DISORDERS OF GALLBLADDER

Embryologically, biliary tree develops from a ventral evagination of the primitive foregut into septum transversus and includes biliary canaliculi, and ductules, hepatic ducts, gallbladder and common bile duct, which ultimately opens at ampulla of Vater in duodenum along with pancreatic duct.

Physiologically, biliary tree transports the hepatic products, i.e. bile to the gut, while gallbladder acts as a reservoir.

Obstructive jaundice is the pathognomonic mani­festation of biliary tract disorders (Ch 15.3). While neonatal cholestatic syndromes have been discussed elsewhere (Ch 12.14), other important biliary tree disorders in older children are as follows:

Acute hydrops is the sudden distension of gallbladder in absence of stones, congenital malformations and bacterial infection. Round worms, prolonged fasting, total parenteral nutrition and enteric fever are common causes of acute hydrops, presenting as right upper quadrant pain, tenderness (Murphy sign) and palpable mass, with or without fever, vomiting and jaundice.

USG reveals distended echo-free gallbladder without dilatation of biliary tree and most cases may be managed conservatively.

Acute acalculous cholecystitis is uncommon in children, seen as a part of generalized septicemia due to any cause, (e.g. enteric fever) or following blunt abdominal trauma. While clinical features are usually indistinguishable from acute hydrops, presence of high fever with chills, primary septicemia and leukocytosis indicate infective etiology and need for surgical exploration. Diagnosis is usually confirmed on laparotomy and cholecystectomy is often needed.

Cholelithiasis (gallstones): Gallstones in children are commonly pigment stones (gt;70-80%), seen in cases with: (a) hemolytic anemia, (b) chronic liver disease, e.g. Wilson disease, (c) history of neonatal cholestasis, and (d) prolonged total parenteral nutrition.

Clinically, these cases may be asymptomatic or present with recurrent upper right quadrant abdominal pain and/or intolerance to fatty foods.

Diagnosis is confirmed on USG and cholecystectomy is the treatment of choice along with operative cholan­giography to exclude other calculi in common bile duct. Presence of gallstones in the bile duct is often associated with acute biliary colic and obstructive jaundice, best diagnosed on MRCP or ERCP. Hemolytic anemia should be excluded in all cases.

Choledochal cysts, i.e. congenital cystic dilatations of common bile duct, present with recurrent cholestatic jaundice, progressive biliary obstruction or biliary cirrhosis. A diverticula of the common bile duct or dilatation of intra-duodenal portion of common duct is termed as choledochocele. Choledochal cysts are classified from Type 1-5 as per Todani classification, the type 5 termed as Caroli disease with intrahepatic cystic lesions. Caroli syndrome refers to caroli disease with congenital hepatic fibrosis.

Clinically, most cases present in childhood with a triad of-abdominal pain, right quadrant mass and cholestatic jaundice. Young infants and children may also present with biliary peritonitis due to spontaneous bile duct perforation.

Diagnosis rests of USG, though MRCP is advised before surgery to delineate the lesions.

Treatment involves primary excision of cyst with Roux-en-Y choledochojejunostomy, advocated even in asymptomatic cases due to risk of malignant trans­formation in later life.

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