Appendicitis is the most common reason for abdominal surgery in children in the United States.

Although it can occur at any age, it is most commonly diagnosed between 10 and 12 years of age and occurs more often in males than females.

Perforation rates may be as high as 60% in children <5 years of age and 100% in infants.

Appendicitis is thought to occur from the occlusion of the appendiceal lumen with an appendicolith or fecalith (feces which has formed a stone), lymph node proliferation, ingested foreign body, tumor, or parasitic infection.

The trajectory of appendicitis has been described as acute progressing to suppurative with further progression to gangrenous, and eventual perforation, and finally abscess formation.

Two major types: nonperforated or acute, and perforated.

Perforated appendix results in increased morbidity, with the diagnosis often made upon presence of an intra-abdominal abscess on diagnostic imaging.

The classic clinical presentation consists of the report of periumbilical pain with progression to right lower quadrant pain (McBurney point) accompanied by anorexia and eventually vomiting and diarrhea.

Nearly 50% of children will have atypical presentations.

A recent systematic review of clinical prediction rules for children with acute abdominal pain concluded that the Pediatric Appendix Score and the Modified Alvarado score were the most validated (Kulik, Uleryk, & Maguire, 2013).

Common signs and symptoms include: fever (more common with perforation), decreased activity level, point tenderness at McBurney point (anatomic marker for the usual location of the appendix, which is one third the distance along a line from the umbilicus to the right anterior iliac spine), and rebound tenderness related to peritonitis associated with perforation.

Specific signs of appendicitis.

Laboratory.

Radiologic evaluation.

Equivocal examination findings.

Acute nonperforated.

Perforated.

Although the majority of abdominal masses in children are benign conditions (e.g., organomegaly, constipation, umbilical hernia), approximately one third of the cancers in children <16 years of age are intra-abdominal in origin.

Benign cystic masses and the most common malignant abdominal masses including neuroblastoma, Wilms tumor, and hepatoblastoma will be described.

Benign cystic lesion.

Neuroblastoma is the most common extracranial tumor in children, age at presentation is 18 months, with the prevalence greatest in children <4 years (85%).

Wilms tumor is the most common renal tumor and the fifth most common pediatric malignancy, with a common presentation age of 1 to 5 years.

Hepatoblastoma is the most common malignant liver tumor with a mean age at diagnosis 1 year, occurring in the right lobe of the liver and associated with extreme prematurity, very low birth weight, Beckwith-Wiedemann syndrome, Gardner syndrome, and familial adenomatous polyposis disease.

Presentations are variable, and commonly, a mass is noted incidentally by a parent, caregiver, or health professional with an asymptomatic child.

General symptoms include: abdominal pain, abdominal distension, constipation, and anorexia.

Tumor-specific symptoms: Refer to Chapter 10 for details of malignant abdominal masses.

Radiologic—After the diagnosis of a mass is made (clinical examination, abdominal flat plate, ultrasound, CT), consider obtaining any additional imaging at a referral center with pediatric oncology, surgery, and radiology to minimize radiation exposure and optimize the required evaluation for the suspected lesion.

Tumor-specific imaging.

CBC, comprehensive metabolic panel, α-fetoprotein, serum ferritin, and tumor markers as indicated by suspected lesion. See tumor-specific oncological studies in section 10.

Pathology.

Benign cystic lesions.

Malignant masses.

The incidence of biliary atresia (BA) is 1:10,000 live births with approximately 300 new cases diagnosed each year in the United States.

Specific etiology remains unknown and thought to be more than one type of this diagnosis.

Davenport (2012) contends there are four types: (1) syndromic BA and associated malformations (i.e., BA splenic malformation syndrome, cat-eye) and random malformations (e.g., esophageal atresia (EA), jejunal atresia, malrotation), (2) cystic BA—cystic change in an obliterated biliary tract, (3) cytomegalovirus-associated BA, in which the infants have positive serology, (4) isolated BA (largest group of infants).

Proposed nongenetic etiologies: infection, intrauterine infection, toxin exposure.

Physical examination findings: jaundice, acholic stool, and dark urine.

Laboratory findings: Fractionated bilirubin will reveal a conjugated hyperbilirubinemia, elevated liver transaminases from hepatocellular injury, and additional studies may be done to evaluate for other infectious causes (e.g., TORCH infections, viral hepatitis) and metabolic causes of cholestatic jaundice.

Radiologic evaluation.

Kasai procedure or portoenterostomy.

Medical therapy for children with chronic disease.

Treatment of cholestasis.

Treatment of portal hypertension.

Liver transplantation.

There is an increase in gallbladder disease in children mainly attributed to improved diagnostic modalities (ultrasound) and the obesity epidemic.

Known spectrum of gallbladder disease ranging from biliary colic/dyskinesia, cholelithiasis, acute acalculous cholecystitis, choledocholithiasis, and cholangitis.

Acute cholecystitis is often attributed to the presence of gallstones; however, there are a myriad of etiologies that contribute to gallstone disease for which the patient may or may not develop cholecystitis or experience an acute illness but undergo cholecystectomy as an elective procedure.

Acalculous is most commonly caused by sepsis or a severe infection.

Calculous from nonhemolytic cholelithiasis; formation of gallstones in the absence of a hemolytic disease.

Calculous, from hemolytic cholelithiasis; bile in these patients has an increased amount of unconjugated bilirubin, leading to the formation of gallbladder sludge, resulting in an increased risk of cholelithiasis.

Acalculous—fever, vomiting, right upper quadrant pain, and positive Murphy sign (pain on deep inspiration when the inflamed gallbladder is palpated).

Calculous—with cholelithiasis—“silent stones” that are recognized incidentally on imaging but without symptoms.

Range of presentation with fever, right upper quadrant/abdominal pain, positive Murphy sign, vomiting.

Laboratory.

Radiologic evaluation.

Acalculous.

Calculous.

Type A—EA without fistula.

Type B—EA with proximal fistula.

Type C—EA with distal fistula; most common type.

Type D—EA with proximal and distal fistulas.

Type E—Tracheoesophageal fistula without atresia.

Newborn with excessive oral secretions, drooling, accompanied by coughing, choking, or sneezing.

Feeding can cause cyanosis, choking, and emesis.

Failure to pass NG or orogastric tube into the stomach.

Chest radiograph—anteroposterior and lateral, which demonstrates NG tube coiled in upper esophagus.

Assess for VACTERAL (Vertebral, Anorectal, Cardiac, Tracheoesophageal, Renal, and Limb anomalies) association.

Surgery.

Obstruction of proximal duodenum secondary to failure of recanalization.

Associated with Trisomy 21; approximately 30% of patients with duodenal atresia will have Trisomy 21.

Bilious emesis in the first hours of life.

Abdominal radiograph—double bubble sign representing the stomach and proximal duodenum (Figure 15.3).

Surgery.

Esophageal.

Gastric/Intestinal.

Esophageal: dysphagia, emesis, food refusal, salivation, coughing, choking, gagging, or asymptomatic.

Gastric/intestinal: typically present after a witnessed or reported event.

Anteroposterior radiograph of chest and/or abdomen.

Esophageal: endoscopy for removal of foreign body.

Occurs when reflux is associated with symptoms or complications.

Risk factors: neurologic impairment, obesity, repaired EA or other congenital esophageal disease, cystic fibrosis, hiatal hernia, repaired achalasia, family history of gastroesophageal reflux disease (GERD).

Primary cause is transient relaxation of the lower esophageal sphincter, which allows gastric contents to move into the esophagus.

Poor weight gain, feeding aversion.

Unexplained crying, choking, or coughing.

Sleep disturbances.

Gagging.

Regurgitating.

Dental erosion (older child).

Dystonic head positioning (Sandifer syndrome).

Abdominal or chest pain (older child).

Upper GI: evaluates for anatomical malformations; not sensitive or specific for GERD.

pH probe: measures lower esophageal acidity over 24 hours; helpful in measuring esophageal acidity exposure, but does not establish relationship with symptoms or disease in most cases; does not measure nonacid reflux.

Combined multiple intraluminal impedance study and pH monitoring: measures both acid and nonacid exposure and is superior to pH monitoring alone in establishing a temporal relationship with symptoms and disease.

Endoscopy and biopsy: not sensitive for diagnosis of GERD, but can exclude other esophageal complications or disorders such as eosinophilic esophagitis.

Radionuclide scintigraphy (milk scan): not specific for diagnosis of GERD; useful in measuring gastric emptying.

Empiric trial of acid suppression: Expert opinion suggests an empiric trial of a proton pump inhibitor in the older child or adolescent with typical symptoms for up to 4 weeks is justified, though not specific for diagnosis of GERD.

Nonpharmacologic.

Medical management.

Surgical management.

Viral.

Bacterial.

Parasitic.

History often includes travel, sick contacts, recent antibiotic use.

Vomiting, diarrhea, and fever are the hallmark symptoms, but all three may not be present.

Normal bowel movement but with increased frequency and larger water content.

Stool output of >3 bowel movements/day.

Symptoms may also include myalgias, headaches, and/or fatigue.

Refer to Section 8 (Fluid and Electrolytes) for classification, evaluation and assessment of dehydration.

Diagnosis can be made from the history and physical examination.

For diarrhea that is bloody and/or persistent, obtain:

Minimal to no clinical dehydration.

Mild to moderate dehydration in acute care setting.

Severe dehydration in acute or intensive-care setting.

Antibiotics may be indicated in certain bacterial infections; contraindicated in E. coli 0157:H7.

Antipyretics and antiemetics can be useful as supportive care.

Hand hygiene is of paramount importance in preventing spread of disease.

Upper GI tract.

Lower GI tract.

Upper GI tract.

Lower GI tract.

Upper GI bleed.

Lower GI bleed.

Hemodynamically unstable (upper & lower GI bleeds).

Hemodynamically stable upper GI bleed.

Hemodynamically stable lower GI bleed: management is dependent on etiology.

Liver provides many functions in the body, including (1) metabolism of glucose, lipid, nitrogen, drugs, and toxins; (2) Synthesis of albumin and coagulation factors; (3) formation of bile and bile acids.

Idiopathic.

Viral.

Bacterial.

Toxins—alcohol, drugs: acetaminophen, nonsteroidal anti-inflammatory drugs, ACE inhibitors, nicotinic acid, isoniazid, sulfonamides, erythromycin; griseofulvin and fluconazole.

Autoimmune.

Metabolic/Genetic—α1-antitrypsin deficiency, Tyrosinemia, Galactosemia, Hypothyroidism, Progressive familial intrahepatic cholestasis.

Steatohepatitis and Nonalcoholic steatohepatitis.

Secondary to comorbidity—(e.g., cardiac, thyroid).

PN-associated cholestasis.

Asymptomatic with abnormalities in laboratory values.

Acute or chronic symptoms: chronic condition can present with acute changes.

General symptoms: fever, malaise, anorexia, nausea, vomiting, abdominal pain, diarrhea. jaundice, joint pains, low-grade fever.

Physical examination findings: jaundice, hepatomegaly, abdominal tenderness.

Liver function tests are not true tests of liver function. These tests are indirect markers of hepatobiliary disease.

Laboratory evaluation.

Imaging.

Liver biopsy.