期刊
JOURNAL OF SURGICAL CASE REPORTS
卷 2022, 期 7, 页码 -出版社
OXFORD UNIV PRESS
DOI: 10.1093/jscr/rjac307
关键词
Alagille syndrome; biliary diversion; cholecystocolostomy; cholestasis
类别
资金
- Chairman's Fellowship, Department of Surgery, Boston Children's Hospital
Alagille syndrome is a disorder characterized by increased cholesterol and bile acids in the blood, leading to debilitating xanthomas and pruritus. This study found that Roux-en-Y cholecystocolostomy can be an effective treatment for AGS that is refractory to medical management.
Alagille syndrome (AGS) is a disorder that leads to increased serum cholesterol and bile acids, which can result in debilitating xanthomas and pruritus. External biliary drainage and transplantation are effective treatments for AGS. Internal biliary diversion with Roux-en-Y cholecystocolostomy has been described for other biliary conditions, but not AGS. Three patients with severe pruritus due to AGS underwent Roux-en-Y cholecystocolostomy for internal biliary drainage. Retrospective analysis compared preoperative and post-operative lab values and symptom scores (0, none-4, severe). Three patients underwent cholecystocolostomy. All patients had at least three diagnostic criteria for AGS. Mean preoperative pruritus score was 3.33 (range, 2-4) and mean post-operative score was 1. Mean preoperative xanthoma score was 1.33 (range, 0-4) and post-operative score was 1 at 2-month follow-up. Roux-en-Y cholecystocolostomy can be considered for AGS, which is refractory to medical management. This procedure accomplishes internal biliary diversion without significant physiologic derangements.
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