Successful management of infantile hepatic hilar hemangioendothelioma with obstructive jaundice and consumption coagulopathy

Kaposiform hemangioendothelioma of the choledochus

“…Involvement of the choledochus by hemangioendothelioma (not mentioned as kaposiform) has been reported in 2 cases [9,10]. They were unlikely KHE according to the description and posted microscopic photographs, so the present case is likely the first report of choledochal involvement by KHE.…”

“…Although there might be options to treat this condition by medical therapy as first-line management, we chose to resect the tumor to obtain certain drainage of the biliary tract and relieve the biliary obstruction and to confirm the diagnosis. In some case reports of hemangioendothelioma presenting with biliary obstruction (biliary or pancreatic origin) [12], conservative therapy using drainage of the bile duct has been performed successfully [10,13]. This therapeutic strategy is based on the concept that almost all hemangioendothelioma in early childhood will naturally regress spontaneously.…”

A Rare Cause for Extrahepatic Biliary Tract Obstruction: Juvenile Hepatic Hilar Hemangioma

“…In conclusion, obstructive jaundice due to extrahepatic compression of the bile duct system can be caused by different tumors. [1][2][3] Our report describes the rare and previously not described entity of juvenile hemangioma. This etiology of extrahepatic bile duct obstruction should be suspected as differential diagnosis in those cases where extrahepatic biliary atresia or inspissated bile syndrome can be ruled out.…”

“…However, those tumors causing obstructive jaundice have been rarely reported in the literature so far. [1][2][3] This unique case highlights that extrahepatic bile duct obstruction can be caused by a juvenile hemangioma atypically located in the hepatic hilum. As in general, therapeutic options include percutaneous transhepatic drainage as well as bypass surgery or biliaryenteric anastomosis.…”

Successful surgical treatment of an infant with infantile hepatic hemangioendothelioma

“…Semptomatik olanlarda ise tedavi seçenekleri; genellikle kişisel olarak hastaya göre değerlendirilir. İHHE'de tedavi endikasyonları kalp yetmezliği, solunum zorluğu, koagülopati, abdominal kompartıman sendromu ve hepatik fonksiyon testlerinin bozulmasıdır (16) . Tedavi gerekliyse, medikal tedavi önceliklidir ve eğer yanıt alınamıyorsa invazif/ cerrahi müdahaleler zorunludur.…”