Choledochoduodenal Fistula (CDF) in an Adolescent with Sickle Cell Disease (SS) and Choledocholithiasis

Abstract

Purpose: A 13-year-old African-American female with SS presented with vasoocclusive crisis of the left thigh and one episode of nonbilious emesis, but without fever or abdominal pain. Three weeks prior to presentation, she was discharged from another hospital with a diagnosis of cholelithiasis. Physical exam demonstrated jaundice, thigh tenderness, but no abdominal tenderness or hepatosplenomegaly. Laboratory findings included WBC 15,800, Hgb 9.0, Retic 11.3%, LDH 552 U/L, lipase 390 U/L, amylase 147 U/L, AST/ALT = 91/42 U/L, Total/Direct Bilirubin=38.7/> 10 mg/dL, Alk-P 250 U/L, GGT 55 U/L. MRCP showed cholelithiasis, choledocholithiasis, a dilated common bile duct (CBD=1.3 cm), mild dilatation of the intrahepatic bile ducts, and a dilated pancreatic duct (4 mm). At ERCP, there was a black calculus above the ampulla consistent with a choledochoduodenal fistula (Figure 1). During sphinctertome advancement into the CBD, the calculus was spontaneously removed. Cholangiogram showed the CBD=1.2 cm and a fistula in the distal CBD. A 1 cm sphincterotomy was performed up to the fistula with good drainage. The duct was swept and multiple small calculi were retrieved. At post-operative day 3, the patient developed fever and right upper abdominal pain. Repeat MRCP showed cholelithiasis with pericholecystic fluid, a small amount of perihepatic fluid, and a CBD=4 mm. HIDA scan confirmed the diagnosis of acute cholecystitis. Intravenous antibiotics led to clinical and biochemical improvement. Discussion: CDF is an uncommon complication of long standing choledocholithiasis. CDF in this patient likely arose from stone erosion through the CBD into the duodenum. This is the first pediatric CDF case report in a SS patient. Since cholelithiasis is often seen in SS, one should be aware of this complication in patients presenting with obstructive jaundice and a normal GGT.Figure 1: No Caption available.