Harwood-Nuss' Clinical Practice of Emergency Medicine, 6 ed.

CHAPTER 269
Appendicitis

Maureen D. McCollough

Appendicitis is the most common surgical emergency in childhood. Despite the decline in mortality since the introduction of antibiotics, over the past 40 years morbidity secondary to delayed diagnosis and perforation has remained unchanged. Perforation rates in children are higher than those in adults and 50% to 70% in preschoolers versus 10% to 20% in adults (1). The clinical progression of symptoms and signs is also more rapid in children. What starts as vague abdominal complaints can quickly progress to ileus, nausea, vomiting, fever, tachycardia, localized pain, and finally peritonitis. The inability of the omentum in young children to wall off localized infections contributes to the development of diffuse peritonitis, which further emphasizes the importance of early intervention (1).

CLINICAL PRESENTATION

The primary cause of appendicitis is obstruction of the appendiceal lumen, caused most commonly by a fecalith (calcified fecal material). Other less common causes of obstruction include inflamed lymphoid tissue, vegetable or fruit seeds, intestinal worms (especially Ascaridae), bacterial infection (with Yersinia, Salmonella, or Shigella), anomalies of mucous-secreting glands (as in cystic fibrosis), and rarely, retained barium from previous radiographic studies (2).

Luminal obstruction of the appendix causes distention and increased intraluminal pressure, resulting in obstruction of the blood supply. Bacterial invasion occurs and ultimately the ischemic appendix becomes gangrenous and perforates. Bacteriologic cultures of perforated appendices have revealed polymicrobial organisms, including Escherichia coli, Bacteroides fragilis, and enteric streptococci.

Obstruction and distention stimulate appendiceal stretch receptors, relaying pain to the tenth thoracic ganglion, resulting in the perception of periumbilical pain. Bacterial invasion and inflammation then lead to fever, nausea, or vomiting in up to 80% of children. As inflammation extends to surrounding tissues, pain depends on the anatomic location of the appendix. Up to one-third of appendices are located in areas other than the usual location, with the result being that up to 30% of children have pain localized to areas other than the right lower quadrant. A retrocecal location may cause back or flank pain, a pelvic appendix may cause suprapubic pain, and a retroileal location may cause pain referred to the testicle.

If appendicitis is allowed to progress appendiceal rupture will occur. In this case the child is typically ill-appearing, toxic, and dehydrated. Particularly in younger children, the incompletely developed greater omentum is incapable of walling off the inflammatory process, resulting in pain that is more diffuse. The pain may decrease in intensity just after perforation, but typically becomes more severe as peritonitis spreads.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis of abdominal pain in children comprises a lengthy list of disorders. The list may divided into five broad categories: (1) intestinal diseases, (2) uterine, tubo-ovarian, or testicular pathology, (3) urinary tract infection or obstruction, (4) systemic illness (respiratory, vasculitic, endocrinologic, and hematologic), and (5) trauma (2).

1. Intestinal diseases: Mesenteric adenitis, viral and bacterial enteritis, constipation, Meckel diverticulitis, intussusception, enteric duplication

2. Uterine, tubo-ovarian, or testicular pathology: Ovarian or testicular torsion, acute epididymitis, pelvic inflammatory disease, ectopic pregnancy, endometriosis, mittelschmerz

3. Urinary tract infection or obstruction: Acute pyelonephritis, cystitis, renal stones

4. Systemic illness (respiratory vasculitic, endrocrine, hematologic): Basal pneumonia, Henoch–Schönlein purpura, sickle cell crisis, diabetic ketoacidosis

5. Trauma: Accidental or nonaccidental injury

It has been reported that 20% to 40% of children with acute appendicitis are given the wrong diagnosis on the first physician visit (1). The most common erroneous diagnoses, in descending order of frequency, accounting for more than 75% of all missed diagnoses, include acute mesenteric lymphadenitis, no organic pathologic condition, acute pelvic inflammatory disease, ovarian cyst, mittelschmerz, and acute gastroenteritis.

Acute Mesenteric Lymphadenitis

Mesenteric adenitis is often confused with acute appendicitis in children. A careful history in these patients almost invariably reveals the presence of upper respiratory symptoms. Laboratory studies are usually of limited value in differentiation, although a relative lymphocytosis may suggest mesenteric adenitis or a generalized viral syndrome. The abdominal pain is usually more diffuse and less severe than with appendicitis, and generalized lymphadenopathy may be noted on physical examination.

Pelvic Inflammatory Disease

A history of vaginal discharge in a sexually active adolescent can often be elicited. Interviewing adolescents alone, without parental presence, may provide a more informative history regarding sexual activity. Pain is most often bilateral and lower in location than with appendicitis. Physical examination reveals pain with motion of the cervix.

Acute Gastroenteritis

Acute gastroenteritis usually can be differentiated from appendicitis. There is often a history of other ill contacts. These patients have nausea, vomiting, watery diarrhea, and intermittent, crampy abdominal pain. Often, the vomiting precedes the diarrhea by 24 hours. Localizing signs on the abdominal examination are usually absent.

Constipation

Constipation is one of the most significant mimics of appendicitis frequently presenting with abdominal pain localized to the right lower quadrant (see Chapter 230). Often there is a history of hard, pellet-like stools, or decreased stool output. The pain is often colicky and waxes and wanes, but overall, the child appears well between colicky episodes. If the diagnosis is in question or appendicitis is still high on the differential, a mineral oil enema or pediatric Fleet enema may be useful to relieve the pain of constipation.

Meckel Diverticulitis

Preoperative differentiation is unnecessary, because the signs and symptoms of Meckel diverticulitis are similar to those of acute appendicitis, and surgical treatment is indicated for both.

Intussusception

Distinguishing between intussusception and acute appendicitis relies significantly on the age of presentation for each disease. Appendicitis is rare in children younger than 2 years of age, whereas nearly all intussusceptions occur in children younger than age 2. Intussusception typically occurs suddenly in an otherwise healthy infant. The infant “doubles up” with apparent colicky pain, but is usually pain-free between attacks. While a bloody mucoid stool may passed several hours after the onset of pain, this is rare and is an indicator of advanced disease. Physical examination may reveal a sausage-shaped mass in the right lower quadrant. Occasionally, infants with intussusception present with altered mental status. The diagnosis can be made using ultrasound. In patients between the ages of 1 and 3 years, both of these two entities should carry equal weight in the differential diagnosis until an imaging study such as ultrasound can help clarify the clinical picture. Treatment of intussusception is reduction by barium or air enema (see Chapter 270).

Henoch–Schönlein Purpura

Henoch–Schönlein purpura, a diffuse vasculitis, classically presents with joint pain and swelling and purpura. Abdominal pain may also be a prominent symptom. Rectal examination should be performed for occult blood, and a urinalysis to evaluate for hematuria due to nephritis.

Mittelschmerz

Patients with mittelschmerz are at the midpoint of their menstrual cycle. The pain of mittelschmerz is usually of sudden onset and may be either localized, generally to one of the lower quadrants, or diffuse.

Ovarian or Testicular Torsion

These are often accompanied by anorexia, nausea, and vomiting. The correct diagnosis in boys can always be made by including the genitalia in the physical examination, which usually reveals the subtle findings of asymmetry in gonadal size or position. In girls, ultrasound can be used to make the diagnosis of ovarian torsion more likely if decreased or absent vascular flow is seen unilaterally. And, it should be remembered that normal examination of the ovary, or normal testicular size or position, does not completely rule out the diagnosis of torsion.

Urinary Tract Infection

Urinary bacteria or white blood cells may be present in children with acute appendicitis if the appendix lies near the ureter or bladder. However, with the exception of an elevated specific gravity due to dehydration, the urinalysis is normal in more than 80% of children with acute appendicitis.

ED EVALUATION

The most important steps in the correct diagnosis of appendicitis are eliciting a thorough history and performing a complete physical examination, including examining the external genitalia in boys and performing a pelvic examination in adolescent girls. The diagnostic value of the rectal examination in children is limited; rectal tenderness is present in half of all children with or without appendicitis, and rectal examination should thus be limited to children with rectal complaints.

Examining a young child with abdominal pain can be challenging. Young children are often frightened of doctors and “white coats,” but observing the child from the doorway or across the room can be very revealing and yield important clues. Clinicians must not underestimate the value of information gleaned during this early subjective assessment. A young child who is ambulating easily around the room, interested in his or her surroundings, is less a cause of concern than a child who is ill-appearing and lying very quietly. A slight limp when the child walks, hesitation to climb onto the examination table, and inability to stretch the right leg may suggest appendicitis even before the full evaluation. The child usually prefers to lie still in the supine position with the thighs, particularly the right one, drawn up. Vital signs are typically normal, but a child with simple appendicitis may have a mild temperature elevation. Tachycardia may be present if the child is dehydrated, febrile, or in pain.

Abdominal palpation should start away from the painful area. Examination of the abdomen for bowel sounds and tenderness can often frighten young children. Distraction, for example by using a hand puppet or other toy, may help to improve the quality of the examination. The classic physical finding is point tenderness at McBurney point (3 to 5 cm from the anterior–superior iliac spine on a straight line drawn from that process to the umbilicus) (2). Muscular resistance to palpation of the abdomen parallels the severity of the inflammatory process. Early in the disease, resistance if present consists of voluntary guarding alone. With the progression of peritoneal irritation, muscle spasm increases and becomes involuntary. Bowel sounds are usually normal in early appendicitis, but advanced inflammation, perforation, or both, usually results in an ileus and a quiet abdomen.

Physical signs classically associated with acute appendicitis include Rovsing sign, psoas sign, and obturator sign (3). Rovsing sign tests for peritoneal inflammation by ascertaining the degree of referred right lower quadrant tenderness elicited from palpation of the left lower quadrant. To elicit the psoas sign, the child should lie on the left side and stretch the psoas muscle by slowly extending the right thigh. Alternatively, the child may lie supine and flex the right hip against a hand placed just above the knee. Pain elicited by either maneuver may indicate an irritating focus in proximity to the psoas muscle. Finally, a positive obturator sign occurs when, in a supine patient, passive internal rotation of the thigh (with the hip flexed to 90 degrees) produces right-sided abdominal pain, indicating irritation around the obturator internus muscle. A positive psoas or obturator sign suggests retrocecal or retrocolic inflammation. Although these three signs are often cited as being associated with appendicitis, few studies have evaluated the accuracy of the clinical examination for appendicitis (3).

Given the breadth of the differential diagnosis evaluations must be stratified for age. An abdominal pain evaluation for a 2-year old must include consideration not only of appendicitis, but also of intussusception. On the other hand, in an adolescent female ovarian pathology must be considered far more strongly than intussusception. Selected laboratory tests, including a urinalysis and CBC, may be indicated along with selected imaging studies including ultrasound, CT, or possibly MRI.

The interpretation and integration of findings from the physical examination, laboratory, and imaging studies does not guarantee that every diagnosis will be made, and the literature on the reliability of signs, symptoms, laboratory markers, and imaging in pediatric appendicitis is limited. A systematic review assessing the reliability of common clinical signs for ruling in appendicitis noted that those with the highest positive likelihood ratios were fever, rebound tenderness, and migration of pain to the right lower quadrant. The clinical signs with the highest negative likelihood ratios for ruling out appendicitis were absence of fever and absence of rebound tenderness (4). A history of previous similar pain also seemed to lower the likelihood of appendicitis. Patients with appendicitis, however, do not always present with the “most reliable signs with the highest positive likelihood ratios.” Becker et al. specifically studied atypical features in children with confirmed appendicitis to quantify their frequency and investigate which features had the strongest negative predictive value. They noted that 44% of patients presented with six or more atypical features, the most common include (1) lack of fever, (2) absence of Rovsing sign, (3) increased or normal bowel sounds, (4) absence of rebound, (5) lack of pain migration, and (6) lack of abrupt onset of pain. The atypical signs with strongest negative predictive properties based on likelihood ratios included (1) WBC <10,000 mm3, (2) an ANC <7,500 mm3, (3) absence of guarding, (4) lack of percussive tenderness, and (5) lack of nausea or emesis (5).

A number of clinical decision rules (CDRs) have been developed to help clinicians manage the diagnostic uncertainties of pediatric appendicitis (6). The MANTRALS score by Alvarado dates back to 1986 and was originally developed for adult appendicitis, but has been modified and studied in cohorts of pediatric patients as well. Other scores of note include the Pediatric Appendicitis Score and that by Kharbanda et al. (6). These scores all harness the high-yield indicators of pediatric appendicitis discussed above, including RLQ tenderness, rebound tenderness, leukocytosis, and nausea or emesis. Collectively, however, their performances vary widely and none has proven reliable as a single diagnostic tool that is more accurate than the clinical intuition of an experienced physician (Mandeville, 2011). Alvarado scores and PAS scores of greater than 7 and 6, respectively, place patients at significantly greater risk for appendicitis but still only achieve sensitivities of 76% and 88%, respectively (Mandeville, 2011).

The application of CDRs in acute appendicitis must therefore fit into a larger picture that includes diagnostic imaging and observation. Ultrasound should be considered a primary modality for the diagnosis of acute appendicitis, in light of the radiation exposure risks associated with CT scans. In children, ultrasound has been reported to have an overall sensitivity and specificity of 90% and 97%, respectively, with an overall accuracy of 96% (7,8). Ultrasound’s greatest liability, however, is the variability of its performance across different users and at different centers. Rates of visualization of the appendix, even at experienced pediatric institutions, is only 56%, and at less experienced centers the rate can be as low as 26% (9). Likewise, the sensitivity of ultrasound may vary widely at different centers, ranging from 69% to 86% in experienced centers and from 35% to 52% in institutions where ultrasound is used less frequently or is less available. Specificity, however, appears to be similar across centers, ranging from 96% to 99% (9). Thus, if a normal appendix can be visualized, appendicitis can be ruled out. If the appendix is not visualized, however, or the findings are equivocal, additional evaluation is warranted.

Relative to ultrasound, CT of the abdomen is more sensitive and nearly as specific, with sensitivity and specificity both >95% and a negative predictive value of 97% (8,10). CT also has been shown to reduce the number of inpatient prediagnosis observation days, operations, and negative laparotomies (11). To balance the superior and more consistent test characteristics of CT against the radiation risk it entails, clinical pathways have been developed that couple CDRs with imaging that stratifies patients and selects imaging modalities according to the likelihood of disease. This maximizes the use of ultrasound as a first-line modality and reserves the use of CT for only the most equivocal cases, which increases its yield. Fleischman et al. published such a pathway that produces a nearly 50% reduction in CT use, from 13% to 7% of cases. Interestingly, their study showed that the accuracy of either a high-risk or a low-risk scoring system was not increased over the clinical impression of an experienced clinician (12).

The newest modality for imaging for appendicitis in young children is focused magnetic resonance imaging (MRI) scanning. Although there are as yet only limited studies (13,14), it appears that MRI provides a sensitivity comparable to that of CTs, but without the radiation exposure. Limitations of MRI include its decreased availability in most medical centers and prolonged scanning time. It should be noted, however, that a less-than-10-minute scanning protocol also appears to be diagnostically successful (15).

Indicated laboratory studies include a complete blood count with differential and urinalysis. Moderate leukocytosis (10,000–18,000 white blood cells [WBC] per mL) with a predominance of polymorphonuclear cells is classically seen but does not necessarily correlate with the severity of the disease (16). A left shift in the differential may precede a total leukocytosis in children. A recent study looked at the use of the WBC and the C-reactive protein (CRP) in the diagnosis of appendicitis in children (17). Used alone, the tests had a sensitivity ranging from 60% to 86%. Used together (either test elevated), there was a sensitivity of 98%, but the 95% CI was as low as 95.3%. It must be noted that a normal WBC or normal CRP does not rule out the diagnosis of appendicitis.

Abdominal radiographs are not particularly helpful, since a fecalith is shown in only 10% to 20% of children with appendicitis. Nonspecific findings on plain abdominal films include isolated distended loops of bowel in the right lower quadrant and obliteration of the psoas shadow. Because lesions that irritate nerves at the T10 to T12 level may simulate the referred pain distribution of appendicitis, a chest film may be required to rule out right lower lobe pneumonia, especially if signs or symptoms of lower respiratory tract disease exist.

There is a higher incidence of perforation in younger children and those with a history of prolonged symptoms. Findings suggestive of perforation include (1) duration of symptoms >36 hours, (2) temperature >102°F, white blood cell count of >15,000/mL, and (3) physical findings of peritonitis.

The surgical consultant should be contacted as soon as the diagnosis of acute appendicitis is entertained, to avoid the complication of late diagnosis and perforation. Perforated appendicitis has a mortality rate of 5% and a morbidity rate of up to 46% in some series, compared with a mortality rate of 0.1% for nonperforated appendicitis (18).

KEY TESTING

• CBC

• Urinalysis

• Ultrasound

• CT only if indicated

• Observation in the face of equivocal findings

ED MANAGEMENT

Appendicitis is a surgical emergency. All children thought to have an abdominal surgical emergency should be seen by a surgeon as soon as possible. The initial emergency department (ED) management of simple and perforated appendicitis is essentially the same. Once the diagnosis is made, the child must be fluid-resuscitated intravenously and kept NPO.

It was previously believed that the use of analgesic medication such as narcotics in a child with abdominal pain would mask the diagnosis and make the evaluation of the child more difficult. Just as with adults, this has been shown to be untrue (19,20). Multiple studies have found no difference in time to surgical decision, number of perforated appendices, or the rate of negative laparotomies when morphine was compared to placebo in children with diagnosed appendicitis (21).

The same can be said for antiemetics in children. It was believed that children given antiemetics such as prochlorperazine or metoclopramide would be overly sedated and would experience more dystonic reactions, making the diagnosis more difficult. However, a survey of pediatricians, emergency physicians, and pediatric emergency physicians reveals that the majority use antiemetics in children who are being evaluated for appendicitis (22). The risk of side effects also appears to be lower than first thought (23).

Ondansetron, a serotonin 5-HT3 receptor antagonist, has been shown to be an effective antiemetic in children presenting to EDs with acute gastroenteritis and other conditions, including appendicitis (24,25).

In a child who has been vomiting, obtaining serum electrolyte levels should be considered while intravenous fluid therapy is started. Children who have clinical signs of dehydration should receive 0.9 NaCl or Ringer lactate boluses at 20 mL/kg to maintain urine output at 1 to 2 mL/kg/hr. Once the child is considered fluid-resuscitated, dextrose 5% in 0.45 normal saline and 20 mEq/L KCl at 1.5 times maintenance should be given, as long as the child is not oliguric. If the child is febrile, an antipyretic can be given orally with a small amount of water allowed.

The use of routine antibiotic coverage in children with uncomplicated appendicitis continues to be debated. It appears that short-term perioperative antibiotic prophylaxis in children with acute nonperforated appendicitis may decrease the rate of postoperative infectious complications (26). Absolute indications for extended therapy and a broader spectrum of antibiotic coverage are systemic signs of toxicity, sepsis, suspected perforation, and the presence of underlying conditions predisposing the patient to complications of bacteremia (i.e., infants younger than 6 months of age and children with congenital or acquired cardiac anomalies, sickle cell disease, diabetes mellitus, chronic renal failure, or other states of immune suppression). One commonly used regimen includes ampicillin, gentamicin, and clindamycin. This regimen ensures coverage of aminoglycoside-resistant enterococci, gram-negative bacilli, and anaerobes, respectively. Monotherapy or other antibiotic combinations can be used as long as the same microbial coverage is obtained in the setting of acute uncomplicated disease. Single-agent treatment with ceftriaxone, piperacillin/tazobactam, or cefoxitin being used with increasing frequency. These appear to be equally efficacious and may result in shortened lengths of stay and reduced hospital charges.

CRITICAL INTERVENTIONS

• Maintain a high index of suspicion for appendicitis in all children with abdominal pain. A retrocecal appendix may cause back or flank pain, a pelvic appendix may cause suprapubic pain, and a retroileal location may cause pain that is referred groin or scrotum.

• Stratify patients and select imaging that balances test performance against the risks of ionizing radiation. After equivocal test results, observation is a valid test.

• Administer 0.9 NaCl or Ringer lactate boluses at 20 mL/kg in children with clinical signs of dehydration to maintain urine output at 1 to 2 mL/kg/hr.

• Obtain a surgical consult as soon as possible to avoid the complications associated with late diagnosis and perforation.

DISPOSITION

All children with appendicitis should be admitted to the hospital for surgical exploration. Laparoscopic appendectomy may be an option is some situations. In certain cases of appendiceal abscess, a surgeon may opt for admission to the hospital for intravenous antibiotics or percutaneous drainage, with an interval appendectomy several weeks later.

If there is doubt about the correct diagnosis but the suspicion for appendicitis is still high, the child should be admitted to the hospital for serial physical examinations. If the suspicion is low but still a consideration, the child may be discharged with clear instructions to return in 6 to 8 hours for a reexamination of the abdomen. If there is any doubt about the ability of the child to return for a revisit, the child should be admitted to the hospital for serial physical examinations.

The child may require transfer to another facility if appropriate surgical staff is unavailable at the initial hospital. This should be done expediently after resuscitation has been started.

Common Pitfalls

• Failure to appreciate atypical presentations of acute appendicitis in children.

• Failure to consider urologic or gynecologic conditions in children.

• Failure to appreciate risk-benefit ratios of different imaging modalities and how they affect decision-making.

• Failure to appreciate the importance of observation as a valid diagnostic test.

REFERENCES

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