Durgin Jonathan M, Crum Robert, Kim Heung Bae, Cuenca Alex G
Department of Surgery, Boston Children's Hospital, Boston, MA, USA.
J Surg Case Rep. 2022 Jul 4;2022(7):rjac307. doi: 10.1093/jscr/rjac307. eCollection 2022 Jul.
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.
阿拉吉耶综合征（AGS）是一种导致血清胆固醇和胆汁酸升高的疾病，可引发使人衰弱的黄瘤和瘙痒。外引流术和肝移植是治疗AGS的有效方法。Roux-en-Y式胆囊结肠吻合术用于内胆汁转流已在其他胆道疾病中有所描述，但尚未用于AGS。3例因AGS导致严重瘙痒的患者接受了Roux-en-Y式胆囊结肠吻合术进行内胆汁引流。回顾性分析比较了术前和术后的实验室检查值及症状评分（0分，无；1至4分，严重程度递增）。3例患者接受了胆囊结肠吻合术。所有患者至少具备三项AGS诊断标准。术前瘙痒平均评分为3.33（范围2至4），术后平均评分为1。术前黄瘤平均评分为1.33（范围0至4），术后2个月随访时评分为1。对于药物治疗无效的AGS患者，可考虑行Roux-en-Y式胆囊结肠吻合术。该手术可实现内胆汁转流，且不会引起明显的生理紊乱。
