간동맥 색전술과 간엽 절제술로 치료한 영아 간내 혈관내피종 1례

A Case of Infantile Hemangioendothelioma of the Liver Treated with Hepatic Embolization and Lobectomy

  • 김재선 (을지의과대학교 노원을지병원 소아과) ;
  • 문수경 (을지의과대학교 노원을지병원 소아과) ;
  • 윤혜선 (을지의과대학교 노원을지병원 소아과) ;
  • 이태석 (을지의과대학교 노원을지병원 소아외과)
  • Kim, Jae Seon (Department of Pediatrics, School of Medicine, Eulji University) ;
  • Moon, Soo Kyung (Department of Pediatrics, School of Medicine, Eulji University) ;
  • Yoon, Hye Seon (Department of Pediatrics, School of Medicine, Eulji University) ;
  • Lee, Tae Seok (Department of Pediatric Surgery, School of Medicine, Eulji University)
  • 투고 : 2004.12.24
  • 심사 : 2005.03.08
  • 발행 : 2005.06.15

초록

저자들은 신생아 시기에 심부전 증세를 보인 간내 혈관내피종 환아에서 내과적 치료와 간동맥 색전술 후 호전을 보이지 않아 간좌엽 절제술을 시행하여 치료한 1례를 경험하였기에 보고하는 바이다.

Infantile hemangioendothelioma(IHE) of the liver is the most common vascular tumor in infants before the age of 6 months. It is a histologically benign tumor with potentially life-threatening complications. The clinical manifestations are variable, ranging from asymptomatic forms to intractable high-output heart failure. In addition, abdominal mass, intraperitoneal hemorrhage due to rupture of mass, respiratory distress, hematologic abnormalities and jaundice can occur. Diagnostic work-up is through doppler ultrasound sonography, computed tomography scan, magnetic resonance imaging and angiography. Treatment consists of medical treatment, interventional therapy, surgical resection and liver transplantation. We experienced symptomatic IHE in a premature neonate who presented with high output heart failure and respiratory distress. Initial medical treatment and steroid therapy failed to improve his condition. Coil embolization of left hepatic artery resulted in improvement of respiratory symptoms. However, a left lobectomy was performed because the mass size was not decreased with development of collateral vessels. The infant was well, after a successful discharge from the hospital.

키워드

참고문헌

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