games are visually related behaviors that should suggest to the practitioner possible vision impairment if this milestone is not achieved on time. By 8-9 months, a child should poke at the holes with pegs and by 12-14 months should be able to put pegs into the appropriate holes. Infants should smile at 2-3 months, and fix and follow by 2-3 months (by 2 months it is a red flag; by 3 months an absolute referral). Strabismus is referred to ophthalmology after 6 months. All children are mildly hyperopic until approximately 10 years old.

12. (C)

13. (A)

14. (B)

15. (D) The most advanced test of visual acuity possible should be used for a child’s developmental age. The Allen cards are a series of familiar object cards that the child is asked to identify at increasing distances. The appropriate vision screening tools for ages 3-5 years are the matching-type tests, namely the HOTV test (chart on wall with letters H, O, T, V and child holds matching cards) and the tumbling Es (chart on wall with the letter E in various rotations and child holds matching cards).

16. (D) This patient by history has myopia, nearsightedness, which is causing her to strain to see far objects—squinting in the car or pulling objects closer to her face to see them more clearly. The presence of clinical symptoms for some time suggests the need for a higher diopter on examination (see Figure 63-5).

17. (A) Almost all infants are born with slight hyperopia that increases throughout childhood, peaks at approximately 6 years old, and then decreases again. Most myopia is physiologic as opposed to pathologic.

art

FIGURE 63-5. Spherical refractive errors as determined by the position of the secondary focal point with respect to the retina. (Reproduced, with permission, from Riordan-Eva P, Whitcher JP. Vaughn & Asbury’s General Ophthalmology, 17th ed. McGraw-Hill; 2008: Fig. 20-21.)


18. (D) The AAP and the American Academy of Ophthalmology recommend routine visual acuity testing at 3 years and again at 5-6 years. Screening for eye disease should be performed regularly, at least in the first 3 months of age and again at 12 months.


SUGGESTED READING

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

Committee on Practice and Ambulatory Medicine; American Academy of Ophthalmology. Eye examination in infants, children, and young adults by pediatricians. Pediatrics. 2003;111(4):902-907.

Curnyn KM. The eye examination in the pediatrician’s office. Pediatr Clin North Am. 2003;50(1):25-40.

Guthrie ME. Congenital esotropia. Ophthalmol Clin North Am. 2001;14(3):419-424.

CASE 64: A 4-YEAR-OLD WITH A NECK MASS

A 4-year-old comes to your office with his father because of a “lump in the neck” that his parents noted 4 days earlier. The boy does not seem bothered by it, and although he is not currently ill, he did have a “cold” about 1 week ago. He has not had a fever, is eating and drinking well, has no specific tooth pain, and no pain on swallowing.

On examination he is afebrile. His oropharynx is normal, nares clear, and his conjunctivae are white. He has a tender 4-cm submandibular lymph node on the left side that has overlying erythema. His abdominal and skin examinations are normal.

SELECT THE ONE BEST ANSWER

1. What is his most likely diagnosis?

(A) cat-scratch disease

(B) “atypical” or environmental Mycobacterium infection

(C) acute lymphadenitis

(D) mumps

(E) tuberculosis (TB)

2. What is the least likely pathogen causing his illness?

(A) “atypical” or environmental mycobacteria

(B) Streptococcus pyogenes (group A streptococci)

(C) S aureus

(D) EBV

(E) anaerobic bacteria

3. What antibiotic will you prescribe for him?

(A) IM ceftriaxone

(B) oral (PO) azithromycin

(C) PO amoxicillin

(D) PO cephalexin

(E) PO amoxicillin-clavulanate

4. Which of the following are also known to cause cervical lymphadenopathy?

(A) Non-Hodgkin lymphoma

(B) Kawasaki disease

(C) systemic lupus erythematosus

(D) A and B

(E) A, B, and C

5. You are called by the parents of a 2-week-old newborn boy, who report poor feeding for 1 day and some neck swelling on the right side. On examination you note a well-hydrated, fussy but consolable infant with a temperature of 101.2°F (38.4°C) rectally and a right-sided 2 × 4-cm spongy, tender neck mass with overlying erythema. The rest of the examination is normal. What are the possible etiologies of the neck mass in this child?

(A) S pyogenes (group A streptococci)

(B) Streptococcus agalactiae

(C) group B streptococci

(D) S aureus

(E) all of the above

6. You see a 3-year-old girl who is brought to your office because of a lump in her neck that her parents noticed about 6 weeks prior. They are concerned that it is still present and not diminishing in size. In all other regards, the parents report she is in good health and has received all of her vaccinations. They live in a suburb and have no pets. They have not traveled in more than a year. On examination, she is afebrile and very cooperative. She has multiple firm, mobile left-sided cervical and submandibular nodes, the largest of which is 4.5 cm. There is no erythema, warmth, or drainage present. Her oral cavity, ears, conjunctivae, and scalp are normal. Her abdominal and skin examinations are normal.

Laboratory data include:

CBC: white blood count (WBC) 8.1 (48% lymphocytes, 33% neutrophils, 15% monocytes, 4% eosinophils)

ESR: 21

Purified protein derivative (PPD): 11 mm

What is the next test that should be ordered?

(A) blood culture

(B) chest radiograph

(C) lymph node biopsy

(D) CT of neck

(E) MRI of neck

7. Which of the following is the most reliable way to ascertain the etiology of the infection in the patient in question 6?

(A) gastric lavage specimen

(B) sputum specimen

(C) urine specimen

(D) blood culture

(E) surgically excised tissue

8. What is the least likely way that nontuberculous mycobacteria are transmitted?

(A) inhalation

(B) ingestion

(C) person to person

(D) direct contact with the organism

(E) animal contact

9. Which of the following is the least likely clinical manifestation of the organism from a nontuberculous mycobacteria infection?

(A) skin ulcer

(B) disseminated infection

(C) bone infections

(D) lymphadenitis

(E) soft tissue infection

10. The lymph node biopsy that you ordered grew Mycobacterium intracellulare, and the blood culture is negative. Which of the following is most likely to lead to a cure from question 6?

(A) ciprofloxacin

(B) doxycycline

(C) azithromycin and ethambutol

(D) azithromycin, ethambutol, and rifampin

(E) surgical excision

11. You see a 4-year-old girl who comes in for a “lump” in her neck that she has had for about 3 weeks. Her parents do not recall any trauma to the area, toothaches, or illnesses in the last month. They are worried that she has “throat cancer” because her grandmother has just been diagnosed with the disease. She has 2 cats at home and has not traveled in the past 6 months. On examination you find a happy, cooperative child who is afebrile. She has a visible asymmetry of the neck. She has several leftsided tender cervical lymph nodes with overlying erythema and warmth. What is the most likely etiology of her disease?

(A) Yersinia pestis

(B) Francisella tularensis

(C) Bartonella henselae

(D) Chlamydia trachomatis

(E) Corynebacterium diphtheriae

12. Which of the following are other possible clinical manifestations of the organism from question 11?

(A) hepatitis

(B) aseptic meningitis

(C) Parinaud oculoglandular syndrome

(D) pharyngitis

(E) all of the above

13. In which seasons is this disease most commonly manifested by the patient in question 11?

(A) winter/spring

(B) summer/fall

(C) summer/winter

(D) spring/fall

(E) no seasonal variance

14. What is the best treatment for this patient from question 11?

(A) reassurance

(B) oral antibiotics

(C) get rid of cats in the home

(D) fine-needle aspiration

(E) incision and drainage

15. You see a 5-year-old girl for a check-up and her parents tell you they are concerned about a small lump on her neck. It has been there for some time but was small and nontender, and they were waiting for their checkup to ask you about it. But 7 days ago she developed an upper respiratory tract infection with sneezing, coughing, and mild fever, and now the lump is larger than it was 2 weeks ago and somewhat tender. On examination you find a happy, cooperative, afebrile young girl. She has mild conjunctival injection bilaterally without discharge, red swollen turbinates and rhinorrhea, and a wet cough. Her lungs are clear. Her neck has a small 0.5-cm bulge in the midline, just above the suprasternal notch. It is tender to the touch and feels slightly boggy. There is no erythema. What is her most likely diagnosis?

(A) thyroid mass

(B) furuncle

(C) lymphadenitis

(D) thyroglossal duct cyst

(E) branchial cleft cyst

16. Another 5-year-old girl is seen for a neck mass that also has been there for some time. She comes in because it is now tender and enlarging. Yesterday some “pus” started coming out of it. She has had no fever, had a “cold” 2 weeks ago, and is generally in good health. On examination she is afebrile. She has a 4-cm tender, smooth, fluctuant mass along the anterior border of the sternocleidomastoid muscle on the left side. There is a small dimple in the middle with yellow crust around it. You are able to express some serosanguineous fluid with mild pressure. The rest of her head and neck examination is normal. What is her diagnosis?

(A) thyroiditis

(B) furuncle

(C) lymphadenitis

(D) thyroglossal duct cyst

(E) branchial cleft cyst

17. What is the best way to evaluate this lesion from question 16?

(A) radiograph of neck

(B) ultrasound of neck

(C) CT of neck

(D) MRI of neck

(E) B and C

18. In regard to question 16, are these more often unilateral or bilateral?

(A) unilateral

(B) bilateral

ANSWERS

1. (C) This is most likely to be acute unilateral pyogenic lymphadenitis, often seen in children 1-4 years old following an upper respiratory tract infection. In more than 50% of cases, it is the submandibular nodes that are affected. The tempo of the illness and the overlying illness are the most important clues. Cat-scratch disease is a possibility but less likely.

2. (D) EBV usually causes either bilateral or generalized lymphadenopathy and more constitutional symptoms. Group A streptococci and S aureus cause more than 80% of cases like these. Infections caused by nontuberculous mycobacteria more often have an insidious onset, although they can uncommonly cause a picture similar to acute pyogenic lymphadenitis.

3. (D) Because staphylococci and streptococci are the primary pathogens, an oral cephalosporin such as cephalexin is the most appropriate choice in areas where the prevalence of MRSA in the community is low. Clindamycin would be a better choice if community-acquired MRSA is common in the patient’s area.

4. (E)

5. (E) S aureus is the most common cause of neonatal acute cervical lymphadenitis, but another important cause of neck mass in the newborn is group B streptococci, the “cellulitis-adenitis” syndrome. This most often presents at 3-7 weeks, 75% of patients are boys, and most have the overlying cellulitis previously described. S agalactiae and group B streptococci are synonyms.

6. (B) It is essential to exclude M tuberculosis as early as possible because of the abnormal PPD (a positive tuberculin skin test in an otherwise healthy 3-yearold is >10 mm). However, caution must be used in interpreting PPD results in children in whom a nontuberculous mycobacterial (NTM) infection is possible because antigens of the latter can elicit a response with PPD. A chest radiograph should be the next test ordered. After that, a lymph node biopsy and a CT scan of the neck are possible further avenues of investigation.

7. (E) Because NTM are present in the environment (soil, water), specimens from nonsterile locations such as stomach, urine, and sputum can be contaminated. A blood culture will only be positive if disseminated disease is present, a rare event. Examination and culture of the tissue obtained by surgical excision is the gold standard to diagnose caseating granulomatous infections in the presence of an 11 mm PPD.

8. (C) NTM are found in soil and water, and transmission can be by inhalation, ingestion, direct contact, and animal contact by means of soil and water. Person-to-person transmission is rare.

9. (B) NTM can cause the other infections listed, but disseminated infection occurs only in immunocompromised hosts. Children with CF may have NTM in their sputum.

10. (E) For NTM in an otherwise healthy child, complete surgical excision almost always leads to cure. Ciprofloxacin and doxycycline may be effective against rapidly growing mycobacteria (M fortuitum, M abscessus). C and D represent multidrug therapy, reserved for immunocompromised patients or those with disseminated disease. Ethambutol is seldom used in children.

11. (C) This is most likely cat-scratch disease caused by Bartonella henselae, although the patient is afebrile. In more than three-fourths of cases, a history of a cat or kitten exposure is given (see Figure 64-1).

art

FIGURE 64-1. Bartonellosis: cat-scratch disease with primary lesion. Erythematous nodule of the cheek of a 9-year-old girl at the site of the cat scratch. Diagnosis was made on the histologic findings of the excised specimen. (Reproduced, with permission, from Wolff K, Johnson RA. Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology, 6th ed. New York: McGraw-Hill; 2009: Fig. 24-55.)


12. (E) In addition to those listed, other manifestations of Bartonella henselae infection include encephalitis, fever of unknown origin, pneumonia, thrombotic thrombocytopenic purpura (TTP), erythema nodosum, and osteolytic lesions. Parinaud oculoglandular syndrome presents as conjunctivitis and preauricular or submandibular lymphadenopathy that is ipsilateral to the primary infection.

13. (A)

14. (A) Antibiotics are usually reserved for disseminated cat-scratch disease. Getting rid of the cats/kittens will not help this episode, although patients should be instructed to avoid rough play with cats and kittens to avoid scratches, and, if scratched, should wash the wound immediately. Incision and drainage as well as surgical excision should be avoided.

15. (D) Thyroglossal duct cysts appear during childhood and often enlarge rapidly in the setting of an infection. They are located in the midline, between the hyoid bone and the suprasternal notch, and move up when the patient sticks the tongue out or during swallowing (see Figure 64-2). The lump could represent thyroid tissue, although unlikely given this history, but must be ruled out before surgery is undertaken to excise the cyst.

art

FIGURE 64-2. Midline mass in an adolescent demonstrating a thyroglossal duct cyst. (Reproduced, with permission, from Tintinalli, JE, Kelen GD, Stapczynski JS. Tintinalli's Emergency Medicine: A Comprehensive Study Guide, 6th ed. New York: McGraw-Hill; 2004: Fig. 243-6.)


16. (E) This most likely is a branchial cleft cyst, located at the anterior border of the sternocleidomastoid (SCM) muscle. When superinfected, like this one, it enlarges and may drain purulent fluid.

17. (E) Typically, ultrasound is the preferred modality to begin the evaluation of branchial clefts in children. CT is often performed as well.

18. (A) 2-3% are bilateral.


SUGGESTED READING

Behrman RE, Kliegman RM, Jenson HB, et al. Nelson Textbook of Pediatrics. Philadelphia, PA: WB Saunders; 2007.

Chesney PJ. Nontuberculous mycobacteria. Pediatr Rev. 2002; 23(9):300-308.

Peters TR, Edwards KM. Cervical lymphadenopathy and adenitis. Pediatr Rev. 2000;21(12):399-404.

Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book 2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009.

CASE 65: A 3-MONTH-OLD BOY WITH APNEA

A 3-month-old boy is brought to your emergency department on Thanksgiving after Mom noted that he “stopped breathing.” He was in his usual state of health until 3 days before presentation when he developed nasal congestion and mild cough. The cough worsened, resulting in decreased intake, and, according to Mom, progressed toward an increased effort of breathing. The night of admission Mom observed a particularly harsh coughing spell, followed by a period where no breathing was noted for approximately 20-30 seconds. Mom denies any color changes, abnormal movements, loss of tone, emesis, or fever. After the breathing resumed, the infant was breathing abnormally fast, and Mom continued to note an increased effort to breathe. She brought him to the emergency department and noted no further episodes on the way.

Past medical history and family history are unremarkable. Immunizations are up to date and he takes no known medications. The infant lives at home with his mom, dad, and 3-year-old sister, all of whom are healthy. There are no smokers in the home. He attends day care.

On your examination, he is resting quietly, with vital signs of T (temperature) 100.7°F (38.2°C) rectally, heart rate (HR) 144, respiratory rate (RR) 62, BP 100/70, and oxygen saturation of 90% on room air (RA). He appears mildly dehydrated but not toxic. The right TM has decreased mobility with poor landmarks, and nasal congestion and discharge are noted. The lung examination reveals tachypnea with subcostal retractions and nasal flaring. He has diffuse wheezes and crackles through both lung fields. A wet cough is noted. Neurologically he has no focal deficits and is alert and responsive. The remainder of the examination is benign.

He is placed on 1 L oxygen by nasal cannula and given an albuterol nebulizer treatment with no apparent response. Chest radiograph demonstrates right middle lobe atelectasis with hyperinflation but no focal infiltrates.

SELECT THE ONE BEST ANSWER

1. Which of the following is the most likely etiology for this patient’s symptoms?

(A) C trachomatis (B) pertussis

(C) respiratory syncytial virus (RSV)

(D) reactive airway disease

(E) none of the above

2. Which of the following would be most useful in directing the immediate course of action for this patient?

(A) CBC with differential

(B) rapid enzyme immunoassay for RSV

(C) EEG

(D) viral culture of nasopharyngeal aspirate

(E) none of the above

3. Which of the following has been proven unquestionably useful for the patient’s therapy?

(A) nebulized albuterol

(B) nebulized budesonide

(C) oral prednisolone

(D) aerosolized ribavirin

(E) none of the above

4. Which of the following is not an important issue with regard to the infection control of RSV?

(A) prevention of fecal-oral spread

(B) good handwashing

(C) institution of hospital contact isolation

(D) parental education

(E) patient cohorting in hospital

5. Which of the following patients is not at increased risk for RSV complications?

(A) a 6-year-old status post bone marrow transplant

(B) a 7-month-old, former 30-week premature infant without chronic lung disease

(C) an 8-month-old with diagnosed chronic lung disease

(D) a full-term infant with congenital heart disease

(E) a 9-month-old, former 27-week premature infant without chronic lung disease

6. Which of the following is false regarding the epidemiology of RSV?

(A) peak months are November through May in temperate climates

(B) the virus can remain viable on countertops for hours

(C) peak age of onset is birth to 2 months

(D) spread is via ocular or nasal direct contact with large droplets in secretions

(E) the virus can be shed for 3-8 days

7. True or False: Prior infection with RSV confers lifelong immunity.

(A) True

(B) False

8. True or False: Most children have been infected with RSV by 4 years old.

(A) True

(B) False

9. For an infant who presents with a staccato cough without fever, what is the most diagnostically useful finding on CBC?

(A) absolute lymphocytosis

(B) neutropenia

(C) thrombocytopenia

(D) eosinophilia

(E) normocytic anemia

10. True or False: Newborns of mothers with untreated C trachomatis during pregnancy should be treated empirically for a chlamydia infection in addition to receiving eye prophylaxis given primarily for gonorrhea.

(A) True

(B) False

11. If a newborn does acquire C trachomatis during delivery, what are the chances that the infant will eventually have a symptomatic infection?

(A) 25-50% for pneumonia

(B) 50-75% for conjunctivitis

(C) 5-20% for pneumonia

(D) 5-20% for conjunctivitis

(E) 50-75% for pneumonia

12. Which of the following statements is true regarding the treatment of infant chlamydial infections?

(A) azithromycin for 5 days is the treatment of choice

(B) erythromycin should not be given to children younger than 6 weeks because of the risk of pyloric stenosis

(C) topical erythromycin alone is adequate for the treatment of conjunctivitis

(D) oral erythromycin should be given for both conjunctivitis and pneumonia

(E) none of the above

13. Which of the following (A-D) is not a clinical presentation of an apparent life-threatening event (ALTE)?

(A) limpness

(B) gagging

(C) cyanosis

(D) plethoric color changes

(E) all of the above could be a clinical presentation of an ALTE

14. Which of the following is a potential diagnosis to explain an ALTE?

(A) gastroesophageal reflux

(B) anemia

(C) pertussis

(D) bronchiolitis

(E) all of the above

15. A 2-week old infant has an apneic event. The infant was born via uncomplicated vaginal delivery and had an unremarkable postnatal course. Mom denies any complications of pregnancy or infections. All appropriate bacterial cultures are pending, and an EEG demonstrates sharp spikes in the temporal lobe. What is the most appropriate next step to help in diagnosis?

(A) send CSF for viral culture

(B) send CSF for a specific polymerase chain reaction (PCR) test

(C) send serum for a specific PCR test

(D) obtain a pH probe study

(E) obtain a head CT

16. What would be the appropriate therapy for herpes simplex virus meningoencephalitis?

(A) acyclovir PO for 4 weeks

(B) acyclovir IV for 3 weeks

(C) ganciclovir IV for 3 weeks

(D) oseltamivir IV for 4 weeks

(E) foscarnet IV for 3 weeks

17. What is the most important goal of hospitalization for an ALTE?

(A) obtaining definitive diagnosis

(B) establishing home apnea monitoring

(C) educating family on infant cardiopulmonary resuscitation (CPR)

(D) involving child protective services

(E) all of the above

ANSWERS

1. (C) The patient described in the vignette most likely has an infectious etiology for his respiratory symptoms. The most likely of the infectious choices in this scenario is RSV. The most common cause of respiratory tract infection in infants and young children, RSV peaks between November and May. Hypoxia and wheezing following congestion and cough are classic clinical features of RSV infection. Chlamydia infects younger patients (2 months) who usually have a staccato cough with, possibly, a history of conjunctivitis. Pertussis, although common in young infants, is less frequently seen after the 4-month immunization visit and tends to present with a paroxysmal cough without wheezing. Both chlamydia and pertussis, like RSV, however, can cause apnea, although chlamydia does so rarely. Reactive airway disease is less likely with a negative family history of asthma, no prior episodes, and lack of response to bronchodilators.

2. (B) With clinical suspicion high for RSV, the most rapid diagnostic tool would be a rapid RSV screen by immunoassay. A positive test would prevent further unnecessary laboratory testing and antibiotic use, as well as allow for proper infection control steps. The sensitivity and specificity of the rapid tests exceed 90%. Although a viral culture would confirm any screen, results take several days and RSV is not recoverable on routine viral culture media. A CBC could suggest concern for other infectious causes (eg, lymphocytosis in pertussis or eosinophilia in Chlamydia) but is not specific. An EEG is often included in the workup of apnea but would not be warranted until an infectious cause is ruled out.

3. (E) The only uncontroversial method for RSV treatment is supportive care. Careful attention should be paid to oral intake because increased respiratory rate can prevent adequate nutritional intake, and IV fluids may be necessary. Supplemental oxygen may be necessary for persistent desaturation. Multiple studies have investigated the role of bronchodilators, steroids, and antivirals, with no consistent results. Although some studies have shown a correlation with ribavirin use and certain relevant clinical outcomes, these studies are also contradictory and inconclusive. Bronchodilators and steroids have shown inconsistent benefit at best and are not cost effective for the minimal improvement they may offer.

4. (A) RSV is transmitted via large-droplet secretions via nasal or ocular contact. Respiratory isolation is not necessary, although the remaining answers are all mainstays for prevention of community and nosocomial transmission.

5. (B) Infants born after a 29-32 week gestation who do not have chronic lung disease are not at increased risk after 6 months of age.

6. (C) Peak age of onset is 2-5 months.

7. (B) False. Reinfection often occurs with RSV, although subsequent infections tend to be less severe.

8. (A) True. Most children in the United States will be infected by the age of 2 years, although more than 75% of hospital admissions for RSV will be in children younger than 1 year.

9. (D) Patients with C trachomatis pneumonia often have eosinophilia on CBC. Although this is not diagnostic, it is highly suggestive of such an infection in a child who fits the classic clinical picture (staccato cough, afebrile pneumonia, 1-3 months of age).

10. (B) False. There are no studies that prove the efficacy of empirical treatment of a newborn born to a Chlamydiapositive mother. Treatment is based on development of symptoms (conjunctivitis, pneumonia).

11. (C) The risk of an infant acquiring Chlamydia pneumonia is 5-20%. The risk of conjunctivitis is greater, 25-50%, although chlamydial conjunctivitis does not present before day of life 5.

12. (D) Oral erythromycin is currently the recommended therapy for both conjunctivitis and pneumonia caused by C trachomatis. Topical therapy for conjunctivitis is ineffective. Azithromycin has not been approved for children younger than 6 months. Although an association between erythromycin and pyloric stenosis has been reported in infants, such an association has not been proven, and thus erythromycin is still recommended for therapy even for infants younger than 6 weeks.

13. (E) An ALTE is defined as any episode that is frightening to the observer. All of the preceding symptoms can be included in that definition. Most ALTE patients require admission and observation/ workup.

14. (E) All of the above are potential etiologies for ALTE. The workup of ALTE is focused on historical and physical findings directing the investigation. A large percentage of patients have no identifiable cause for the ALTE and have no repeat events either in the hospital or after discharge.

15. (B) The patient described above has an ALTE secondary to seizure activity consistent with neonatal herpes infection. Confirmation of a herpes CNS infection could be achieved by CSF PCR. A CSF viral culture is rarely positive, although it should be attempted. CT scans demonstrate changes that are consistent but not diagnostic. After obtaining CSF for PCR, antivirals targeting herpes should be used pending the result.

16. (B) Acyclovir is the drug of choice for CNS HSV infection in neonates. The dose is 20 mg/kg per dose 3 times per day for 14-21 days.

17. (C) The most important discharge goal for ALTE is to educate parents on infant CPR. Home monitoring has not been proven to reduce the incidence of SIDS and often causes more parental stress. Most cases of ALTE are of unknown etiology. Although child protective services may be warranted in some cases of ALTE when the clinical history is suspicious, most cases only require parental education.


SUGGESTED READING

Meissner HC, Long SS; American Academy of Pediatrics Committee on Infectious Diseases and Committee on Fetus and Newborn. Revised indications for the use of palivizumab and respiratory syncytial virus immune globulin intravenous for the prevention of respiratory syncytial virus infections. Pediatrics. 2003;112:1447-1452.

Pickering LK, Baker CJ, Kimberlin DW, Long SS, eds. Red Book2009 Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009.

RSV pages. Centers for Disease Control and Prevention Web site. http://www.cdc.gov/rsv/index.html. Accessed June 2010.

CASE 66: A 2-YEAR-OLD BOY WHO NEEDS A CHECKUP

A 2-year-old boy presents for a routine health supervision visit. He is a new patient to your clinic and arrives with few medical records. Mom reports that he has been well his whole life, although his previous doctor mentioned that he was “behind on the things he does.” She does not have any concerns. He started day care 4 months prior and is starting to attempt toilet training. Bowel movements are every other day and can be hard. His diet consists of eating most foods, with minimal vegetables, but limited “junk food,” and he drinks 4-5 glasses of whole milk per day. Developmentally he can brush his teeth with help but does not dress/undress himself. He can build a tower of 6 cubes, climbs steps, but does not kick a ball. He has a 20-word vocabulary and is just starting to combine 2-word phrases. Family history is negative for any chronic illness.

On physical examination, his height is 88 cm and weight is 13.2 kg. Significant findings include mild pallor of the oral mucosa, a I/VI systolic ejection murmur with good pulses, dry plaques on the antecubital fossae bilaterally, and no focal neurologic findings. Otherwise his examination is unremarkable.

SELECT THE ONE BEST ANSWER

1. What screening test should be ordered for this patient?

(A) folate level

(B) lipid panel

(C) complete blood count

(D) PPD

(E) echocardiography

2. A CBC is obtained. The patient has a hemoglobin of 10.3, hematocrit of 30.7, and a mean corpuscular volume (MCV) of 65. Which of the following is not in your differential?

(A) iron deficiency anemia

(B) thalassemia

(C) folate deficiency

(D) lead poisoning

(E) anemia of chronic disease

3. Which of the following iron studies are consistent with iron deficiency anemia?

(A) low MCV, normal red [blood cell] distribution width (RDW), normal total iron-binding capacity (TIBC), high free erythrocyte porphyrin (FEP)

(B) low MCV, normal RDW, normal TIBC, normal FEP

(C) low MCV, high RDW, high TIBC, high FEP

(D) low MCV, normal RDW, low TIBC, high FEP

(E) none of the above

4. Based on your history, what other tests might be warranted for this child?

(A) Denver II developmental screening

(B) serum lead level

(C) environmental exposure screen

(D) A and B

(E) A, B, and C

5. Which of the following situations would not warrant a risk-based evaluation for lead poisoning?

(A) patient lives in a house built in 1960

(B) patient has a history of pica

(C) patient has a sibling with history of lead poisoning

(D) patient frequently visits a house built in 1960 that was renovated 4 months ago

(E) A and D

6. The Centers for Disease Control and Prevention (CDC) recommends universal screening for lead poisoning for all children between 9 and 12 months.

(A) true

(B) false

7. Which of the following is not an effect of elevated blood lead levels?

(A) colic

(B) nephropathy

(C) advanced pubertal development

(D) encephalopathy

(E) hemolytic anemia

8. The patient’s lead level is 18 μg/dL. What is the next step?

(A) begin oral chelation therapy

(B) repeat the lead level in 1 week

(C) begin nutritional and environmental counseling

(D) stop the supplemental iron therapy

(E) none of the above

9. At what lead level should chelation be initiated?

(A) 25 μg/dL

(B) 35 μg/dL

(C) 45 μg/dL

(D) 55 μg/dL

(E) 70 μg/dL

10. Which of the following is true regarding chelation?

(A) dimethylsuccinic acid (DMSA) is initially given for 21 days

(B) hospitalization for therapy is not required until a level is higher than 75 μg/dL

(C) parenteral agents include DMSA and calcium disodium ethylenediaminetetraacetic acid (EDTA)

(D) calcium disodium EDTA is toxic when given with iron

(E) DMSA side effects include decreased absolute neutrophil count (ANC) and increased liver function tests (LFTs)

11. Which of the following is not a potential side effect of bronchoalveolar lavage (BAL)?

(A) fever

(B) anaphylaxis

(C) tachycardia

(D) hypotension

(E) salivation

12. Which of the following is a contraindication to BAL therapy?

(A) iron therapy

(B) renal insufficiency

(C) hepatic insufficiency

(D) G6PD

(E) encephalopathy

13. Which of the following is not a potential environmental exposure for lead in the United States?

(A) old furniture

(B) food cans

(C) folk remedies

(D) nearby industry

(E) target shooting

14. According to the second National Health and Nutrition Examination Survey (NHANES II) data, which of the following are independent risk factor for elevated blood lead levels?

(A) poverty

(B) age younger than 6 years

(C) African American ethnicity

(D) dwelling in the city

(E) all of the above

15. Which of the following is a method of prevention for lead intoxication?

(A) frequent meals

(B) meals with high vitamin C, low calcium

(C) use of a low-phosphate detergent for cleansing

(D) limit iron intake

(E) increase fat in meals

16. All of the following regarding lead toxicity and postexposure prevention are true except

(A) painting over existing lead-based paint creates only temporary protection

(B) soil coverage with fabric and ground cover limits ground exposure to lead

(C) use of glass and carbon water filters prevents water transmission

(D) use of high-efficiency particulate air (HEPA) vacuum for cleaning is necessary to remove lead from the home

(E) cleaning can temporarily increase the ingestion risk

17. Which of the following is an indication for cholesterol screening in a child older than 2 years of age?

(A) a grandmother who died of a myocardial infarction (MI) at the age of 60 years

(B) a grandfather with documented hypercholesterolemia

(C) an uncle with an MI at age 45 years

(D) a father with angina at age 45 years

(E) none of the above

18. Which of the following patients should be immediately screened for TB?

(A) an international adoptee from Thailand

(B) a sibling of an asymptomatic known HIVinfected patient

(C) the child of a mother with a positive PPD and normal chest radiograph treated with a 9-month course of isoniazid

(D) A and C

(E) B and C

19. Which of the following findings would be considered a developmental delay on the Denver II for a 2-year-old?

(A) inability to wash and dry hands

(B) inability to combine words

(C) inability to kick a ball forward

(D) having half understandable speech

(E) inability to point to 4 pictures

art

FIGURE 66-1. A.The peripheral blood in severe megaloblastic anemia. B. The bone marrow in severe megaloblastic anemia. (Reproduced, with permission, from Fauci AS, Kasper DL, Braunwald E, et al. Harrison’s Principles of Internal Medicine, 17th ed. New York: McGraw-Hill; 2008: Fig. 100-2ab.)


ANSWERS

1. (C) With his nutritional history and physical symptoms, this patient is at risk for iron-deficiency anemia. A CBC would be beneficial to address an immediate and treatable concern; measuring the hemoglobin and hematocrit alone would not be enough information to diagnose iron deficiency because a high RDW and low MCV, if present, would be strongly suggestive. Both a PPD and lipid screen would be appropriate at this age if the history suggested corresponding risk factors. Echocardiography and a folate-level determination are not routine screening tests based on this patient’s history and clinical examination.

2. (C) Folate deficiency is a macrocytic anemia, not a microcytic anemia (see Figure 66-1). The remaining 4 disease states can all be associated with microcytic anemia.

3. (C) Iron studies can often be used to differentiate the main causes of microcytic anemia. The iron studies in option C are most consistent with lead poisoning, thalassemia trait, and chronic disease. Patients with iron deficiency anemia will also have a low serum iron, unlike thalassemia trait and lead poisoning.

4. (E) The given history of development is consistent with developmental delay. To assess this delay, a complete Denver II should be performed. If the patient has 2 or more “delays” on examination, he should be referred for evaluation for services. With developmental delay and microcytic anemia, this patient may have an elevated lead level. Both a serum lead level and an environmental exposure screen are warranted at this time.

5. (A) The AAP and CDC recommend screening questions for lead poisoning risk. Children who live in or visit homes built before 1950 or those built before 1978 with renovations in the past 6 months warrant lead screening. Also, if they have any demonstrated pica or siblings/playmates with lead poisoning, they warrant testing.

6. (B) False. The CDC recommends universal screening in all high-risk areas based on the prevalence of elevated lead levels. Patients in low-to-moderate risk areas should be tested based on screening criteria given in answer 5.

7. (C) Of those listed, the only one not seen in acute or chronic lead poisoning is advanced pubertal development. Mildly elevated blood lead levels have been associated with delayed breast and pubic hair development and decreased height in girls.

8. (C) Currently the CDC recommends retesting lead levels in 3 months if the lead level is 10-19 μg/dL. Nutritional and environmental counseling should be initiated in any patient with a level greater than 14 μg/dL. Chelation is not warranted at this lead level. Iron therapy, although controversial during IV chelation therapy for lead poisoning, is nevertheless recommended in patients with documented iron deficiency anemia.

9. (C) Standard chelation should be used if the lead level is 45 μg/dL or higher. According to CDC recommendations, a level of 45 μg/dL or higher should be repeated and verified within 48 hours.

10. (E) Chelation can be given orally or parenterally. Some form of chelation is begun at a level of 45 μg/dL or higher, with parenteral treatment in the hospital setting started for a level of 70 μg/dL or higher. The oral chelation agent used is DMSA (succimer), which can be given initially for 19 days. This chelation must occur in a lead-free environment. Parenteral chelation, which can be begun at levels 45 μg/dL or higher, includes calcium disodium EDTA and BAL simultaneously. BAL is toxic when given with iron.

11. (D) Side effects of BAL include fever, tachycardia, hypertension, salivation, tingling around the mouth, anaphylaxis, and hemolysis in G6PD patients.

12. (C) Hepatic insufficiency is a contraindication to BAL/dimercaprol therapy. Peanut allergy is also a contraindication because BAL/dimercaprol is dissolved in peanut oil for administration. BAL administration should be used cautiously in those with renal impairment and in the presence of G6PD.

13. (B) All of the above historically have been noted to contain lead, although food cans have been lead free since U.S. legislation in 1977 limited lead in household paint and gasoline. This legislation also stopped the soldering of cans for food. The remaining items are all still possible sources of lead.

14. (E) All of the given answers are independent risk factors for elevated blood lead level.

15. (A) Recommendations for prevention of lead intoxication include a balanced diet with frequent meals that are high in iron, vitamin C, and calcium, all of which compete with lead for GI absorption.

16. (C) Ion-exchange and reverse-osmosis filters, as well as distillation, are effective in removing lead from water sources. However, glass and carbon filters, the most common ones found in homes, do not remove heavy metals, including lead.

17. (D) The American Heart Association recommends cholesterol screening for patients older than 2 years who have a parent or grandparent with documented premature cardiovascular disease (angina, MI, sudden cardiac death, cerebrovascular disease, coronary bypass, angioplasty, or peripheral vascular disease that occurred when younger than 55 years), a parent with documented hypercholesterolemia, or an unknown family history.

18. (A) Immediate testing should be performed on (1) contacts of anyone with confirmed or suspected TB, (2) children with radiographic or clinical findings of TB, (3) children immigrating from endemic countries (eg, Asia, Middle East, Africa, Latin America), and (4) children with travel histories to endemic countries and/or significant contact with indigenous peoples.

19. (C) By the age of 2 years, according to the Denver II, children should be able to kick a ball forward. The remaining choices are all within normal range of abilities for a 2-year-old.


SUGGESTED READING

Campbell C, Osterhoudt KC. Prevention of childhood lead poisoning. Curr Opin Pediatr. 2000;12:428-437.

Markowitz M. Lead poisoning. Pediatr Rev. 2000;21:327-335.

Wright RO, Tsaih S-W, Schwartz J, Wright RJ, Hu H. Association between iron deficiency and blood level in a longitudinal analysis of children followed in an urban primary care clinic. J Pediatr. 2003;142:9-14.

Wu AC, Lesperance L, Bernstein H. Screening for iron deficiency. Pediatr Rev. 2002;23:171-178.

CASE 67: A 2-YEAR-OLD WHO REFUSES TO WALK

A 2-year-old boy presents to the emergency department with a 1-day history of refusing to walk. Mom reports that she had first noticed him limping slightly 3-4 days ago, but he was otherwise well. He was not complaining of pain, but Mom noticed that when he stood, he did not bear weight on his right leg. On the day of admission she noticed that he refused to walk. She denies a history of bites or specific trauma, but she does admit that he is “always running into things.” He had a cough and congestion the previous week and has “felt warm” for 1-2 days but has otherwise been well. She denies any preceding sore throat or rash.

On examination the patient is fussy and uncooperative. His temperature is 101.3°F (38.5° C) rectally, HR 115, RR 22, and BP normal. He has no rashes or discolorations of his skin and is well hydrated. He refuses to ambulate. On musculoskeletal examination the patient has a tender, warm, and swollen right knee. There is mild erythema but no induration. He resists range of motion activities at the knee but has full range of motion and no tenderness on examination of the hip. Otherwise his examination is unremarkable.

SELECT THE ONE BEST ANSWER

1. All are appropriate choices for initial workup except

(A) bone scan

(B) hip and knee films

(C) CBC with differential

(D) ESR

(E) CRP

2. The plain films demonstrate soft tissue swelling surrounding the knee, but no findings consistent with fracture or osteomyelitis. The hip x-ray is normal. The CBC shows a leukocyte count of 18.4 × 103 with 28% bands; the ESR is 68 mm/hour, and the CRP is 94 mg/L. Which of the following is least likely in the differential at this point?

(A) osteomyelitis

(B) transient synovitis

(C) septic arthritis

(D) cellulitis

(E) postinfectious arthritis

3. What would be the best test/procedure to diagnose a septic joint?

(A) urine antigen detection test

(B) ultrasound

(C) CRP

(D) joint aspiration

(E) MRI

4. The patient’s ultrasound demonstrates no effusion, and osteomyelitis is now strongly suspected. What is the most likely causative organism morphology and biochemical reaction?

(A) gram-negative bacilli without lactose fermentation

(B) gram-positive cocci in chains

(C) gram-negative bacilli without sucrose fermentation

(D) gram-negative coccobacilli

(E) gram-positive cocci in clusters

5. Knowing the likely pathogens for osteomyelitis, in a region where MRSA isolates are common, what would be an appropriate empirical therapy?

(A) clindamycin

(B) penicillinase-resistant penicillin

(C) third-generation cephalosporin

(D) combination of A and C

(E) combination of B and C

6. Which of the following is the most specific method for confirmatory diagnosis of osteomyelitis?

(A) MRI

(B) conventional radiograph

(C) bone scan

(D) needle aspirate of the bone

(E) CT scan

7. Which of the following is the most common pathogenesis of osteomyelitis in children?

(A) contiguity

(B) direct invasion

(C) hematogenous spread

(D) unknown

(E) B and C are approximately equal

8. All of the following are complications of osteomyelitis except

(A) fracture

(B) septic joint

(C) subperiosteal abscess

(D) leg shortening

(E) no exceptions; all of the above are complications of osteomyelitis

9. Which of the following is the most common site of septic arthritis in children older than 1 year?

(A) ankle

(B) shoulder

(C) wrist

(D) knee

(E) hip

10. Which of the following is the cornerstone of the diagnosis of septic arthritis?

(A) MRI

(B) plain films

(C) blood culture

(D) presence of warmth at the affected joint

(E) arthrocentesis and fluid culture

11. A 13-year-old boy presents after spring break with a chief complaint of knee pain. He first noted the bilateral knee pain last summer, and it abated until recently. He denies playing any sports, but does perform in a hiphop dance group when on school vacations. He denies fever or any other symptoms. He is not sexually active and denies any trauma. On examination he has no trouble with ambulation, has no joint effusions, has full range of motion at the hip and knee, but complains of pain on palpation of the proximal tibia. What is the most appropriate next step in care?

(A) MRI of the knee

(B) plain films of the knee

(C) plain films of the hip

(D) arthroscopy

(E) reassurance and rest

12. What is the most common cause of septic arthritis?

(A) H influenzae

(B) S aureus

(C) E coli

(D) Salmonella species

(E) M pneumoniae

13. Which of the following is true regarding toddlers’ fractures?

(A) they result from major injury (eg, a fall from a great height)

(B) they are best seen on lateral radiographic view

(C) they occur in the distal third of the tibia

(D) they are best treated with rest and NSAIDs

(E) they are most commonly seen between 3 and 4 years of age

14. In which of the following patients are radiographs not indicated with a clinical history and examination that is consistent with Osgood-Schlatter disease?

(A) atypical complaints of pain

(B) erythema or warmth over the tibia

(C) pain not directly over the tubercle

(D) bilateral pain and symptoms

(E) all require imaging

15. What is the classic presentation of arthritis in a child with rheumatic fever?

(A) finger/joint involvement

(B) migratory among the large joints

(C) unilateral wrist

(D) bilateral ankle

(E) hip involvement

16. Which is true of the treatment of toxic synovitis?

(A) antibiotics are the mainstay of therapy

(B) aggressive physical therapy should be initiated early

(C) joint aspiration is diagnostic

(D) NSAIDs are the initial therapy

(E) more than 50% will recur with or without therapy

ANSWERS

1. (A) Although a bone scan is useful in diagnosis of osteomyelitis, it is not an optimal choice for initial investigation of a joint effusion. Plain films are not sensitive initially for osteomyelitis and rarely assist in the diagnosis of a septic joint, but they will help to rule out more concerning diagnoses such as malignancies and detect any underlying fractures. A CBC with differential, ESR, and CRP are helpful inflammatory markers for diagnosis and to follow progression of the illness.

2. (B) The most worrisome diagnosis at this point is septic arthritis. Reactive arthritis and periarticular cellulitis must still be considered but are less likely. However, transient synovitis is most common in the hip. It can be differentiated from septic arthritis by laboratory results. Septic arthritis is more likely to have an increased leukocyte count with a left shift and an increased ESR and CRP. With these laboratory results, transient synovitis is highly unlikely.

3. (D) Although ultrasound will reveal an effusion, the presence of an effusion does not definitively diagnose septic arthritis. Analysis of fluid obtained from an aspiration would demonstrate a leukocytic effusion, thus differentiating it from noninflammatory causes of joint effusion. Aspirated joint fluid might reveal bacteria on Gram stain or culture.

4. (E) Although all the choices are morphologies of known causes of osteomyelitis, the most common cause is S aureus, gram positive organisms in clusters. Salmonella is common among patients with sickle cell anemia, and Pseudomonas may be the cause following puncture wounds. The gramnegative coccobacillus Kingella kingae is an emerging cause of osteomyelitis in Israel, but it is far less common than S aureus.

5. (A) Although all the choices would provide some coverage for S aureus, it is important to cover the most important etiologies and resistant organisms well. In regions that have a high prevalence of MRSA, oxacillin, nafcillin, and related compounds would no longer be appropriate empirical choices. In such areas, clindamycin would be an appropriate agent. Ceftriaxone might be a reasonable addition if the patient has known or suspected sickle cell anemia because Salmonella osteomyelitis can occur in these patients as well as S aureus.

6. (D) Biopsy of the metaphysis of the affected bone is the most specific way to diagnose osteomyelitis, although it is not often performed. MRI and bone scan are 80-100% sensitive, but specificities can be lower than 50%. However, they are the preferential diagnostic imaging tools, especially in the absence of surgical aspiration, or to locate the area of inflammation. CT will probably not be helpful. Plain films can demonstrate evidence of osteomyelitis, but only after 10-14 days of infection.

7. (C) Hematogenous spread is the most common pathogenic mechanism in children. The vascularization of the metaphysis allows for bacterial seeding of the marrow cavity. Inflammation ensues and increases intraosseous pressure, which in turn hampers normal bone circulation. This can lead to necrosis and potential spread of infection to the epiphysis and joints. Direct invasion of bacteria into bone occurs in children but is most often secondary to trauma.

8. (E) All of these are potential complications of osteomyelitis. Septic arthritis can often result from contiguous spread of osteomyelitis. Hyperemia around the area of inflammation can lead to leg lengthening; proliferating cartilage can lead to leg shortening. Fractures can result from local destruction.

9. (D) Although all of the above joints may become infected, the most common site in older children (>1 year) is the knee. The hip is the most common site in younger infants.

10. (E) Although culture is key to identification of the causative organism, a blood culture is positive in no more than 50% of patients, and joint fluid culture is positive in 35-80% of patients. Fluid analysis is more immediately helpful in the diagnosis of septic arthritis: WBC higher than 100,000, depressed glucose levels, and increased lactic acid and lactate dehydrogenase (LDH) are the key findings. Although synovial fluid is essential to obtain, in extremely ill patients, treatment should not be delayed because one-third of joint cultures and nearly one-half of blood cultures remain positive even after initiation of antibiotics.

11. (E) The most likely diagnosis is Osgood-Schlatter disease (see Figure 67-1). With the patient’s history of recurrence during periods of increased activity, the best treatment for the patient is rest and NSAIDs as needed. A radiograph of the knee is reasonable if atypical clinical features are present.

art

FIGURE 67-1. Osgood-Schlatter disease. The radiograph would show characteristic fragmentation of the tibial tubercle apophysis, similar to the diagram. (Reproduced, with permission, from Skinner HB. Current Diagnosis & Treatment in Orthopedics, 4th ed. New York: McGraw-Hill; 2006: Fig. 11-29.)


12. (B) The leading cause of septic arthritis in children is S aureus. H influenzae type b was an important cause in infants and children before the introduction of immunization. SalmonellaE coli, and M pneumoniae rarely infect joints.

13. (C) Toddlers’ fractures are commonly seen between 9 months and 3 years as a result of minor falls, trips, or twists. The children present with inability to weight-bear and pain on dorsiflexion of the ankle. On internal oblique view, the fracture is most often noted in the distal third of the tibia. A fracture that is in the midshaft would be far more suggestive of abuse. Toddlers’ fractures should be casted for 5-6 weeks. This diagnosis should always be considered in a limping child of this age.

14. (D) Bilateral disease is commonly seen in Osgood-Schlatter and should not in and of itself prompt further imaging. The remaining choices are all suggestive of some other pathology and warrant further radiologic investigation.

15. (B) The classic presentation of rheumatic fever is that of a child with migratory polyarthritis in large joints. Although smaller joints (including wrist and hand) can be involved, they are more often one of many joints involved. Most patients have large joints involved first.

16. (D) Toxic synovitis, a diagnosis of exclusion, is best treated with rest and NSAIDs. Antibiotic therapy would be appropriate for septic arthritis diagnosed by aspiration but not for toxic synovitis.


SUGGESTED READING

Jung ST. Significance of laboratory and radiologic findings for differentiating between septic arthritis and transient synovitis of the hip. J Pediatr Orthop. 2003;23:368-372.

Kim MK, Karpas AK. The limping child. Clin Pediatr Emerg Med. 2002;3:129-137.

Miller ML, Cassidy JT. Postinfectious arthritis and related conditions. In: Behrman R, Kliegman R, Jenson H, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: WB Saunders; 2007.

CASE 68: A 2-YEAR-OLD WITH RECURRENT WHEEZING

A 2-year-old boy presents to your clinic for evaluation of respiratory difficulties. His mom reports he was in his usual state of health until yesterday when he began having shortness of breath, cough, and an occasional wheeze. Mom gave him 2 puffs of his brother’s albuterol inhaler, which initially provided some relief. Mom denies any preceding symptoms, such as rhinorrhea, fever, or other URI symptoms. Mom also denies smokers in the home, pets, or carpets, but she notes that the symptoms did start after playing next door with his 4-year-old neighbor. The patient has wheezed once before when he was 4 months old and was diagnosed with RSV.

On physical examination, the patient is in moderate respiratory distress. His oxygen saturation is 92% on RA, with a RR of 44. Other vital signs are normal. His lung examination is notable for diffuse expiratory wheezing with variably decreased breath sounds at the right base, obvious retractions, and nasal flaring. There is decreased air exchange and an inspiratory-toexpiratory ratio of 1:3.5. The rest of his examination is normal except for erythematous, dry, pruritic plaques on the flexor surface of his elbows.

SELECT THE ONE BEST ANSWER

1. What is the most likely cause of this patient’s respiratory distress?

(A) viral infection

(B) foreign body aspiration

(C) asthma

(D) bacterial pneumonia

(E) anaphylaxis

2. Your initial step in treatment in your office is

(A) supplemental oxygen

(B) oral steroids

(C) systemic steroids

(D) inhaled bronchodilator

(E) subcutaneous epinephrine

3. Metered-dose inhalers can be as effective as a nebulizer in delivering medication if used correctly

(A) True

(B) False

4. After 2 treatments, the patient improves, and you continue to obtain a history from Mom. Which topic would have no correlation with the prevalence of asthma?

(A) history of breastfeeding

(B) socioeconomic status

(C) suburban versus inner-city residence

(D) gender

(E) smoke exposure

5. Which of the following can be safely eaten by breastfeeding moms hoping to delay onset of foodassociated atopic dermatitis?

(A) milk

(B) fish

(C) eggs

(D) peanuts

(E) none of the above

6. The mom reports that her son has excessive cough with viral infections. He gets short of breath and coughs when running around almost every day. He also often coughs at night (waking up 1-2 times per night). You diagnose what type of asthma?

(A) mild intermittent

(B) mild persistent

(C) moderate persistent

(D) moderate intermittent

(E) severe persistent

7. You are concerned by the patient’s respiratory status and obtain a chest radiograph. It demonstrates hyperinflation with peribronchial cuffing and a right upper lobe volume loss. He is afebrile. What is the next appropriate step?

(A) chest tube

(B) ceftriaxone IM

(C) azithromycin PO

(D) bronchoscopy

(E) no procedure or antibiotic

8. What is the best treatment for discharge from the outpatient facility?

(A) inhaled bronchodilator daily

(B) inhaled bronchodilator as needed (PRN)

(C) inhaled bronchodilator PRN and oral leukotriene inhibitor daily

(D) inhaled steroid daily and oral leukotriene inhibitor daily

(E) inhaled bronchodilator PRN and daily inhaled steroid

9. All of the following are true regarding asthma education except

(A) asthma action plans are warranted and efficacious

(B) peak flow monitoring and/or symptom monitoring are not reliable methods of self-management

(C) asthma action plans must be reviewed and revised by the physician

(D) all action plans should include contacts for urgent care

(E) skill sets to be taught include inhaler use, selfmonitoring, and environmental control

10. What are the long-term adverse effects of daily use of inhaled low to medium doses of corticosteroids?

(A) suppression of the hypothalamic-pituitary axis

(B) irreversible linear growth reduction

(C) decreased bone density

(D) cataracts

(E) none of the above

11. Which of the following patients would be classified with mild intermittent asthma?

(A) symptoms 2-3 times per week, rare night symptoms, and 90% forced expiratory volume in the first second of expiration (FEV1)

(B) symptoms daily, night symptoms 1-2 times per week, and 75% FEV1

(C) symptoms once per week, night symptoms less than 2 times per month, and 85% FEV1

(D) symptoms daily, night symptoms nightly, and less than 60% FEV1

(E) none of the above

12. Which of the following is not known to exacerbate asthma symptoms?

(A) weather changes

(B) mite exposure

(C) beta-agonist drugs

(D) aspirin

(E) smog

13. Which of the following statements is false regarding a child’s risk of having an atopic disease based on family history?

(A) 29% chance of an atopic disease if a sibling has an atopic disease

(B) 50% chance of an atopic disease if one parent has an atopic disease

(C) 72% chance of the same atopic disease as the parents if the parents share a common atopic disease

(D) 13% chance of atopic disease if neither parent has an atopic disease

(E) none of the above

14. Which of the following statements is true regarding atopic diseases?

(A) more than 50% of atopic dermatitis cases do not present until after 2 years of age

(B) allergic rhinitis usually precedes asthma and atopic dermatitis

(C) atopic dermatitis is the most common atopic disease

(D) hot and humid climates are risk factors for atopic dermatitis

(E) allergic rhinitis prevalence is increasing most in 2- to 5-year-olds

15. Which of the following contribute to or exacerbate asthma or allergies?

(A) maternal tobacco smoking

(B) indoor pets

(C) dust mites

(D) B and C

(E) A, B, and C

16. All of the following are recommended for prolonged dust mite exposure reduction except

(A) encase mattress and pillow in an allergenimpermeable cover

(B) wash sheets in water hotter than 130°F (54.4°C) weekly

(C) decrease indoor humidity

(D) remove bedroom carpet

(E) use a chemical cidal agent such as benzyl benzoate

17. The underlying pathology of asthma is characterized by inflammation of the airway. This inflammation is mediated by many factors, one of which is major basic protein. What is the source of major basic protein?

(A) mast cells

(B) IgE receptors

(C) neutrophils

(D) interleukin (IL)-2

(E) eosinophils

18. What percentage of asthmatic children will outgrow symptoms by adulthood?

(A) 10%

(B) 20%

(C) 30%

(D) 40%

(E) 50%

ANSWERS

1. (C) The combination of a history of RSV, current wheezing, and a family history of asthma suggests the most likely diagnosis is asthma. Although foreign body is a consideration for this patient with his history of playing with a neighbor his age, the diffuse symptoms on physical examination do not support such a diagnosis. Viral infection and bacterial pneumonia are not likely considering his lack of associated symptoms, and anaphylaxis is not supported by the vital signs (which would demonstrate hypotension and laryngeal edema along with bronchospasm).

2. (D) The first-line therapy for an acute asthma exacerbation is an inhaled bronchodilator (such as albuterol). Oral steroids are often used in conjunction with inhaled steroids to decrease the airway edema, but they have a much slower onset of action. Supplemental oxygen is a good supportive measure but will not treat the underlying pathology.

3. (A) Studies have demonstrated that when used properly, both the nebulizer and inhaler with spacer are equally efficacious in the treatment of acute asthma.

4. (A) Although breastfeeding in the first year of life in combination with certain food avoidance can delay the onset of atopic disease, this is mostly with respect to food-associated atopic dermatitis. There is no change in the incidence or age of onset of asthma or incidence of allergic rhinitis. Male gender, low socioeconomic status, inner-city residence, and smoke exposure (both secondhand and maternal smoking) are all risk factors for asthma.

5. (E) All of the foods should be avoided by any breastfeeding mom with a strong family history of food-associated atopic dermatitis, urticaria, or GI disease. Although such avoidances will decrease the incidence of such illness at 1 year, there is no such decrease at 2 years of age.

6. (C) Based on the history of night symptoms greater than once a week and possible daily symptoms, this patient would be considered to have moderate persistent asthma. For more information on classification and treatment of asthma, see http://www.nhlbi.nih.gov/guidelines/asthma.

7. (E) The patient has all the symptoms of an asthma exacerbation with a chest radiograph that is consistent with atelectasis. This requires no additional treatment beyond the treatment for the asthma exacerbation. The use of antibiotics in asthma is reserved for the occurrence of comorbid infections such as sinusitis or bacterial pneumonia.

8. (E) Current asthma guidelines recommend a controller medication consisting of an inhaled steroid with a rescue medicine of an inhaled bronchodilator for moderate-persistent asthma. Although leukotriene inhibitors with steroids are recommended as an alternative therapy for moderate-persistent asthma, bronchodilators with inhaled steroid are first-line therapeutic agents.

9. (B) It is the opinion of the National Asthma Education and Prevention Program (NAEPP) that asthma education includes the development of an action plan. Key to this plan is the ability to self-assess and manage symptoms and medications. Peak flow measurements and symptom monitoring, individually or in combination, are methods toward this end, and they are recommended for all moderate to severe asthmatics.

10. (E) Current studies have demonstrated that low to medium doses of inhaled corticosteroids have no significant adverse effects. Although some studies suggest an association of reduction in linear bone growth with long-term inhaled steroids, this effect is likely nonprogressive and reversible.

11. (C) Patients with mild-intermittent asthmatics have symptoms during the day less than 2 times per week with nighttime symptoms twice per week or less, use their albuterol inhaler 2 times per week or less, and have no interference with their regular activity. Pulmonary functions tests (PFTs) show FEVmore than 80% of predicted and normal FEVbetween exacerbations.

12. (C) Beta-agonist drugs are part of the treatment plan for asthma. Conversely, beta-adrenergic blocking drugs are known to trigger asthma exacerbations. Weather changes, allergen exposures such as mites, aspirin, and smog can all exacerbate asthma as well.

13. (B) There is a 50% chance of atopic disease in a child born to parents who both have an atopic disease. The percentage if one parent has an atopic disease is lower. Those odds increase to 72% if the parents have the same disease. Although 20-30% of the population has an atopic disease, a family history is a strong predictor of a patient’s risk, although environmental influences also play a strong role. Also, when a patient has one atopic disease, he or she has a threefold increase in risk for another atopic disease.

14. (D) Both hot and humid and cold and dry climates are risk factors for atopic dermatitis. Atopic dermatitis presents by 2 years of age in about 90% of patients. Allergic rhinitis, the most common atopic disease, is normally preceded by asthma and/or atopic dermatitis, and it is increasing in prevalence in 8- to 13-year-olds.

art

FIGURE 68-1. Eosinophils. Eosinophils are about the same size as neutrophils but have bilobed nuclei and abundant coarse cytoplasmic granules. The cytoplasm is often filled with brightly eosinophilic specific granules, but also includes some azurophilic granules. A. The micrograph shows an eosinophil next to a neutrophil for comparison of its nucleus and granules. X1500. Wright. B. Even with granules filling the cytoplasm, the two nuclear lobes of eosinophils are usually clear. X1500. Giemsa. C. TEM of a sectioned eosinophil clearly shows the unique specific granules, as oval structures with disk-shaped electron-dense crystalline cores (EG). These along with lysosomes and a few mitochondria (M) fill the cytoplasm around the bilobed nucleus (N). X20,000. (Reproduced, with permission, from Mescher AL. Junquiera’s Basic Histology: Text and Atlas, 12th ed. New York: McGraw-Hill; Fig. 12-9.)


15. (E)

16. (E) Although cidal agents such as benzyl benzoate kill the mites and denature the antigens, the effects are not maintained for prolonged periods, nor are the effects largely significant. These methods are not recommended routinely. The other answer choices are all recommended and proven to be effective in consistently reducing the exposure to dust mites.

17. (E) Although all the choices are involved in the pathology of asthma, it is the eosinophil that is the source of major basic protein that injures airway epithelium and enhances bronchial responsiveness (see Figure 68-1).

18. (E) At least half of all asthmatic children outgrow symptoms by adulthood, although those with severe asthma or multiple allergies are far less likely to do so.


SUGGESTED READING

The NAEPP Expert Panel Report Guidelines for the Diagnosis and Management of Asthma—Update on Selected Topics 2007. NIH Publication No. 97-4051. Washington, DC: National Institutes of Health. National Heart Lung and Blood Institute Web site. http://www.nhlbi.nih.gov/guidelines/asthma/index.htm. Accessed June 2010.

Lemanske RF Jr, Busse WW. Asthma. J Allergy Clin Immunol. 2003;111(2 suppl):s502-519.

Nimmagadda SR, Evans RE 3rd. Allergy: etiology and epidemiology. Pediatr Rev. 1999;20:111-115.

CASE 69: A 5-YEAR-OLD WITH HIVES AFTER 10 DAYS OF ANTIBIOTICS

The mother of a 5-year-old boy calls one summer night to ask about her son’s hives that developed earlier in the evening. The rash is described as red splotches with central clearing. Mom reports that he is itching quite a bit but is breathing well and has no lip involvement. There is no joint pain or abdominal pain. He had a URI 10 days ago and was started on penicillin at a local emergency department yesterday for a “throat infection.” Mom does report that he has been playing outside a lot and has been “getting eaten by bugs.” His diet has not changed significantly, and contains no seafood or peanuts, but he has been drinking a lot of fruit punch. Otherwise he has been doing well with no vomiting, diarrhea, or change in activity or appetite.

SELECT THE ONE BEST ANSWER

1. Which of the following is the most likely cause of the patient’s rash?

(A) type 1 immediate allergic reaction

(B) type 1 accelerated allergic reaction

(C) type 2 allergic reaction

(D) type 3 allergic reaction

(E) type 4 allergic reaction

2. The single most important intervention for this patient is

(A) discontinuation of the antibiotic

(B) antihistamines

(C) topical steroids

(D) topical antipruritics

(E) none of the above

3. True or False. An immediate (<24-hour) reaction to penicillin is most likely IgE-mediated, whereas a reaction occurring later than 24 hours is probably not.

(A) True

(B) False

4. You decide to test this patient for a penicillin allergy. Which of the following is the most appropriate method for nonemergently diagnosing a penicillin allergy?

(A) penicillin challenge in the intensive care unit (ICU)

(B) skin testing

(C) radioallergosorbent test (RAST)

(D) CAP-RAST testing

(E) none of the above

5. Regarding skin testing for penicillin allergy, which of the following is false?

(A) negative skin tests occur in most of those with a clinical history

(B) positive skin tests occur in 4% of patients without a clinical history

(C) patients may be tested once off prescription antihistamines for 48 hours

(D) the risk of severe reaction during skin testing is less than 1%

(E) skin testing is more sensitive than RAST testing

6. Which of the following is not a contraindication to penicillin skin testing?

(A) presence of a diffuse rash

(B) use of diphenhydramine the previous day

(C) absence of positive skin controls

(D) history of Stevens-Johnson after penicillin exposure

(E) history of moderate allergic reaction

7. Which of the following is true regarding RAST testing?

(A) it provides immediate results

(B) the presence of a rash limits its use

(C) it is not affected by steroid use

(D) it has a mild risk of allergic reaction

(E) it can be used with a broad selection of antigens

8. Besides drug exposure, all of the following are well-known causes of urticarial reaction except

(A) artificial flavoring/coloring

(B) vancomycin

(C) latex

(D) radiocontrast media

(E) all of the above can cause urticaria; no exception

9. If the boy mentioned in the vignette was having bronchospasm, hypotension, and airway edema, what is the initial treatment of choice?

(A) epinephrine IV (1:1000 dilution)

(B) epinephrine subcutaneous (SC) (1:1000 dilution)

(C) epinephrine IV (1:10000 dilution)

(D) diphenhydramine IV

(E) hydrocortisone IV

10. Which of the following is not a known etiology of anaphylaxis?

(A) milk ingestion

(B) radiographic contrast media

(C) hymenoptera stings

(D) siphonaptera bites

(E) exercise

11. All of the following are true regarding the risk for anaphylaxis except

(A) reactions to food occur more frequently among children than adults

(B) atopic children have a higher risk of reactions to drugs than the general population

(C) asthmatic children have a higher risk of reaction to food than the general population

(D) males have a higher risk of reaction to hymenoptera stings than females

(E) a recent exposure to the offending allergen increases the risk of reaction

12. Which of the following does not support the diagnosis of anaphylaxis?

(A) laryngeal edema, bronchospasm, hypotension

(B) elevated plasma histamine levels

(C) demonstration of immediate hypersensitivity by skin test/RAST

(D) lowered plasma tryptase levels

(E) all of the above are supportive

13. What is the risk of recurrent anaphylaxis after an episode of anaphylaxis associated with an insect sting?

(A) 20%

(B) 40%

(C) 60%

(D) 80%

(E) 100%

14. True or False: Large local reactions to insect stings are IgE mediated (allergic)

(A) True

(B) False

15. Which of the following is true regarding venom skin testing?

(A) it is available only in honeybee and yellow jacket preparations

(B) it indicates reactivity of the patient

(C) testing should be delayed for at least 2 weeks after a reaction from the sting

(D) antihistamines can interfere with results

(E) a negative test excludes the presence of venomspecific IgE antibodies

16. Which of the following is true regarding radiocontrast media reaction?

(A) the etiology is unknown but appears to be IgEmediated

(B) allergic and asthmatic patients have an increased incidence

(C) reactions are immediate in all cases

(D) anaphylaxis is the most common reaction

(E) approximately 80% experience vasomotor reactions

ANSWERS

1. (B) This clinical description is most consistent with accelerated hypersensitivity in the form of urticaria. The proposed mechanism of such a reaction is a delayed type 1 hypersensitivity reaction. Although antibiotics can also cause a type 4 hypersensitivity reaction, the reaction would appear more as a morbilliform, erythematous, pruritic rash with possible associated fever.

2. (A) The primary therapy is to discontinue the offending agent. The remaining therapies can all be helpful depending on the extent and severity of the reaction.

3. (A) True

4. (B) Skin testing is said to be a reliable method for documenting a true penicillin allergy but in practice is seldom performed. Benzylpenicilloyl poly-llysine, the major determinant, is formed as a result of the beta-lactam ring opening. This major determinant is generally responsible for the urticarial reactions, whereas the minor determinants predict anaphylaxis more consistently but are not currently available commercially. Penicillin is the only antibiotic for which skin testing is available.

5. (C) Patients who are to undergo skin testing must have ceased taking short-acting antihistamines such as diphenhydramine for at least 48 hours before testing. Generally, medium-acting antihistamines should be stopped for 5 days, and longer-acting antihistamines for 6 weeks. Skin testing may also be clinically affected by long-term corticosteroids, topical steroids, and tricyclic antidepressants.

6. (E) Although skin testing is contraindicated in those with severe allergic reaction, a moderate reaction does not limit the use. Those with severe reactions should either be given an alternative drug choice, or if none is available, desensitization without skin testing should be considered.

7. (C) RAST is an in vitro option for people who cannot discontinue use of antihistamines or other medications or have conditions such as a rash that interfere with skin testing. It is also useful in those for whom anaphylaxis is a distinct risk. Its disadvantages include the delay in results and the limited specificity to clinically significant antigens.

8. (E) All of the substances listed can cause urticaria. Although rapid vancomycin infusion is known to cause red man syndrome (a histamine release syndrome associated with rash and occasionally hypotension), it can have accompanying urticaria.

Latex is being seen more frequently as a cause of allergic reaction, including urticaria and anaphylaxis (see Figure 69-1).

art

FIGURE 69-1. Red Man Syndrome. Facial and neck flushing are manifestations that may be seen with red man syndrome from intravenous vancomycin infusion. (Reproduced, with permission, from Knoop KJ, Stack LB, Storrow AS, et al. Atlas of Emergency Medicine, 3rd ed. New York: McGraw-Hill; 2010:576. Photo contributor: R. Jason Thurman, MD.)


9. (C) The treatment of choice for severe anaphylaxis, as demonstrated by the symptoms above, is IV epinephrine in 1:10000 dilution, administered every 5-10 minutes while on a cardiac monitor. Initially IM administration of a 1:1000 dilution may be a more available option and should be used when an IV is not yet in place. Hydrocortisone and diphenhydramine are both adjunctive therapies.

10. (D) Siphonaptera, or flea bites, are not a known cause of anaphylaxis, although they can cause a significant local reaction. All the remaining items have been known to cause anaphylaxis, including exercise-induced anaphylaxis.

11. (B) Although atopic children do have a greater severity of reaction to drugs, they do not have an increased risk of reaction compared with the general public. Males have a higher risk of reaction to stings most likely as a function of their greater exposure to outdoor activities.

12. (D) Plasma tryptase levels are elevated in anaphylaxis because tryptase is a protease specific to mast cells. Similarly, histamine is released during mast cell activation.

13. (C)

14. (B) False. Large local reactions to insect stings are not IgE mediated and therefore not allergic in origin. However, once sensitized, a repeat sting may cause symptoms that can progress to IgE release and systemic symptoms.

15. (D) Venom skin testing is available in 5 preparations, including honeybee, yellow jacket, yellow hornet, white-faced hornet, and wasp. The testing can only determine prior exposure, not reactivity. A positive test with a strong clinical history is predictive, although a negative test does not exclude the possibility in such a patient. Testing should be delayed for 6 weeks after a reaction. Antihistamines interfere with penicillin skin testing.

16. (B) Despite the noted increased incidence of radiocontrast media reactions in allergic and asthmatic patients, recent studies have demonstrated a probably non–IgE-mediated-mechanism. Instead, these reactions are perceived to be mast-cell-mediated. Nonetheless, approximately 5-8% of patients experience some type of immediate vasomotor response, with a small portion having delayed reactions. Urticaria is the most common reaction.


SUGGESTED READING

Hay WW Jr, Hayward AR, Levin MJ, et al. Current Pediatric Diagnosis and Treatment. New York, NY: McGraw-Hill; 2008.

Lasley MV, Shapiro GG. Testing for allergy. Pediatr Rev. 2000;21:39-43.

Weiss ME, Adikinson NF. Immediate hypersensitivity reactions to penicillin and related antibiotics. Clin Allergy. 1988;18: 515-540.

Winberry SL, Liberman PL. Anaphylaxis. Immunol Allergy Clin North Am. 1995;15:477.

CASE 70: A 3-MONTH-OLD WITH POOR WEIGHT GAIN, RECURRENT INFECTION, AND DIARRHEA

A 3-month-old male infant has been brought to your clinic for evaluation of diarrhea. Mom reports that he has had diarrhea “since birth” and has seen at least 3 doctors over the course of 3 months. Each doctor switched the patient’s formula, initially from breast milk through multiple types of standard cow’s milk and soy formula followed by elemental formula, all with no relief. The stools are described as nonbloody, runny, and occur about 7 times per day. Mom denies any emesis or difficulty with feeds (no cyanosis, diaphoresis, arching, or discomfort). On review of systems she does note that he has had several episodes of cough and respiratory distress requiring antibiotics, as well as recurrent thrush. The birth history is unremarkable except for a lack of prenatal care. The birthweight was 3.6 kg, length 50 cm, head circumference (HC) 35.5 cm. Both mom and infant were discharged on postnatal day 2.

The physical examination is noteworthy for a small child who weighs 4.0 kg. The length is 55 cm and the HC is 39.5 cm. He is alert and active. His oral cavity has white plaques but no vesicles or other lesions. The cardiac examination has a regular rate and rhythm, no murmurs, and good peripheral pulses. The chest examination reveals coarse breath sounds with intermittent crackles at left base. The abdominal examination reveals a liver edge approximately 2 cm below the costal margin, with a spleen edge palpable 1.5 cm below the costal margin. There is no lymphadenopathy. Otherwise the physical examination is unremarkable.

SELECT THE ONE BEST ANSWER

1. What is the most likely cause of the patient’s symptoms?

(A) B-cell deficiency

(B) T-cell deficiency

(C) phagocyte deficiency

(D) abnormal mucociliary clearance

(E) lactase deficiency

2. Which of the following would not be included in the initial workup of this patient?

(A) Western blot for HIV

(B) CBC with differential

(C) quantitative immunoglobulins

(D) lymphocyte subsets

(E) stool culture

3. The results of the initial testing reveal the following:

HIV enzyme-linked immunosorbent assay

(ELISA): nonreactive

Absolute lymphocyte count: 2200/mm3

CD3T cells: 15%

Immunoglobulins: Normal

HIV DNA PCR: nonreactive

Which of the following is the most likely diagnosis with this information?

(A) DiGeorge syndrome

(B) Wiskott-Aldrich syndrome

(C) congenital HIV

(D) severe combined immunodeficiency (SCID)

(E) chronic mucocutaneous candidiasis

4. Which of the following tests can confirm the definitive diagnosis of SCID?

(A) search for a JAK3 mutation

(B) search for a RAG1 or RAG2 mutation

(C) search for a IL-7Rα mutation

(D) less than 2% activity in ADA control

(E) all of the above

5. Which of the following is the best treatment for the above patient?

(A) routine IVIG transfusions

(B) HLA-identical stem cell transplantation

(C) haploidentical donor stem cell transplantation

(D) gene therapy

(E) none of the above

6. Which of the following lymphocyte subset markers reflect B cells?

(A) CD3

(B) CD4

(C) CD14

(D) CD16

(E) CD20

7. Which of the following is the most common immunodeficiency?

(A) hyper IgM syndrome

(B) CF

(C) IgA deficiency

(D) common variable immunodeficiency

(E) cyclic neutropenia

8. A defect in which of the following would most likely account for a patient with recurrent staphylococcal infections, partial albinism, and long-term risk of hemophagocytosis?

(A) leukocyte adhesion

(B) lysosomal transport

(C) phagocyte oxidase

(D) elastase

(E) none of the above

9. A 6-year-old boy comes to the emergency department with fever, cough, and chest pain. While there he is noted to have hemoptysis and is saturating 87% on RA. A chest radiograph reveals bilateral lower lobe infiltrates. He is admitted and begun on ceftriaxone and vancomycin. With no improvement after 36 hours and an unchanged chest radiograph, bronchoscopy reveals large mucus plugs and positive culture for Aspergillus nidulans. Which of the following immunodeficiencies would most likely underlie this illness?

(A) Wiskott-Aldrich

(B) leukocyte adhesion deficiency (LAD) type 1

(C) common variable immunodeficiency

(D) chronic granulomatous disease

(E) Kostmann syndrome

10. Which of the following bacterial etiologies commonly cause infections among patients with chronic granulomatous disease?

(A) S pneumoniae

(B) Klebsiella spp.

(C) S aureus

(D) A and C

(E) B and C

11. The genetic defect in chronic granulomatous disease results in abnormal function of what chemical reactant?

(A) leukocyte integrins

(B) myeloperoxidase

(C) NADPH-oxidase

(D) adenosine deaminase

(E) LYST protein

12. Which of the following is suggestive of chronic granulomatous disease (CGD)?

(A) positive Rebuck skin window

(B) abnormal neutrophil flow cytometry

(C) abnormal nitroblue tetrazolium test

(D) abnormal giant granules in neutrophils

(E) none of the above

13. Treatment strategies for chronic granulomatous disease include all except

(A) trimethoprim-sulfamethoxazole prophylaxis

(B) interferon (IFN)-gamma

(C) aggressive bacterial species-specific antibiotic therapy

(D) gene therapy

(E) A and C

14. Which of the following is true regarding cutaneous delayed-type hypersensitivity testing?

(A) it is reliable after the age of 6 months

(B) it employs tetanus toxoid, rubella antigen, and Candida antigens

(C) it is a definitive diagnostic tool

(D) a positive test is considered more than 5 mm induration in all cases

(E) a positive test reflects abnormal cellular immunity

15. Which of the following is not true regarding humoral immune deficiencies?

(A) presentation is usually after 6 months of life

(B) sinopulmonary infections are common

(C) H influenzae is a common pathogen

(D) bone marrow transplant is the mainstay of therapy

(E) hypogammaglobulinemia is a hallmark

16. Which of the following viruses is not inactivated during IVIG preparation?

(A) hepatitis A

(B) hepatitis B

(C) hepatitis C

(D) HIV

(E) herpes viruses

17. How often should IVIG be given for humoral immunodeficiencies?

(A) every 1-2 weeks

(B) every 3-4 weeks

(C) every 1-2 months

(D) every 3-4 months

(E) weekly

18. Patients with which humoral immune disorder cannot receive standard IVIG?

(A) common variable immunodeficiency

(B) Bruton gamma-globulinemia

(C) selective IgA deficiency

(D) IgG subclass deficiency

(E) none of the above

ANSWERS

1. (B) The most likely etiology for this patient’s symptoms is a defect in T-cell function and/or number. Isolated B-cell deficiencies do not normally present until after 6 months when maternal antibody wanes. Phagocyte dysfunction syndromes do not present with recurrent pulmonary infection or failure to thrive (FTT). A combined immunodeficiency could present like this, but at this age, the T-cell deficiency would be the primary driver of clinical symptoms. Although lactase deficiency could explain the diarrhea, it would not explain the recurrent infections. Abnormal mucociliary clearance, as occurs in CF, could present with diarrhea, FTT, and respiratory symptoms but is less likely to cause thrush; there is an associated lack of lymphoid tissue.

2. (A) Initial evaluation for this patient should be directed at uncovering an underlying immune deficiency. Although HIV should be considered, the Western blot should not be used as a screening test; a DNA PCR would be the appropriate initial laboratory evaluation in this age group. Infant or maternal HIV ELISA testing may also provide useful information but neither would be diagnostic in the infant. A CBC with differential, quantitative immunoglobulins and lymphocyte subsets would all be useful. A stool culture would screen for bacterial pathogens causing the diarrhea.

3. (D) Severe combined immunodeficiency best explains the symptoms and laboratory findings of this vignette. Although congenital HIV can present in this way, most HIV-positive children have a positive DNA PCR by 2-3 months. Patients with Wiskott-Aldrich syndrome present with recurrent pneumonia, thrombocytopenia, and eczema, the last of which this patient does not have. DiGeorge syndrome normally has dysmorphisms, hypocalcemia, and the patient is usually only mildly lymphopenic. The laboratory results do not support chronic mucocutaneous candidiasis.

4. (E) SCID is a heterogenous group of disorders that arise from an abnormality in the function of T cells and B cells. All of the answers are distinct genetic types of SCID, and for diagnosis, an attempt at identifying the specific genetic defect must be made; the molecular diagnosis is unknown in 14% of cases. All genetic defects leading to SCID are autosomal recessive except for one, IL2RG, which is X-linked.

5. (B) Although both HLA-identical and haploidentical donor stem cell transplantation are used in SCID, HLA-identical has the lowest risk of graft-versushost disease (GVHD) and a more favorable prognosis. Haploidentical donor stem cell transplantation does not have as high a success rate, but is used if necessary. IVIG is used in B-cell disorders. Although there is a promising future in gene therapy for some forms of SCID, it is currently not an approved approach to care. Without a stem cell transplantation, most patients with SCID will die before their first birthday; with transplantation before 3.5 months, the chance of survival increases to 97%.

6. (E) Lymphocytes are represented by CD3, CD4, and CD8 markers among others. Natural killer cells are represented by CD16, CD56, and CD57 markers. Monocytes are represented by CD14. B cells are represented by CD19 and CD20.

7. (C) IgA deficiency occurs in about 1 in 333 people; 1 in 700 U.S. whites are estimated to have IgA deficiency. Most are asymptomatic.

8. (B) The described patient has Chediak-Higashi, a disorder caused by mutation in the lysosomal transport protein. Affected individuals have recurrent skin and respiratory pyogenic infections, partial oculocutaneous albinism, and neurologic disturbances (neuropathy, photophobia, seizures). Patients who survive the infections eventually enter the “accelerated phase” of lymphocytic infiltration of most organ systems, leading to death. Laboratory findings include mild decrease in neutrophil counts with giant cytoplasmic granules.

9. (D) Invasive and pulmonary aspergillosis are commonly seen in several immunodeficiencies, with Aspergillus nidulans representing a species particularly virulent in chronic granulomatous disease. Kostmann syndrome, or congenital neutropenia, would have presented by now with recurrent infections and fever. Wiskott-Aldrich is an X-linked disorder that involves eczema, thrombocytopenia, and immunodeficiency. Common variable immunodeficiency, a B-cell disorder, would have presented after 6 months of age with encapsulated organism infections. Although LAD is also a disorder of phagocytes, it is not as commonly associated with aspergillosis.

10. (E) Patients with chronic granulomatous disease are susceptible to catalase-producing organisms. These organisms include, among others, StaphylococcusBurkholderiaAspergillusSerratia, and Klebsiella species. S pneumoniae, however, is catalase negative.

11. (C) Chronic granulomatous disease results from a genetic defect in one component of the NADPHoxidase system in phagocytes (see Figure 70-1). These defects limit the enzyme activity that allows phagocytes to produce superoxide, which forms other reactive oxidants. This leads to an inability to defend against catalase-producing organisms.

12. (C) The initial screening test for CGD is the nitroblue tetrazolium (NBT) test. In this study, normal neutrophils produce superoxide that is reduced by NBT and create a dark blue coloration to cells. When the superoxide is absent or low, an abnormally low number of cells are altered to blue color. This test can then be confirmed by other more quantitative tests, such as a cytochrome reduction assay.

13. (E) Although all of the above are possible treatments for CGD, gene therapy is currently only theoretical and studies are ongoing to test their efficacy and stability.

14. (D) Cutaneous delayed-type hypersensitivity testing is an in vivo test for cellular immunity. A positive test (>5-mm response in all subjects) reflects intact cellular immunity. The test uses intradermal injections of tetanus toxoid, mumps antigen, and Candida antigen. All negative tests should be either repeated or followed by flow cytometry and in vitro assays of T-cell function; age younger than 1 year and concurrent infection can limit the reliability of results.

15. (D) The mainstay of therapy for humoral deficiencies is IVIG replacement therapy.

16. (A) The incubation of fractionated immune globulin during the manufacturing of IVIG results in the inactivation of enveloped viruses (eg, HIV, hepatitis B, hepatitis C, herpes viruses). It does not, however, inactivate nonenveloped viruses, which include parvovirus and hepatitis A.

art

FIGURE 70-1. Pathogenesis of chronic granulomatous disease. Schematic diagram of the manner in which the metabolic deficiency of the CGD neutrophil predisposes the host to infection. Normal neutrophils accumulate hydrogen peroxide in the phagosome containing ingested Escherichia coli. Myeloperoxidase is delivered to the phagosome by degranulation, as indicated by the closed circles. In this setting, hydrogen peroxide acts as a substrate for myeloperoxidase to oxidize halide to hypochlorous acid and chloramines, which kill the microbes. The quantity of hydrogen peroxide produced by normal neutrophils is sufficient to exceed the capacity of catalase, a hydrogen peroxide-catabolizing enzyme of many aerobic mieroorganisms, including most gram negative enteric bacteria, S aureus, C albicans, and Aspergillus species. When organisms such as E coli gain entry into the CGD neutrophils, they are not exposed to hydrogen peroxide because the neutrophils do not produce it, and the hydrogen peroxide generated by microbes themselves is destroyed by their own catalase. When CGD neutrophils ingest streptococci or pneumococci, the organisms generate enough hydrogen peroxide to result in a microbicidal effect. On the other hand, as indicated in the middle figure, catalase-positive microbes, such as E coli, can survive within the phagosome of the CGD neutrophil. (Reproduced, with permission, from Lichtman MA, Beutler E, Kipps TJ, et al. Williams Hematology, 7th ed. New York: McGraw-Hill; 2006: Fig 66-8.)


17. (B) IVIG is given every 3-4 weeks, based on its half-life, which is 21-28 days.

18. (C) Anaphylaxis may occur in patients with selective IgA deficiency as a result of anti-IgA antibodies reacting with trace IgA in the IVIG. For this reason, IgA-deficient patients should receive IVIG with lower IgA content.


SUGGESTED READING

Bonilla FA, Geha RS. Primary immunodeficiency diseases. J Allergy Clin Immunol. 2003;111(2 suppl):S571-S578.

Buckley RH. Primary cellular immunodeficiencies. J Allergy Clin Immunol. 2002;109:747-757.

Schubal SJ. Treatment of antibody deficiency syndromes. Pediatr Rev. 2000;21:358-359.

Winkelstein JA, Marino MC, Johnston RB Jr, et al. Chronic granulomatous disease. Report on a national registry of 368 patients. Medicine (Baltimore). 2000;79:155-169.