A Case of Hemangioendothelioma of the Porta Hepatis With Obstructive Jaundice

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  • Yamamori Nobuko
    First Department of Surgery, Nagoya City University Medical School
  • Hashimoto Takashi
    First Department of Surgery, Nagoya City University Medical School
  • Simizu Yasunobu
    First Department of Surgery, Nagoya City University Medical School
  • Otobe Yoshihiro
    First Department of Surgery, Nagoya City University Medical School
  • Nakamura Tukasa
    First Department of Surgery, Nagoya City University Medical School
  • Minami Munehito
    First Department of Surgery, Nagoya City University Medical School
  • Yura Jiro
    First Department of Surgery, Nagoya City University Medical School

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  • 胆道閉鎖症の病態を呈した肝門部血管内皮腫の1治験例

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Abstract

A case of hemangioendothelioma originated in the porta hepatis with the symptom mimicking to that of biliary atresia is presented, which is the first case ever reported in the world. A 4-month-old female was admitted due to jaundice. Abdominal echogram revealed a distended gallbladder, stenosis of common hepatic duct at porta hepatis surrounded with high echoic area, and dilatation of the left intrahepatic bile duct. Percutaneous cholangiography showed complete obstruction at porta hepatis and normal bifurcation of intrahepatic bile duct. The diagnosis of biliary atresia was made, and the abdomen was explored on 139-days after birth. The hepatoduodenal ligament was invaded by sclerotic mass from porta hepatis to T-junction including cystic duct continuously, and bile duct in its sclerotic portion was obstructed completely. All sclerotic portion was resected, and portoenterostomy (original Kasai's procedure) was performed. The histological apperance of specimen was benign hemangioendothelioma surrounding common hepatic duct, cystic duct, and common bile duct. In a 37-mo follow-up, the child has remained well, and is completely asymptomatic.

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