Hépatologie Pédiatrique and Centre de Référence National de l'Atrésie des Voies Biliaires, Le Kremlin-Bicêtre, France.
Gastroenterology. 2010 Dec;139(6):1952-60. doi: 10.1053/j.gastro.2010.07.004. Epub 2010 Jul 14.
BACKGROUND & AIMS: Biliary atresia is the most common cause of childhood cirrhosis. We investigated prospectively the development of portal hypertension in 139 children with biliary atresia, the risk of gastrointestinal (GI) bleeding in the first years of life, and associations between endoscopic patterns of varices and risk.
Children with clinical or ultrasonographic signs of portal hypertension underwent upper GI endoscopy examinations (n = 125, median age of 13 months). Information was recorded about esophageal varices and grade, red wale markings on the variceal wall, gastric varices along the cardia, and portal hypertensive gastropathy. A second endoscopy examination was performed in 64 children after a mean interval of 51 months to study their progression or regression.
At the first endoscopy examination, 88 of 125 children had esophageal varices, including 74 who were younger than 2 years. Grade II and III varices, red markings, gastric varices, and signs of gastropathy were present in 29, 30, 24, and 27 children, respectively. At the second endoscopy examination, progression, stability, and regression of endoscopic signs were observed in 37, 18, and 9 of the 64 children, respectively. Twenty-eight children had GI bleeding at a median age of 17 months. Multivariate analysis showed that red markings, and most importantly gastric varices, were independent factors associated with bleeding.
Children with biliary atresia have a high risk of portal hypertension in the first years of life. Spontaneous regression of varices is rare. Children with a combination of esophageal varices and red markings and/or gastric varices along the cardia should receive primary prophylaxis of bleeding.
胆道闭锁是儿童肝硬化最常见的原因。我们前瞻性研究了 139 例胆道闭锁患儿门静脉高压的发展，生命最初几年胃肠道出血的风险，以及内镜下静脉曲张模式与风险的关系。
有门静脉高压临床或超声征象的患儿行上消化道内镜检查（n=125，中位年龄 13 个月）。记录食管静脉曲张和分级、静脉壁红色条纹、贲门胃底静脉曲张和门静脉高压性胃病。64 例患儿平均间隔 51 个月后进行第二次内镜检查，以研究其进展或消退情况。
125 例患儿中有 88 例在第一次内镜检查时存在食管静脉曲张，其中 74 例年龄小于 2 岁。29 例、30 例、24 例和 27 例患儿分别存在Ⅱ级和Ⅲ级静脉曲张、红色标记、胃底静脉曲张和胃病征象。64 例患儿中的 37 例在第二次内镜检查时出现进展、稳定和消退，18 例和 9 例分别稳定和消退。28 例患儿在中位年龄 17 个月时发生胃肠道出血。多变量分析显示红色标记和最重要的胃底静脉曲张是与出血相关的独立因素。
胆道闭锁患儿在生命最初几年有发生门静脉高压的高风险。静脉曲张自发消退罕见。存在食管静脉曲张和红色标记和/或贲门胃底静脉曲张的患儿应进行预防性出血治疗。
