Biliary dilatation secondary to lithiasis in a child affected by Langerhans' cell histiocytosis

Abstract

Langerhans' cell histiocytosis (LCH) is a disease of unknown pathogenesis, caused by clonal proliferation of Langerhans' cells. Liver involvement results in a cholangiopathy, which has the radiologic appearance of sclerosing cholangitis. Only 1 case of obstructive jaundice due to common bile duct stone in a patient with LCH has been described. We present a case of a 31-month-old child with LCH and liver involvement on the waiting list for liver transplantation. During the follow-up, there was a rapid onset of jaundice due to sludge and lithiasis. The patient was treated first with an endoscopic biliary plastic stent and then with percutaneous biliary drainage and bilioenteric anastomosis.

Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

“…Authors of previous studies have reported jaundice in case of LCH in association with hydrops fetalis [11] in a congenital systemic Langerhan cell histiocytosis variant causing excessive hemolysis, hyperbilirubinemia, hypoalbuminemia and failure to thrive in a neonate; as reported by Boccon-Gibod et al, [12]. LCH may also be associated with liver dysfunction with a radiologic appearance of sclerosing cholangitis due to cholangiopathy and stone in the common bile duct causing obstructive jaundice [13]. One case study reports the presence of biliary wall calcification in association with periductal fibrosis and periportal fibrous septa with nodules; consistent with biliary cirrhosis causing jaundice [14].…”