Abstract

Hemobilia is an aberrant connection of hepatic biliary and vascular systems. It usually occurs post traumatic, blunt trauma or iatrogenic. Non traumatic hemobilia is a rare occurrence which there are few case reports in children which were due to pancreatic pseudocyst, choledochal cyst, hepatic abscess, hepatic fascioliasis and Meta chromatic leukodystrophy. All reported cases are female except one with von Willebrand’s disease. In few patients Meta chromatic leukodystrophy (MLD) was the cause of hemobilia. MLD is a rare neurological disease which is caused by Sulfatids deposition in nervous tissue and it has three subtypes, late infantile, juvenile and adult type. The gall bladder may be involved in the disease course and presents with different symptoms like abdominal pain or hemobilia. We present an eight-year-old girl with late infantile MLD who presented with upper GI bleeding and in evaluation the diagnosis of hemobilia and gall bladder polyps was confirmed.

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