Refractory obstructive jaundice in a child affected with thalassodrepanocytosis: a new endoscopic approach

Liver involvement, including elevated direct-reacting bilirubin levels, is common in patients with sickle cell disease. Fifty to seventy percent of sickle cell patients have pigmented gallstones due to precipitation of unconjugated bilirubin, and cholelithiasis or choledocholithiasis are common complications. The highest prevalence of these complications occurs in patients with Gilbert's syndrome because of the combined effect of increased bilirubin production and reduced bilirubin-diphosphate-glucuronosyltransferase enzyme activity. Cholelithiasis is also a common complication in patients with thalassemia. Endoscopic removal of choledochal stones does not always resolve the clinical picture, as in cases of dysfunction of the Vater's papilla, increased bile density due to persistently impaired bile flow or distortion of the choledocus due to dilatation, or inflammation secondary to gallstone.

We report here a case of severe and persistent obstructive jaundice in a child affected with thalassodrepanocytosis and Gilbert's syndrome, previously, and unsuccessfully, treated with endoscopic removal of choledochal stones. Deep and thorough biliary washing, and stenting with a new removable polytetrafluoroethylene (PTFE)-covered flared-type stent led to complete resolution of the obstructive jaundice.

This report shows that an aggressive endoscopic approach in this select category of patients can help resolve the severe complication of hemolytic anemia, thus avoiding surgery.