van Wessel Daan B, Witt Mauri, Bax Niels, Verkade Henkjan J, Scheenstra René, de Kleine Ruben H, Hulscher Jan B
Department of Pediatric Surgery, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands.
Department of Pediatric Gastroenterology and Hepatology, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Groningen, The Netherlands.
Eur J Pediatr Surg. 2018 Oct;28(5):439-444. doi: 10.1055/s-0037-1603987. Epub 2017 Jun 28.
Portal hypertension often occurs in biliary atresia (BA). The subsequent development of esophageal varices and bleeding from these varices are a well-known complication. We aim to describe the incidence and severity of variceal bleeding in patients with BA. In addition, we describe the characteristics of patients who experienced variceal bleeds.
We included all infants treated for BA at our center between March 1987 and August 2015. Variceal bleeding was defined as hematemesis and/or melena with presence of varices at endoscopy. Findings at endoscopy and ultrasound, laboratory tests, clearance of jaundice, fibrosis-grade at Kasai portoenterostomy, and several varices prediction scores were documented. Routine endoscopies were not performed.
In this study, 74 patients were included. During follow-up, 18 out of 74 patients (24%) developed variceal bleeding at an age of 9 months (range, 4-111). Twelve patients were listed for liver transplantation at the time of bleeding. Patients who did not clear their jaundice developed variceal bleeds more often and earlier in life. Bleeds were treated with sclerotherapy, banding, or octreotide. Four patients did not receive treatment. No bleeding-related mortality occurred.
One-fourth of the children diagnosed with BA experience variceal bleeds during follow-up. Most of these children are younger than 1 year and often already listed for transplantation. Major complications did not occur after variceal bleeding.
门静脉高压常发生于胆道闭锁(BA)。随后食管静脉曲张的发展以及这些静脉曲张出血是一种众所周知的并发症。我们旨在描述BA患者静脉曲张出血的发生率和严重程度。此外,我们描述了发生静脉曲张出血的患者的特征。
我们纳入了1987年3月至2015年8月在我们中心接受BA治疗的所有婴儿。静脉曲张出血定义为内镜检查时存在静脉曲张的呕血和/或黑便。记录内镜检查和超声检查结果、实验室检查、黄疸清除情况、肝门空肠吻合术时的纤维化分级以及几种静脉曲张预测评分。未进行常规内镜检查。
本研究纳入了74例患者。在随访期间,74例患者中有18例(24%)在9个月龄(范围4 - 111个月)时发生静脉曲张出血。12例患者在出血时被列入肝移植名单。黄疸未清除的患者发生静脉曲张出血的频率更高且在生命早期发生。出血采用硬化疗法、套扎术或奥曲肽治疗。4例患者未接受治疗。未发生与出血相关的死亡。
四分之一被诊断为BA的儿童在随访期间发生静脉曲张出血。这些儿童大多数年龄小于1岁,且常常已被列入移植名单。静脉曲张出血后未发生重大并发症。