Infantile choledochal cyst presenting with an epigastric bilioma: An iceberg phenomenon

INTRODUCTION: Choledochal cysts (CC) are rare congenital biliary dilatations that are capable of presenting with a gamut of clinical findings. Perforation, a rather rare presentation, can account for 1.8–7% of cases. In an infant with non-specific abdominal complaints and a vaguely palpable upper abdominal mass, a perforated CC may be overlooked, leading to a delayed or misdiagnosis and a detrimental outcome. CASE PRESENTATION: We describe a 10-month-old girl who presented to the office with upper abdominal pain and associated fullness for two weeks. An abdominal ultrasonogram revealed perihepatic collections and an evident dilatation of the common bile duct. The bile aspirated from the collections prompted pigtail drainage as an emergency. Magnetic resonance cholangiopancreatography later revealed a spontaneously perforated CC with residual bilioma. Elective resection after six weeks, followed by bilio-enteric reconstruction, resulted in an uneventful recovery. CLINICAL DISCUSSION: Biliary peritonitis and localized biliomas are rare forms among the wide range of findings that can accompany CC. Satisfactory outcomes can be achieved with a two-stage procedure that entails biliary drainage with sepsis control, followed by resection of the CC and bilio-enteric reconstruction a few weeks later. CONCLUSION: It is important to be aware of the spectrum of findings that CC may present. We discussed our experience successfully treating a perforated type IVa CC.