NORMAL SKELETAL MATURATION
The growth plate in the newborn is generally not constituted as an effective structure until 12–24 months.
The metaphysis is the most metabolically active area.
Children with sickle cell disease are prone to Salmonella osteomyelitis (but remember, the most common cause even in these children is Staphylococcus aureus).
PEDIATRIC SKELETON
The anatomy, biomechanics, and physiology of the child’s skeleton are very different when compared to adults, differences in fracture pattern, diagnostic problems, and treatment regimens.
Bone is more porous and elastic.
The most obvious anatomic differences in the pediatric bones are the presence of growth plates and the thick periosteum.
The physis (growth plate) is the weakest site in a child’s bone.
A thick periosteal sleeve makes fractures more stable.
Remodeling capabilities and rapid healing.
In addition to S aureus, young infants may develop osteomyelitis caused by Streptococcus agalactiae or enteric gram-negative bacteria.
OSTEOMYELITIS
A previously ambulatory 18-month-old girl refuses to walk. She has marked tenderness over the distal left femur. The child has a temperature of 101.6°F (38.7°C), erythrocyte sedimentation rate (ESR) of 72 mm/hr, and white blood cell count (WBC) of 18.5. Radiographs reveal no bony abnormalities. Think: Osteomyelitis.
The initial signs and symptoms are often nonspecific. Refusal to walk, limping, or reluctance to move the affected extremity is common presentation. Fever is usually present. Focal tenderness over a long bone may be an important clue for diagnosis. The WBC count is usually and ESR is elevated. Initial plain radiograph may be normal or show only soft tissue swelling. Radionuclide bone scans usually are positive within 48–72 hr of onset of illness. Bone aspiration may reveal an etiologic agent.
Consider Kingella kingae in children who attend day care. (Remember, K kingae is a fastidious organism found in normal respiratory flora.)
DEFINITION
Inflammation of the bone caused by infection.
Can be acute (< 2 weeks) or chronic.
Cultures for K kingae may need to be incubated longer than usual laboratory protocol.
EPIDEMIOLOGY
Preschool-age children (50%).
Male preponderance.
More common in African-American children.
Puncture wounds to the foot may result in osteomyelitis caused by mixed flora, including Pseudomonas.
ETIOLOGY
Most often bacterial.
See Table 19-1 for causes of osteomyelitis by age group.
Overall, Staphylococcus aureus is the most common bug.
TABLE 19-1. Causes of Osteomyelitis by Age
PATHOPHYSIOLOGY
Primarily hematogenous.
Spread from contiguous infected structures.
Direct inoculation.
The most common site for osteomyelitis is the rapidly growing end (metaphysis) of long bones.
SIGNS AND SYMPTOMS
Infants and young children:
Fever, irritability, and lethargy.
Refusal to walk or bear weight.
Older children:
May localize pain
Limping
Physical examination:
Painful local swelling
Point tenderness
Local warmth
Erythema
Nearly 50% of hematogenous osteomyelitis occurs in the tibia or femur.
DIAGNOSIS
Leukocytosis.
Elevated ESR: Sensitive marker for osteomyelitis, mean ∼70 mm/hr. Peaks 3–5 days.
Elevated C-reactive protein (CRP): peaks at 48 hr.
CRP typically returns to normal 7–10 days after appropriate therapy but the ESR may remain elevated for 3 or 4 weeks, even with appropriate therapy.
Growth on blood culture.
Radiographic findings (see Figure 19-1):
Lucent areas in bone represent cortical destruction.
Periosteal elevation. Periosteal and lytic changes in the bone may not be seen until substantial bone destruction has occurred.
Plain films may be normal for 7–10 days in up to two-thirds of children.
Consider osteomyelitis in any child with use of a limb and fever.
Every attempt should be made to establish a microbiologic diagnosis.
FIGURE 19-1. Acute hematogenous osteomyelitis of the proximal humerus.
Mottling and patchy radiolucencies are present in the metaphyseal region. (Reproduced, with permission, from Wilson FC, Lin PP. General Orthopedics. New York: McGraw-Hill, 1997.)
Radionuclide scintigraphy (bone scan):
Common isotopes used include technetium, gallium, and indium.
Can detect osteomyelitis within 24–48 hr of onset with ∼90% sensitivity.
Caution: Radionuclide scans may be positive in other illnesses that result in osteoblastic activity, including malignancy, trauma, cellulitis, postsurgery, and arthritis.
Magnetic resonance imaging (MRI):
Provides anatomic detail not seen with bone scan.
Useful for visualizing soft tissue abscess associated with osteomyelitis, bone marrow edema, and bone destruction.
Contrast enhancement with gadolinium.
DIFFERENTIAL DIAGNOSIS
Septic arthritis (can coexist).
Fracture.
Cellulitis.
Transient synovitis.
Acute leukemia or neuroblastoma.
Slipped capital femoral epiphysis (SCFE).
The ESR and CRP can be followed to assess response of osteomyelitis to therapy. They should if treatment is working.
TREATMENT
Admit all children with osteomyelitis.
Orthopedic consultation.
Parenteral antibiotics pending cultures (obtain blood, bone, and joint aspirate cultures before antibiotic administration).
Infants and children: Penicillinase-resistant penicillin (oxacillin) and cephalosporin (cefotaxime).
Older children (> 5 yr): nafcillin or vancomycin.
Consider surgical drainage if:
Pus is obtained from aspirate.
No response to 24–48 hr of antibiotics.
Osteomyelitis without radiographic change should not be treated with antibiotics until an osseous specimen is obtained.
COMPLICATIONS
Pathologic fractures.
Chronic osteomyelitis.
Leg length discrepancy.
SEPTIC ARTHRITIS
A 14-year-old boy presents to the emergency department (ED) because of right knee pain for the past 2 days. Three days prior to the onset of the pain, he hit his knee on a pool table. Vitals: Temperature 100.6°F (38.1°C), pulse rate 100, respirations 24. On physical exam, the knee is slightly swollen and tender and is held in flexion. Think: The most important initial diagnostic procedure is aspiration of the knee for smear and culture.
Although more common in children, it can occurs in all ages. Trauma can be the precipitant of infection. Staphylococcus aureus is the most common cause of septic arthritis. Physical examination may show local erythema, warmth, and swelling. The key to the diagnosis is the detection of bacteria in the synovial fluid either by Gram stain or by a culture; therefore, synovial fluid aspiration should be performed.
Most common cause of polyarticular septic arthritis is Neisseria gonorrhoeae.
A 5-year-old boy who has a definite history of penicillin allergy develops osteomyelitis. Smear of the aspirate shows gram-positive cocci in clusters. Think: Treat child with vancomycin.
Vancomycin is directed against gram-positive organisms and can be given to patients who can not receive penicillins and cephalosporins.
DEFINITION
A microbial invasion of joint space.
Adolescent intravenous (IV) drug abusers are at risk for gram-negative septic arthritis.
ETIOLOGY
Neonates:
S aureus (most common cause of septic arthritis in all ages).
S agalactiae.
Gram-negative enteric bacilli (K kingae has replaced Haemophilus influenzae type b [Hib] as the most common gram-negative arthritis in the child 2 months to 5 years old).
Older children (very similar to osteomyelitis):
S aureus: Remember—infection with community-acquired methicillin-resistant S aureus (CA-MRSA) is becoming more common.
S pyogenes.
S pneumoniae.
Gonococcus.
Candida albicans must also be considered in neonates and premature infants with septic arthritis.
EPIDEMIOLOGY
Relatively common in infancy and childhood; can occur in all ages.
PATHOPHYSIOLOGY
Organisms may invade the joint by:
Direct inoculation.
Contiguous spread.
Bacteremia (most common route).
Septic arthritis is an orthopedic emergency.
SIGNS AND SYMPTOMS
Pain.
Joint stiffness.
Erythema.
Edema.
Limp and unable to bear weight.
Two-thirds of cases of septic arthritis occur in weight-bearing (hip or knee) joints and involve a single joint (monoarticular).
LABORATORY
Complete blood count (CBC)—a normal WBC does not rule out diagnosis.
Elevated ESR.
Blood culture.
Joint aspiration.
MANAGEMENT
Admit all children with septic arthritis.
Orthopedic consultation (it is an orthopedic emergency)
Joint aspiration.
Parenteral antibiotics immediately after joint aspiration.
The knee is the most frequently infected pediatric joint, but the hip is known to have the most severe consequences.
COMPLICATIONS
Potential for severe complications:
Spread—results in osteomyelitis.
Avascular necrosis.
Angular deformities.
Leg length discrepancy.
Fever is not necessary for diagnosis of septic arthritis.
TRANSIENT SYNOVITIS
An 18-month-old infant develops a temperature of 105°F (40.6°C) and refuses to bear weight on her right leg. Physical exam reveals a swollen and warm right knee that the infant will not allow to be flexed or extended. The infant was diagnosed with varicella 3 weeks prior to the onset of this illness. Think: The most appropriate diagnostic test would be a synovial fluid analysis.
This patient has a high suspicion for septic arthritis. Joint aspiration is the gold standard for diagnosing septic arthritis and should be performed whenever septic arthritis is suspected. Septic arthritis (due to group A β-hemolytic streptococcus) can occur after a viral infection such as varicella. Children with septic arthritis usually have a history of high fever and restriction of movements.
DEFINITION
A reactive arthritis.
The most common cause of hip pain in childhood.
Predominates in children 5–10 years old.
ETIOLOGY
Cause remains uncertain.
Often follows an upper respiratory infection (URI).
Most common mimic of septic arthritis is transient synovitis. Examining the joint aspirate can differentiate.
SIGNS AND SYMPTOMS
Unilateral hip or groin pain is the most common complaint.
Painful limp.
Usually afebrile and nontoxic appearance.
DIAGNOSIS
Diagnosis of exclusion. Transient synovitis must be distinguished from septic arthritis. The two disorders present on a continuum and may overlap. Where doubt exists, ultrasound-guided or fluoroscopically guided diagnostic aspiration should be performed.
Radiographs are usually normal.
Plain films do not diagnose or exclude a hip effusion.
The appearance of a septic arthritis of the hip may be identical.
MANAGEMENT
First, rule out septic arthritis.
Supportive therapy.
Nonsteroidal anti-inflammatory drugs (NSAIDs).
Complete recovery occurs within a few weeks.
OSGOOD-SCHLATTER DISEASE
A 16-year-old boy complains of right knee pain. On examination, there is significant tenderness and swelling over the tibial tuberosity. He is otherwise healthy. Think: Osgood–Schlatter disease; treat with activity restriction. Osgood-Schlatter disease is a chronic overuse injury and a common cause of knee pain. It occurs due to forceful contraction of the extensor mechanism in sports such as jumping. It is a clinical diagnosis. Tenderness over the proximal tibial tuberosity at the site of patellar insertion is often present. However, plain radiographs are helpful to rule out other causes of knee pain.
Pain is most pronounced over the tibial tubercle in Osgood-Schlatter disease.
DEFINITION
An inflammatory disorder of the proximal tibial physis where the patellar tendon inserts on the tibia.
Benign, self-limited extra-articular disease.
Typical history: Nonspecific aching knee pain exacerbated by exercise.
ETIOLOGY
Traction apophysitis/repetitive trauma.
Chronic microtrauma to the tibial tuberosity secondary to overuse of the quadriceps muscle.
Osgood-Schlatter disease is a common cause of knee pain in the adolescent.
RISK FACTORS
Boys between ages 11 and 18 yr.
Rapid skeletal growth.
Involvement in repetitive jumping sports.
Septic arthritis may coexist with osteomyelitis at sites where the metaphysis lies within the joint capsule:
Proximal femur–hip joint
Proximal humerus– shoulder joint
Distal lateral tibia–ankle joint
Proximal radius–elbow joint
SIGNS AND SYMPTOMS
Knee pain (tibial tuberosity pain).
Reproduced by extending the knee against resistance.
Knee joint examination is normal.
Tibial tuberosity swelling.
Absence of effusion or condylar tenderness.
DIAGNOSIS
Diagnosis is primarily clinical.
X-ray of the knee may show evidence of fragmentation of the tibial tubercle (see Figure 19-2). Compare with opposite side.
TREATMENT
Relative rest.
Restriction of activities as tolerated (patients can still engage in activities even with pain; they will eventually grow out of it).
Knee immobilizer only for severe cases.
Complete resolution through physeal closure.
The major consequence of bacterial invasion of a joint is permanent damage to joint cartilage.
FIGURE 19-2. Osgood-Schlatter disease.
Note the elevation and irregularity of the tibal tubercle. (Reproduced, with permission, from Wilson FC, Lin PP. General Orthopedics. New York: McGraw-Hill, 1997.)
LEGG-CALVÉ-PERTHES DISEASE
A 6-year-old boy presents with hip and knee pain. He has been limping. On examination, he is unable to abduct or internally rotate his hip. Think: Legg-Calvé-Perthes disease.
Legg-Calvé-Perthes disease is osteonecrosis of the capital femoral epiphysis of the femoral head. It is due to vascular changes within the proximal femur. Limping is the most common symptom. Pain may be poorly localized in the groin or referred to the thigh or knee joint. It is therefore important to recognize that thigh or knee pain in the child may be due to hip pathology. Plain x-rays of the hip are helpful in making the diagnosis. It occurs at an earlier age than slipped capital femoral epiphysis (SCFE).
DEFINITION
Avascular necrosis of femoral head (occurs when blood supply to the proximal femoral epiphysis is disrupted).
Toxic synovitis is the most common cause of limping and acute hip pain in children aged 3–10 yr.
ETIOLOGY
Idiopathic.
Some precipitants include sickle cell disease, steroids, trauma, and infection.
EPIDEMIOLOGY
Male-to-female ratio: 4:1.
Highest incidence is during periods of rapid growth of the epiphyses (ages 4–8 yr).
SIGNS AND SYMPTOMS
Insidious onset. Symptoms generally begin with minor trauma.
Limp with or without pain.
Pain (activity related and relieved by rest).
Limited hip motion, particularly abduction and medial rotation.
range of motion.
Knee pain is common.
Classic presentation of Legg-Calvé-Perthes disease is a “painless limp.”
RADIOLOGY
Anteroposterior (AP) and frog-leg lateral position. X-ray findings correlate with the progression and extent of necrosis (see Figure 19-3).
Early: Effusion of the joint, widening of the joint space and periarticular swelling.
Few weeks: bone density around the joint, collapse of the femoral head (affected side appears smaller than the unaffected femoral head).
Late: New bone replaces necrotic bone.
MANAGEMENT
Pediatric orthopedic consultation.
Protect joint.
Abduction orthoses to “contain” the femoral head on the acetabulum.
Rest and NSAIDs.
Surgery for 6- to 10-year-olds with large areas of necrosis.
COMPLICATIONS
Limb length discrepancy.
FIGURE 19-3. Radiograph of pelvis demonstrating changes of Legg-Calvé-Perthes disease.
Note the sclerotic, flattened, and fragmented right femoral head.
SLIPPED CAPITAL FEMORAL EPIPHYSIS (SCFE)
An obese 14-year-old boy has pain in the left anterior thigh for 2 months. On physical exam, there is limited passive flexion and internal rotation of his hip. Think: The most likely diagnosis is SCFE.
SCFE is the most common hip disorder of adolescence and occurs at the time of the pubertal growth spurt. Referred pain (groin, thigh, or knee pain) is a common presentation. AP and frog-leg lateral views of the pelvis should be obtained.
Knee pain in a child warrants a complete hip examination.
DEFINITION
Type of Salter I fracture of the proximal femoral growth plate.
Disruption of the proximal femoral epiphysis through the physeal plate.
Epiphysis is usually displaced medially and posteriorly.
SCFE is the second most commonly missed time-sensitive pediatric orthopedic problem (fracture is most common).
ETIOLOGY
Most cases are idiopathic.
Weak growth plate (physis is weak prior to closure).
Local trauma.
TYPES
Acute (< 3 weeks).
Chronic (> 3 weeks).
Remember, slips can occur in children of normal weight.
RISK FACTORS
Obesity.
Hypothyroidism.
Hypogonadism.
Growth hormone (GH) administration.
Renal osteodystrophy.
Radiation therapy.
MRI can reveal avascular necrosis, whereas conventional radiographs may appear normal.
SIGNS AND SYMPTOMS
Pain can be located anywhere between the groin and medial knee.
Limping.
Internal rotation, flexion, and abduction are lost.
Painful limp.
Leg tends to roll into external rotation.
SCFE is the most common orthopedic hip disorder occurring in adolescence.
DIAGNOSIS
AP and frog-leg lateral of both hips (Figure 19-4).
Ice cream scoop (epiphysis) falling off its cone.
The frog-leg lateral film demonstrates subtle displacement more clearly.
Earliest sign is widening of epiphysis.
Always examine and obtain x-ray of the contralateral hip.
A diagnosis of a preslip can be made with bone scintigraphy.
In the presence of suspicion for SCFE, both hips should be imaged.
COMPLICATIONS
Avascular necrosis of capital femoral epiphysis.
Chondrolysis.
FIGURE 19-4. Hip radiographs in a 13-year-old girl with mildly slipped capital femoral epiphysis (SCFE) on the right.
Note on the AP view that a line drawn along the superior border of the femoral neck (Klein line) shows less femoral head superior to the line on the right than it does in the normal hip on the left.
Nonunion.
Premature closure of the epiphyseal plate.
Fifteen percent of children who have SCFE have mostly knee or distal thigh pain.
TREATMENT
Orthopedic consultation.
Removal of weight bearing from the affected limb (crutches or wheelchair in overweight patients).
Internal fixation using central percutaneous pin fixation with one or more cannulated screws is the treatment of choice (Figure 19-5).
Bilateral SCFE is common.
FIGURE 19-5. SCFE after screw fixation (same patient as Figure 19-4).
TENOSYNOVITIS
DEFINITION
Inflammation of the tendon and tendon sheath.
ETIOLOGY
Trauma
Overuse
Klein line: On the AP view of the hip, a line drawn along the superior border of the femoral neck should pass through a portion of the femoral head. If not, consider SCFE.
TYPES
de Quervain tenosynovitis of the wrist (ie, abductor pollicis longus and extensor pollicis brevis tendons).
Volar flexor tenosynovitis (ie, trigger finger).
MANAGEMENT
Rest.
NSAIDs.
Thumb spica wrist splint.
THE LIMPING CHILD
Thorough history and physical.
Assess gait with patient barefoot based on age.
Plain-film radiographs typically initial study.
Consider lab work based on differential diagnosis (ie, CBC, ESR).
DIFFERENTIAL DIAGNOSIS
JUVENILE RHEUMATOID ARTHRITIS (JRA)
DEFINITION
Chronic disease characterized by inflammation of the joints.
ETIOLOGY
Unknown.
Seek help from your radiology and orthopedic colleagues if clinical suspicion for SCFE is high but the films are negative.
CLASSIFICATION
Polyarticular (35%):
Five or more joints.
Symmetric distribution.
Both large and small joints.
Pauciarticular (50%):
Fewer than five joints.
Asymmetric distribution.
Often large weight-bearing joints.
Iridocyclitis (50%).
Systemic (20%):
Fever, rash, arthritis, and visceral involvement.
Also known as Still disease.
The most common cause of chest pain in children is idiopathic.
DIAGNOSTIC CRITERIA
Age of onset under 16 yr.
Arthritis in one or more joints.
Duration ≥ 6 weeks.
Exclusion of other causes.
See Table 19-2 for diagnosis based on joint fluid analysis.
Rheumatoid factor (RF) tends to be negative in early childhood in JRA. RF is positive in about 15% of patients, usually when onset of polyarticular disease occurs after the age of 8 yr.
SIGNS AND SYMPTOMS
Polyarticular:
Symmetric, chronic pain and swelling of joints.
Systemic features are less prominent.
Long-term arthritis; symptoms wax and wane.
Pauciarticular:
Asymmetric chronic arthritis of a few large joints.
Systemic features are uncommon.
TABLE 19-2. Joint Fluid Analysis
Systemic:
Salmon-pink macular rash.
Systemic symptoms: Arthritis, hepatosplenomegaly, leukocytosis, and polyserositis.
Episodic, remission of systemic features within 1 yr.
TREATMENT
The goal of treatment is to restore function, relieve pain, and maintain joint motion.
NSAIDs.
Range-of-motion and muscle-strengthening exercises.
Methotrexate, anti–tumor necrosis factor (TNF) antibodies, or antipyrimidine medication for patients who do not respond to NSAIDs.
A normal ESR does not exclude the diagnosis of JRA.
REITER SYNDROME
DEFINITION
Triad of asymmetric arthritis, urethritis, and uveitis.
ETIOLOGY
Thought to be a reactive arthritis after infection with gram-negative (Salmonella, Shigella, Yersinia, Campylobacter, Chlamydia, Mycoplasma, and Ureaplasma) in persons with human lymphocyte antigen (HLA)-B27.
The presence of HLA-B27 is a major determinant of disease severity in Reiter’s syndrome and a predictor of recurrence.
DIAGNOSIS
Bone density is preserved.
Proliferative bone formation is present.
Routine ophthalmologic screening should be performed every 3–6 months for 4 yr for all children with arthritis to look for iridocyclitis.
CHILDHOOD FRACTURES (NOT RELATED TO ABUSE)
Torus Fracture (Figure 19-6)
Latin torus = buckle.
Buckle fracture: A buckle in the concave cortex of malleable bone under compression.
Impaction injury in children in which the bone cortex is buckled but not disrupted.
Stable fracture.
Greenstick Fracture (Figure 19-7)
Definition: A break in the convex cortex under tension caused by the bending of malleable bone.
Angulation beyond the limits of plastic deformation.
Incomplete fracture in which cortex is disrupted on only one side.
Represents bone failure on the tension side and a plastic or bend deformity on the compression side.
Reiter syndrome: Can’t pee, can’t see, can’t climb a tree.
FIGURE 19-6. Torus fracture.
Toddler Fracture (Figure 19-8)
Nondisplaced spiral fracture of the tibia.
Symptoms include pain, refusal to walk, and minor swelling.
There is often no history of trauma, or a history of a twisting motion of the leg with a planted foot.
Differential diagnosis: Should include nonaccidental trauma.
Treatment: Immobilization for a few weeks to protect the limb and to relieve pain.
Salter-Harris Fracture Classification
See Figure 19-9.
FIGURE 19-7. Greenstick fracture.
FIGURE 19-8. Toddler fracture.
(Reproduced, with permission, from Schwartz DT, Reisdorff BJ. Emergency Radiology. New York: McGraw-Hill, 2000: 602.)
FIGURE 19-9. Salter-Harris fracture classification.
SPRAINS
DEFINITION
Sprain: Injury to ligament.
Strain: Injury to muscle-tendon unit.
Ankle Sprain
Inversion: Injury to lateral ligament (85%).
Anterior talofibular injures first.
Posterior talofibular—severe pain.
Eversion: Injury to medial ligament (15%).
Deltoid ligament injury most common.
More severe than inversion.
Sprain is a diagnosis of exclusion in children.
SIGNS AND SYMPTOMS
Grade I: Pain/tenderness without loss of motion.
Grade II: Pain/tenderness, ecchymosis with some loss of range of motion.
Grade III: Ligament is completely disrupted; pain/tenderness, swelling and ecchymosis, joint instability, and complete loss of range of motion.
MANAGEMENT
The goal of treatment is to local edema and residual stiffness.
RICE therapy—rest, ice, compression, elevation.
Protection includes joint immobilization at a right angle, elastic (Ace) bandage wrap, and Jones’s dressing for more severe injuries. Splinting the affected joint protects against injury and relieves swelling and pain.
Crutches and crutch gait training.
NSAIDs as needed for analgesia.
Early use of joint and appropriate rehabilitation is key to healing process.
SUBLUXATION OF RADIAL HEAD
A 2-year-old boy complains of left arm pain. He holds his arm in a flexed, pronated position and refuses to supinate his forearm during examination. His mother remembers pulling him by the arm yesterday. Think: Subluxation of the radial head (nursemaid’s elbow).
Subluxation of the radial head is a common traumatic elbow injury in children. Average age is between 2 and 4 yr. It is due to a sudden longitudinal pull on the forearm while the child’s arm is in pronation. Child keeps his arm in passive pronation, with slight flexion at the elbow. Radiographs are not required if the history suggestive of this injury.
DEFINITION
Subluxation of the radial head.
ETIOLOGY
Slippage of the head of the radius under the annular ligament.
Most common cause is axial traction.
Sudden longitudinal pull on the forearm while the child’s arm is in pronation.
Stretching of the annular ligament allows fibers to slip between the capitellum and the head of the radius.
EPIDEMIOLOGY
Common age: 1–4 yr.
More frequent under 2 yr.
Left arm predominance.
Rare after the age of 6 yr (annular ligament becomes thick and strong by age 5 yr).
SIGNS AND SYMPTOMS
Child suddenly refuses to use an arm.
Elbow fully pronated/inability of the child to supinate the arm.
DIAGNOSIS
Diagnosis is made primarily by history.
Imaging studies are unnecessary.
MANAGEMENT
Elbow is placed in full supination and slowly moved to full flexion.
Alternatively, overpronation of the forearm is also effective.
A click at the level of the radial head signifies reduction (see Figure 19-10).
Relief of pain is remarkable.
FIGURE 19-10. Reduction of nursemaid’s elbow.
OSTEOSARCOMA
A patient has had dull, aching pain for several months that has suddenly become more severe. Think: Osteosarcoma.
Osteosarcoma is a common cancer in adolescence. Symptoms may be present for a significant period of time before it is diagnosed. Pain, particularly with activity, is a common symptom. Distal femur and proximal tibia are commonly involved bones. On examination, palpable mass may be present. Since osteosarcoma is not radiosensitive, surgery may be needed.
DEFINITION
Malignant tumor arising from osteoblasts.
Osteosarcoma is the sixth most common malignancy in children and the third most common in adolescents.
EPIDEMIOLOGY
The most frequent sites of origin are the metaphyseal regions.
Most osteosarcomas develop in patients 10–20 years of age.
Osteosarcomas most frequently occur during periods of maximal growth.
SIGNS AND SYMPTOMS
Bone pain.
Typically long bones (distal femur and proximal tibia) and flat bones (pelvis 10%).
Osteosarcoma is the most common primary malignant neoplasm of bone (60%).
FIGURE 19-11. Osteosarcoma of proximal humerus.
Note disorganized appearance of bony cortex (arrow).
RADIOLOGY
Radiographs show mixed sclerotic and lytic lesion arising in the metaphyseal region, often described as a sunburst pattern (Figure 19-11).
All patients with osteosarcoma should undergo computed tomographic (CT) scanning to detect metastatic pulmonary disease.
MANAGEMENT
Bone tumors generally are sensitive to radiation and chemotherapy.
Amputation and limb salvage are effective in achieving local control.
PROGNOSIS
Seventy-five percent survival in nonmetastatic disease.
Death is usually due to pulmonary metastasis.
Widely metastatic disease carries poor prognosis.
EWING’S SARCOMA
A 10-year-old boy complains of pain in his left leg. On examination, there is localized swelling and pain in the middle of his left femur. His temperature is 100.8°F (38.2°C), and ESR is elevated. Further questioning reveals a 2-month history of increasing fatigue and weight loss. Think: Ewing’s sarcoma.
Ewing’s sarcoma is a common malignant bone tumor in young patients. Most patients present with either pain or a mass. Most common site for metastases is lung. Periosteal reaction and new bone formation with an onion-skin appearance are suggestive of Ewing’s sarcoma. Most tumors are considered radiosensitive.
DEFINITION
Malignant tumor of bone arising in medullary tissue.
Primary site is split almost evenly between the extremities and central axis.
EPIDEMIOLOGY
Most common bone lesion in first decade.
Second to osteosarcoma in second decade.
However, still rare—only 200 new cases/yr.
Very strong Caucasian and male predilection, hereditary.
Bone pain is a presenting symptom of Ewing’s sarcoma in 80–90%.
SIGNS AND SYMPTOMS
Bone pain.
Systemic signs: Fever, weight loss, fatigue.
RADIOLOGY
Calcified periosteal elevation, termed onion skin.
Radiolucent lytic bone lesions in the diaphyseal region.
Evaluation of patients with Ewing’s sarcoma should include a CT to define the extent of metastatic disease.
TREATMENT
Radiotherapy.
Chemotherapy.
Surgical resection.
Autologous bone marrow transplant for high-risk patients.
Metastasis is present in 25% of patients with Ewing’s sarcoma at diagnosis. The most common sites of metastasis are the lungs, bone (spine), and bone marrow.
PROGNOSIS
Patients with a small localized tumor have a 50–70% long-term disease-free survival rate.
Patients with metastatic disease have a poor prognosis.
BENIGN BONE TUMORS
Osteoid Osteoma
DEFINITION
Reactive lesion of bone.
SIGNS AND SYMPTOMS
Pain (evening or at night), relieved with aspirin.
Point tenderness.
Predominantly found in boys.
Osteoid osteomas are most common in the femur and tibia.
RADIOLOGY
Osteosclerosis surrounds small radiolucent nidus.
MANAGEMENT
Salicylates relieve pain.
Surgical incision of the nidus is curative.
PROGNOSIS
Prognosis is excellent. There have been no known cases of malignant transformation, although the lesion has been known to reoccur.
Enchondroma
DEFINITION
Cartilaginous lesions.
SIGNS AND SYMPTOMS
Tubular bones of hands and feet.
Pathologic fractures.
Swollen bone.
Ollier disease (if multiple lesions are present).
Enchondromas have a predilection for the phalanges.
RADIOLOGY
Radiolucent diaphyseal or metaphyseal lesion.
Often described as “fingernail streaks in bones.”
MANAGEMENT
Surgical curettage and bone grafting.
Mafurci syndrome is multiple enchondromas and aniomas of the soft tissue.
PROGNOSIS
Prognosis is excellent. Malignant transformation may occur, but is very rare in childhood.
Osteochondroma (Exostosis)
DEFINITION
Most common bone tumor in children.
Disturbance in enchondral growth.
Benign cartilage-capped protrusion of osseous tissue arising from the surface of bone.
SIGNS AND SYMPTOMS
Painless, hard, nontender mass.
Distal metaphysis of femur, proximal humerus, and proximal tibia.
Grows with child until skeletal maturity
RADIOLOGY
Pedunculated or sessile mass in the metaphyseal region of long bones.
Baker cysts are the most common mass in the popliteal fossa.
MANAGEMENT
Excision if symptomatic.
PROGNOSIS
Prognosis is excellent. Malignant transformation is very rare.
It is important to exclude deep vein thrombosis (DVT) in patients with a popliteal cyst and leg swelling.
Baker’s Cysts
DEFINITION
Herniation of the synovium in the knee joint into the popliteal region.
A Baker’s cyst is lined by a true synovium, as it is an extension of the knee joint.
SIGNS AND SYMPTOMS
Popliteal mass
Commonly transilluminates
DIAGNOSIS
Aspiration of mucinous fluid from popliteal fossa.
MANAGEMENT
Baker’s cysts are benign.
Nearly always disappears with time in children.
Avoid surgery (only for significant pain).
DEVELOPMENTAL DYSPLASIA OF THE HIP (DDH)
While doing a physical exam on a 3-month-old female infant, the physician notices that her left knee is lower when her hips are flexed. The infant was born to a P1G1 mother via a breech vaginal delivery. Think: DDH.
It is called Galeazzi’s sign, which is an apparent shortening of the femur on the side of the dislocated hip and is noted by placing both hips in 90 degrees of flexion and comparing the height of the knees. Screening examination should include the Ortolani test and the provocative maneuver of Barlow. Risk factors include female gender, breech presentation, and positive family history for DDH. Ultrasound can be obtained in infants younger than 6 months. It is a treatable condition with successful treatment if intervention starts early. Pavlik harness is the treatment of choice in the first 6 months of life.
Associated anomalies with DDH:
Torticollis
Clubfeet
Metatarsus adductus
DEFINITION
Abnormal growth and development of the hip resulting in an abnormal relationship between the proximal femur and the acetabulum.
Ortolani test: Slowly abduct flexed hip. The femoral head will shift into the acetabulum producing a clunk.
EPIDEMIOLOGY
One in 1000 live births.
Tenfold risk in sibling of child with DDH.
Female > male. Breech female is at highest risk.
Barlow test: Dislocate the hip by flexing and adducting the hip with axial pressure.
PATHOPHYSIOLOGY
At birth there is a lack of development of both acetabulum and femur.
Progressive with growth.
Reversible if corrected in first few days or weeks.
Forced abduction of the hips in DDH is contraindicated because of risk of avascular necrosis.
SIGNS AND SYMPTOMS
Newborn:
Ortolani: Reduction maneuver.
Barlow: Provocative test potential for dislocation of a nondisplaced hip.
Asymmetric skin folds (40%).
3–6 months:
Limited abduction.
Allis’s or Galeazzi’s sign: Knee is lower on affected side when hips are flexed.
12 months (unilateral dislocation): Trendelenburg sign—painless limp and lurch to the affected side with ambulation. When the child stands on the affected leg, there is a dip of the pelvis on the opposite side, due to a weakness of the gluteus medius muscle.
12 months (bilateral dislocation):
Waddling gait.
Lumbar lordosis due to flexion contractures.
Signs of instability are more reliable than x-ray in DDH.
IMAGING
< 6 months: Ultrasound (acetabulum and proximal femur are predominantly cartilaginous).
> 6 months: Radiographs (proximal femoral epiphysis ossifies by 4–6 months).
In DDH, after 3–6 months, muscle contractures develop, and the Barlow and Ortolani tests become negative.
TREATMENT
Newborn to 6 months: Pavlik harness (flexion and abduction of the hip).
6 months to 3 years: Skin traction for 3 weeks to relax soft tissues around the hip prior to closed or open reduction. After 6 months of age, the failure rate for the Pavlik harness is > 50%.
> 3 yr: Operations to correct deformities of the acetabulum and femur.
Triple diapers have no place in the treatment of DDH.
OSTEOGENESIS IMPERFECTA (OI)
A 2-year-old child is brought in with a right radial fracture after lightly bumping his arm. An x-ray shows multiple healing fractures. On examination, the child has blue sclera, thin skin, and hypoplastic teeth. Think: OI. OI is also called brittle bone disease. Triad: fragile bones, blue sclerae, and early deafness. The teeth frequently have dentinogenesis imperfecta. The enamel is normal, but the dentin is dysplastic. Radiographic appearance may vary according to the type of disease and its severity and include osteopenia and fractures. In infancy, these features may result in evaluation for nonaccidental injury.
Double or triple diapers are not adequate to obtain a proper position and are no longer indicated treatment of DDH.
DEFINITION
Rare, inherited disorder of connective tissue, characterized by multiple and recurrent fractures.
OI is an autosomal-dominant disorder that occurs in all racial and ethnic groups.
X-ray is not helpful in the newborn. After 6–8 weeks, x-rays begin to show signs of dislocation (lateral displacement of the femoral head).
ETIOLOGY
Molecular genetics have identified more than 150 mutations in the genes that encode for type 1 collagen.
There are four types of OI: Types I and IV are mild and present with an risk of fractures. Type II is lethal in the newborn period, and type III is a severe form causing significant bony deformity secondary to multiple fractures.
Ten percent of OI patients have the severe neonatal form of the disease.
OI is the most common osteoporosis syndrome in children.
SIGNS AND SYMPTOMS
Bone fragility.
Easy bruising.
Repeated fracture after mild trauma.
Deafness.
Blue sclera.
Hyperextensibility of ligaments.
Normal intelligence.
Type I collagen fibers are found in bones, organ capsules, fascia, cornea, sclera, tendons, meninges, and the dermis.
DIAGNOSIS
Radiographic findings:
Osteopenia.
Wormian bones.
Thin cortices.
Bowing.
Normal callus formation.
Collagen synthesis analysis.
TREATMENT
Bisphosphonates.
Surgical correction of long-bone deformities.
Trauma prevention.
PROGNOSIS
Prognosis is poor, and most patients are confined to wheelchairs by adulthood.
GENETIC COUNSELING
Genetic counseling should be offered.
Risk of an affected individual passing the gene to his or her offspring is 50%.
KLIPPEL-FEIL SYNDROME
DEFINITION
Congenital fusion of a variable number of cervical vertebrae.
Children with Klippel-Feil syndrome are at risk for:
Atlantoaxial instability
Neurologic impairment
ETIOLOGY
Failure of normal segmentation in the cervical spine.
SIGNS AND SYMPTOMS
Classic clinical triad:
Short neck.
Low hairline.
Limitation of neck motion.
Associated with:
Renal anomalies.
Scoliosis.
Spina bifida.
Deafness.
DIAGNOSIS
Children with Klippel-Feil syndrome should have the following tests performed:
Renal ultrasound.
Hearing test.
Lateral flexion-extension radiographs of cervical spine.
TREATMENT
Annual evaluation.
Avoid violent activities.
Close evaluation of immediate family members.
TORTICOLLIS
DEFINITION
Twisted or wry neck.
Torqueo = to twist; collum = neck
ETIOLOGY
Congenital: Injury to the sternocleidomastoid muscle during delivery.
Acquired: Rotatory subluxation of the upper cervical spine.
MANAGEMENT
Congenital: Physical therapy for stretching.
Acquired:
Warm soaks.
Analgesics.
Mild anti-inflammatory agents.
Soft cervical collar.
Passive stretching.
Torticollis is the most common cause of neck muscle strain.
MUSCULAR DYSTROPHIES
Duchenne’s Muscular Dystrophy (DMD)
A 3-year-old boy must use his hands to push himself up when rising from a seated position. Think: Gower’s maneuver.
The Gower test indicates proximal muscle weakness, which is described as in the ability to rise from the floor without assistance of the upper extremities. DMD is a sex-linked recessive inherited trait that occurs in males. Since children with DMD usually reach early motor milestones at appropriate times, diagnosis may be delayed. However, the diagnosis becomes evident between 3 and 6 years of age. Creatine kinase (CK) should be obtained, which is elevated (50–100 times normal). DNA analysis confirms the diagnosis.
DEFINITION
Degenerative disease of muscles. DMD is characterized by early childhood onset, typically within the first 5 yr.
INHERITANCE
X-linked recessive.
One in 3600 males.
SIGNS AND SYMPTOMS
Clumsiness.
Easy fatigability.
Symmetric involvement.
Axial and proximal before distal.
Pelvic girdle, with shoulder girdle usually later.
Rapid progression.
Loss of ambulation by 8–12 yr.
Pseudohypertrophy of calves.
Cardiomegaly—varied severity.
DMD is the most common muscular dystrophy.
DIAGNOSIS
Serum CK is markedly elevated.
Muscle biopsy is pathognomonic—degeneration and variation in fiber size and proliferation of connective tissue. No dystrophin present.
DMD is associated with:
Mental retardation
Cardiomyopathy
MANAGEMENT
Encourage ambulation.
Prevent contractures with passive stretching.
Death in patients with DMD occurs through cardiac or respiratory failure.
Becker’s Muscular Dystrophy (BMD)
DEFINITION
Milder form of muscular dystrophy.
INHERITANCE
X-linked recessive.
SIGNS AND SYMPTOMS
Late childhood onset, typically between 5 and 15 yr.
Slow progression.
Proximal muscle weakness.
Prominence of calf muscles.
Inability to walk occurs after 16 yr.
DIAGNOSIS
Muscle biopsy shows degeneration of muscle fibers. Dystrophin is reduced or abnormal.
Myotonic Muscular Dystrophy (MMD)
INHERITANCE
Autosomal dominant.
SIGNS AND SYMPTOMS
Congenital MMD affects infants and is more severe than the adult form.
Adult-onset MMD has a variable onset, typically in the teens to adulthood.
Muscle weakness of voluntary muscles in the face, distal limbs, and diaphragm.
Involuntary clenching of hands and jaw, ptosis, and respiratory difficulty.
Limb Girdle Muscular Dystrophy
DEFINITION
Two types:
Pelvifemoral (Leyden-Möbius).
Scapulohumeral (Erb’s juvenile).
INHERITANCE
Autosomal recessive, with high sporadic incidence.
SIGNS AND SYMPTOMS
Variable age of onset; childhood to early adult (present in second or third decade).
Pelvic girdle usually involved first and to greater extent.
Shoulder girdle often asymmetric.
DIAGNOSIS
Muscle biopsy shows dystrophic muscle changes. Dystrophin is normal.
MANAGEMENT
Promote ambulation.
Physiotherapy.
Mildly progressive, life expectancy mid to late adulthood.
Facioscapulohumeral Muscular Dystrophy
INHERITANCE
Autosomal dominant.
SIGNS AND SYMPTOMS
Variable.
Slow progression.
Diminished facial movements: inability to close eyes, smile, or whistle.
Weakness of the shoulder girdle: Difficulty raising arms over head.
Normal life span.
DERMATOMYOSITIS/POLYMYOSITIS
DEFINITION
Polymyositis primarily affects skeletal muscle.
Dermatomyositis: Skin eruption + myopathy.
EPIDEMIOLOGY
Female > male.
Age 5–14 yr.
In adults, dermatomyositis and polymyositis are associated with malignancy and rheumatic disease. Myositis is not associated with cancer in children.
SIGNS AND SYMPTOMS
Symmetric proximal muscle weakness.
Violaceous rash—symmetric, erythematous rash on extensor surfaces, upper eyelids, and knuckles. Rash around eyes called heliotrope rash.
Worrisome triad (not common):
Dysphagia
Dysphonia
Dyspnea
Dermatomyositis affects proximal muscles more than distal muscles, and weakness usually starts in the legs. An inability to climb stairs may be the first warning sign.
DIAGNOSIS
ESR, serum CK, and aldolase reflect the activity of the disease.
Electromyography (EMG) is used to distinguish myopathic from neuropathic causes of muscle weakness.
TREATMENT
Prednisone.
Intravenous immune globulin (IVIG), cyclosporine, or methotrexate in refractory cases.
PROGNOSIS
Most children will recover in 1–3 yr.
CONNECTIVE TISSUE DISEASES
Marfan Syndrome
DEFINITION
Genetic defect of genes coding for the connective tissue protein fibrillin.
The most worrisome complications of Marfan syndrome are aortic dilation, aortic regurgitation, and aortic aneurysms.
INHERITANCE
Autosomal dominant.
SIGNS AND SYMPTOMS
Musculoskeletal:
Tall stature.
Long, thin digits (arachnodactyly).
Hyperextensible joints.
High arched palate.
Cardiac: Dilation of the aorta.
Ocular: Ectopia lentis—lens dislocation (which progresses over time).
Ehlers-Danlos Syndrome (EDS)
DEFINITION
Group of genetically heterogenous connective tissue disorders.
ETIOLOGY
Quantitative deficiency of collagen causing poor cross-linking of collagen.
Autosomal dominant.
Type IV EDS is associated with a weakened uterus, blood vessels, or intestines. It is important to identify patients with EDS type IV because of the grave consequences of the disease. Women with EDS type IV should be counseled to avoid pregnancy.
SIGNS AND SYMPTOMS
Children with EDS are normal at birth.
Skin hyperelasticity.
Fragility of the skin and blood vessels.
Joint hypermobility.
Propensity for tissue rupture.
MANAGEMENT
Symptomatic.
Preventive.
Prolonged wound fixation.
Genetic counseling.
Scoliosis
DEFINITION
More than 10-degree curvature of spine in the lateral plane due to the rotation of the involved vertebrae (see Figure 19-12).
FIGURE 19-12. Radiograph of spine demonstrating marked scoliosis.
ETIOLOGY
Eighty percent of cases are idiopathic.
Scoliosis is associated with:
Neurofibromatosis.
Marfan syndrome.
Cerebral palsy.
Muscular dystrophy.
Poliomyelitis.
Myelodysplasia.
Congenital vertebral anomalies (hemivertebrae, unilateral vertebral bridge).
EPIDEMIOLOGY
Four to five times more common in girls.
Age of onset: 9–10 yr for girls, 11–12 yr for boys.
SIGNS AND SYMPTOMS
Usually asymptomatic.
Severe curvature may impairment of pulmonary function.
DIAGNOSIS
X-ray of entire spine in both the AP and lateral planes.
To examine children, have the patient bend forward 90 degrees with the hands joined in the midline. An abnormal finding consists of asymmetry of the height of the ribs or paravertebral muscles on one side.
Thirty percent of family members of patients with scoliosis are also affected. Siblings of affected children should be carefully examined.
MANAGEMENT
Treatment depends on the curve magnitude, skeletal maturity, and risk of progression:
Curve < 20 degrees: Physical therapy and back exercises aimed at strengthening back muscles.
Curve 20–40 degrees in a skeletally immature child: Orthopedic back brace. A back brace does not the curve, but prevents further curve progression.
Curve > 40 degrees: Spinal fusion to correct deformity.
Screening for scoliosis should begin at age 6–7 yr.
PROGNOSIS
Curve > 60 degrees: Associated with poor pulmonary function. Large thoracic curves are associated with a shortened life span.
Curve < 40 degrees: Usually do not progress. Small curves are well tolerated.
Risk of progression higher in younger childhood.
Kyphosis
DEFINITION
Posterior curvature of the spine.
ETIOLOGY
Scheuermann thoracic kyphosis is a structural deformity of the thoracic spine.
SIGNS AND SYMPTOMS
Pain
Progressive deformity
Neurologic compromise
Cardiovascular complaints
Cosmetic issues
RADIOLOGY
Diagnosis is confirmed on lateral radiographs.
X-ray shows anterior wedging of at least 5 degrees of three or more adjacent thoracic vertebral bodies.
Spondylolysis
DEFINITION
Fracture of the pars interarticularis due to repetitive stress to this area.
ETIOLOGY
Spondylosis occurs as a result of new bone formation in areas where the annular ligament is stressed.
TYPES
Congenital: Cervical.
Acquired: Lumbar, most often at L5 (85% of cases).
Spondylolysis is the most common cause of low back pain in adolescent athletes. This injury is most commonly seen in gymnasts, dancers, and football players.
SIGNS AND SYMPTOMS
Cervical pain.
Low back pain, worse during the adolescent growth spurt and with spine extension.
Radicular symptoms are not common.
DIAGNOSIS
Oblique x-ray view of the spine will show the characteristic “Scottie dog sign.”
TREATMENT
NSAIDs.
Strength and stretching exercises.
Lumbosacral back brace.
Spondylolisthesis
DEFINITION
Anterior or posterior displacement of one vertebral body on the next due to bilateral pars interarticularis injury.
SIGNS AND SYMPTOMS
A palpable “step-off” at the lumbosacral area.
Limited lumbar flexibility.
DIAGNOSIS
Lateral x-ray views show displacement of one vertebral body from another.
Grade 1: < 25% displacement
Grade 2: 25–50%
Grade 3: 50–75%
Grade 4: 75–100%
Grade 5: Complete displacement
MANAGEMENT
Treatment depends on grade of lesion:
< 30% displacement: No restrictions on sports activities, but requires routine follow-up.
> 50% displacement: In situ posterior spinal fusion or bracing.
Children at highest risk for diskitis:
Immunocompromised
Systemic infections
Postsurgery
COMPLICATIONS
Deformity
Disability
Diskitis
DEFINITION
Pyogenic infection of the intervertebral disk space.
An uncommon primary infection of the nucleus pulposus, with secondary involvement of the cartilaginous end plate and vertebral body.
The lumbar spine is the most common site of involvement for diskitis.
ETIOLOGY
Most present prior to 10 years of age.
Spontaneous.
S aureus is the most common organism causing diskitis.
SIGNS AND SYMPTOMS
Moderate to severe pain.
Pain is localized to the level of involvement and exacerbated by movement.
Radicular symptoms.
LABS
MRI is the radiographic study of choice.
Elevated ESR.
Plain radiographs are usually not helpful for early diagnosis of diskitis.
MANAGEMENT
Intravenous antibiotics.
Surgery is often not necessary.
RENAL OSTEODYSTROPHY
DEFINITION
Bone diseases resulting from defective mineralization due to renal failure.
SIGNS AND SYMPTOMS
Growth retardation
Muscle weakness
Bone pain
Skeletal deformities
Slipped epiphyses
DIAGNOSIS
Normal to serum calcium.
Normal to phosphorus.
alkaline phosphatase.
Normal parathyroid hormone (PTH) levels.
Radiographs of the hands, wrists, and knees show subperiosteal resorption of bone with widening of the metaphyses.
In children, renal osteodystrophy resembles rickets.
TREATMENT
Low-phosphate formula.
Enhance fecal phosphate excretion with oral calcium carbonate, an antacid that also binds phosphate in the intestinal tract.
The goals of treatment include normalization of the serum calcium and phosphorus levels and maintenance of the intact PTH level in the range of 200–400 pg/mL.
OSTEOCHONDRITIS DISSECANS
DEFINITION
Avascular necrosis of bone adjacent to articular cartilage.
SIGNS AND SYMPTOMS
Vague pain (typically in the knee or ankle).
With joint flexed, may be able to palpate defect below articular cartilage.
May present as a loose body in the joint.
Most common in the lateral portion of medial femoral condyle.
DIAGNOSIS
X-rays show characteristic appearance of subcondylar osteonecrosis.
MRI may be useful to confirm diagnosis.
TREATMENT
Children < 11 years—typically observed with serial radiographs to assess healing.
Adolescents—excision of loose fragments if small. Replacement with fixation if large. Sometimes area can be drilled to promote revascularization and healing.
PROGNOSIS
Typically good with appropriate intervention.