Ileal exclusion for Byler's disease: An alternative surgical approach with promising early results for pruritus
Ileal exclusion for Byler's disease: An alternative surgical approach with promising early results for pruritus
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DOI：
10.1016/s0022-3468(98)90435-3
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发表时间：
1998-02-01
影响因子：
2.4
通讯作者：
Burnweit, CA
中科院分区：
文献类型：
--
作者：
Hollands, CM;Rivera-Pedrogo, FJ;Burnweit, CA
背景/目的：进行性家族性肝内胆汁淤积症(Byler病)通常以瘙痒引起的自残为特征，对药物治疗的反应最小。引起胆汁淤积的患者很可能进展为肝硬变，需要进行移植。部分胆道外分流术已用于治疗黄疸和衰弱性瘙痒，但所有潜在的并发症和长期造口的美学问题都采用了这种方法。方法：作者描述了一种最初为先前接受过胆囊切除的患者开发的末端回肠隔断术。在3年的时间里，我们确定了7名患有拜勒氏病的肝脏组织学特征的儿童，并伴有慢性胆汁淤积的临床照片，但没有明确的代谢或解剖异常。前两名患者接受了胆囊肠皮肤吻合术，到目前为止，这是这种情况的推荐治疗方法。第三例患者以前曾接受过胆囊切除术，因此除小肠的15%远端外，还进行了回结肠吻合术。这名儿童的瘙痒症状完全缓解，没有腹泻的迹象。在标准化这一入路之前，又进行了两次末端回肠切除手术，结果相似。作者使用Siebert‘s曲线图将冠跟长度与小肠长度联系起来，从而近似计算出小肠长度。确定平均值和低于平均值的一个标准差之间的中点，从回盲瓣向后测量估计的小肠长度的15%，然后使用线性吻合器进行分割。在回肠近端和盲肠之间建立了吻合口，绕过了末端回肠。结果：5名儿童中有4名的瘙痒和自残症状得到缓解，没有腹泻的迹象。末端回肠搭桥术提供了一种无吻合口、完全可逆的“胆道分流术”。结论：少数患者的早期结果是有希望的，但在提倡将其作为Byler病的主要治疗方法之前，需要对生长、发育、肝功能和组织学进行长期评估。版权所有(C)1998年，W.B.桑德斯公司。
Background/Purpose: Progressive familial intrahepatic cholestasis (Byler's disease) is often characterized by pruritus-induced self-mutilation with minimal response to medical therapy. The causative cholestasis is likely to progress to cirrhosis necessitating transplantation. Partial external biliary diversion has been used with promising results for the jaundice and debilitating pruritus but all the potential complications and aesthetic concerns of long-term stomas attend this approach.Methods: The authors describe a terminal ileal exclusion that was first developed for patients who had previously undergone cholecystectomy. Over a 3-year period, we identified for study seven children with liver histology characteristic of Byler's disease accompanying a clinical picture of chronic cholestasis without a defined metabolic or anatomic abnormality. The first two patients underwent a cholecystojejunal cutaneous stoma, until now, the recommended treatment for this condition. The third had previously undergone cholecystectomy so an ileocolonic anastomosis was performed excluding the distal 15% of the small bowel. This child had complete relief of pruritus without evidence of diarrhea. Two more terminal ileal exclusions were performed with similar results before standardizing this approach. The authors approximated small intestinal length using Siebert's graph relating crown-heel length to small intestinal length. The midpoint between the mean and one standard deviation below the mean was determined, Fifteen percent of the estimated small bowel length was measured back from the ileocecal valve and then divided using a linear stapling device. A stapled anastomosis was created between the proximal ileum and the cecum, bypassing the terminal ileum.Results: Four of five children have had relief from their pruritus and self-mutilation with no evidence of diarrhea. Terminal ileal bypass offers a stoma-free, completely reversible "biliary diversion."Conclusion: Early results on a few patients are promising, but long-term evaluation of growth, development, and liver function and histology is needed before advocating this as the primary therapy for Byler's disease. Copyright (C) 1998 by W.B. Saunders Company.