Department of Pediatrics, GMC Srinagar, India.
Department of Pediatric Gastroenterology, Indraprastha Apollo Hospitals, New Delhi, India.
Indian Pediatr. 2019 Nov 15;56(11):965-967.
We performed a review of case records of children diagnosed with hepatic venous outflow tract obstruction at our center in last 10 years. Out of 11 cases identified, 6 had variable blocks in the hepatic venous system and 4 had combined hepatic venous and inferior vena cava (IVC) block. One child with paroxysmal nocturnal hemoglobinuria (PNH) had isolated IVC involvement. Angioplasty was attempted in 3 patients; among them 2 had successful outcome. Seven children with advanced liver disease underwent transplantation, which was successful in six. With availability of modalities like interventional radiology and transplantation, the overall prognosis of hepatic venous outflow tract obstruction seems to be good when managed in a well-equipped center.
我们对过去 10 年来在我们中心诊断为肝静脉流出道梗阻的患儿的病历进行了回顾。在确定的 11 例中，6 例存在肝静脉系统的可变阻塞，4 例存在肝静脉和下腔静脉（IVC）联合阻塞。1 例阵发性夜间血红蛋白尿（PNH）患儿仅有孤立的 IVC 受累。对 3 例患儿进行了血管成形术尝试；其中 2 例取得了成功。7 例晚期肝病患儿接受了移植，其中 6 例成功。随着介入放射学和移植等手段的出现，在设备齐全的中心进行治疗时，肝静脉流出道梗阻的总体预后似乎较好。
