Abstract: Caroli syndrome, also known as congenital cystic dilatation of intrahepatic bile duct accompanied by congenital liver fibrosis, is a rare congenital disease in children. For patients with diffuse disease involvement, orthotopic liver transplantation is the choice of treatment. This casewas a 6-month-old female infant, development lagging behind, weighsonly 3.8 kg, had abdominal distension, with massive ascites and anemia, In order to prolong the waiting time for liver transplantation, she plans to undergo laparoscopic choledochal cyst resection and hepatic duct jejunostomy. Anesthesia management is challenging. Now we will report an anesthesia case of Caroli syndrome diagnosed and treated in our hospital and do a literature review, and discuss the anesthesia management of low birth weight children.

Key words: caroli syndrome, low birth weight children, anesthesia management

摘要： Caroli综合征又称先天性肝内胆管囊性扩张症并伴有先天性肝纤维化,是一种罕见的小儿先天性疾病。对于弥漫性病变累及的患者,选择的治疗方法是原位肝移植。本例为6月龄女婴,发育严重落后,体重仅为3.8kg,腹胀明显,合并大量腹水、贫血,为延长等待肝移植的时间,拟行腹腔镜下胆总管囊肿切除后肝总管空肠吻合术,使麻醉管理具有挑战性,现将我院诊治的一例Caroli综合征的麻醉汇报并做文献复习,并探讨低体重患儿的麻醉管理。

关键词: Caroli综合征, 低体重患儿, 麻醉管理