Bile Plug Syndrome Mimicking Choledochal Cyst

Abstract

Patients suspected of having neonatal cholestatic hepatitis should be investigated keeping the differential diagnosis in mind. Choledochal cyst is a common surgical condition, where imaging findings, such as those of sonography or magnetic resonance (MR) cholangiography, are important for diagnosis. However, bile plug syndrome (inspissated bile syndrome) is rare and can be the cause of bile duct obstruction or dilatation, mimicking choledochal cyst. Surgical management is not necessary in such cases if the condition is not severe. A two-week-old baby, born at 35 wk of gestation by Cesarean section for fetal distress, was transferred to our neonatal intensive care unit. Cephalohematoma and subacute subdural hemorrhage were noted on the brain MRI. Laboratory tests showed low hemoglobin levels (Hb 7.3 g/dl), elevated reticulocyte count (5.59%), hyperbilirubinemia (total bilirubin 15.5mg/dl, direct bilirubin 12.5 mg/dl), and elevated liver function tests (ALP 1000 IU/L, GGT 439 IU/L, AST/ALT 316/ 204 IU/L). She received packed red blood cells and ursodeoxycholic acid (15 mg/kg/d). It was supposed that brain hemorrhage might have triggered the cholestasis and conjugated hyperbilirubinemia. Abdominal ultrasonography presented common bile duct dilatation of 6 mm sized diameter with sludge (Fig. 1a). Type I choledochal cyst (Todani classification) with sludge was presumed based onMR cholangiography findings (Fig. 1b). Prematurity, hemolysis and possible sepsis supported the diagnosis of bile plug syndrome, although MRCP image showed fusiform choledochal cyst rather than inspissated bile