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Chapter 358 Diseases of the Gallbladder
Frederick J. Suchy
The gallbladder is congenitally absent in about 0.1% of the population. Hypoplasia or absence of the gallbladder can be associated with extrahepatic biliary atresia or cystic fibrosis. Duplication of the gallbladder occurs rarely. Gallbladder ectopia can occur with a transverse, intrahepatic, left-sided, or retroplaced location. Multiseptate gallbladder, characterized by the presence of multiple septa dividing the gallbladder lumen, is another rare congenital anomaly of the gallbladder.
Table 358-1 CONDITIONS ASSOCIATED WITH HYDROPS OF THE GALLBLADDER
Acute noncalculous, noninflammatory distention of the gallbladder can occur in infants and children. It is defined by the absence of calculi, bacterial infection, or congenital anomalies of the biliary system. The disorder can complicate acute infections, but the cause is often not identified. Hydrops of the gallbladder can also develop in patients receiving long-term parenteral nutrition, presumably as a result of gallbladder stasis during the period of enteral fasting. Hydrops is distinguished from acalculous cholecystitis by the absence of a significant inflammatory process and a generally benign prognosis.
Affected patients usually have right upper quadrant (RUQ) pain with a palpable mass. Fever, vomiting, and jaundice may be present and are usually associated with a systemic illness such as streptococcal infection. Ultrasonography shows a markedly distended, echo-free gallbladder, without dilatation of the biliary tree. Acute hydrops is usually treated conservatively with a focus on supportive care and managing the intercurrent illness; cholecystostomy and drainage are rarely needed. Spontaneous resolution and return of normal gallbladder function usually occur over a period of several weeks. If a laparotomy is required, a large, edematous gallbladder is found to contain white, yellow, or green bile. Obstruction of the cystic duct by mesenteric adenopathy is occasionally observed. Cholecystectomy is required if the gallbladder is gangrenous. Pathologic examination of the gallbladder wall shows edema and mild inflammation. Cultures of bile are usually sterile.
Acute acalculous cholecystitis is uncommon in children and is usually caused by infection. Pathogens include streptococci (groups A and B), gram-negative organisms, particularly Salmonella and Leptospira interrogans. Parasitic infestation with ascaris or Giardia lamblia may be found. Calculous cholecystitis rarely follows abdominal trauma or burn injury or is associated with a systemic vasculitis, such as periarteritis nodosa.
Clinical features include RUQ or epigastric pain, nausea, vomiting, fever, and jaundice. RUQ guarding and tenderness are present. Ultrasonography discloses an enlarged, thick-walled gallbladder, without calculi. Serum alkaline phosphatase (ALP) activity and direct-reacting bilirubin levels are elevated. Leukocytosis is usual.
Patients can recover with treatment of systemic and biliary infection. Because the gallbladder can become gangrenous, daily ultrasonography is useful in monitoring gallbladder distention and wall thickness. Cholecystectomy is required in patients who fail to improve with conservative management. Cholecystostomy drainage is an alternative approach in a critically ill patient.
Cholelithiasis is relatively rare in otherwise healthy children, occurring more commonly in patients with various predisposing disorders (Table 358-2
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