Drastich Pavel, Oliverius Martin
Hepatogastroenterology Department, Institute for Clinical and Experimental Medicine, Prague, Czech Republic.
Dig Dis. 2017;35(1-2):127-133. doi: 10.1159/000449093. Epub 2017 Feb 1.
Most patients with Crohn's disease (CD) require one or more operations during their lifetime. Repeated resections and surgical complications may result in short gut in a subset of patients, typically those with extensive small bowel disease or a penetrating CD phenotype. The effects of short bowel syndrome (SBS) can range in seriousness from mild to life-threatening advanced intestinal failure. Worldwide, CD is the second leading indication for intestinal transplantation (ITx) in SBS, but the overall incidence of ITx is quite low. Key Messages: Currently, total parenteral nutrition (TPN) is the preferred treatment option for patients with SBS because of its superior survival outcome. However, TPN can fail from loss of venous access due to catheter-associated thromboses, recurrent catheter-related blood stream infections, or intestinal-failure-associated liver dysfunction. Three types of transplantations are available for CD patients - small bowel alone, liver plus small bowel and multivisceral, which includes other intra-abdominal organs. An abdominal wall transplant is required in case of abdominal wall defects or lack of free intra-abdominal space. The current 5-year survival rate of 54% following ITx of the isolated small bowel appears worse than that associated with TPN. However, outcomes are substantially improving because of surgical and technical advances and progress in medical therapy. On the other hand, ITx carries the risk of both complications (e.g., rejection, infections, and post transplant lymphoproliferative disorders) and adverse events associated with immunosuppression. CD recurrence has been reported in a few patients, but this primarily histologic recurrence might not be of great clinical importance.
ITx has become a well-established treatment for those who fail on TPN and who have life-threatening complications. Fortunately, it concerns only a small proportion of CD patients, but it does offer reasonable survival and quality of life. Primary management of patients with small bowel failure should be provided by a center experienced in medical intestinal rehabilitation, nutrition, and transplantation of other solid organs.
大多数克罗恩病(CD)患者在其一生中需要进行一次或多次手术。反复切除和手术并发症可能导致一部分患者出现短肠,尤其是那些患有广泛小肠疾病或穿透性CD表型的患者。短肠综合征(SBS)的影响严重程度不一,从轻度到危及生命的晚期肠衰竭。在全球范围内,CD是SBS患者进行肠移植(ITx)的第二大主要指征,但ITx的总体发生率相当低。
目前,全肠外营养(TPN)是SBS患者的首选治疗方案,因为其生存结果更佳。然而,TPN可能会因导管相关血栓形成、反复的导管相关血流感染或肠衰竭相关肝功能障碍导致静脉通路丧失而失败。CD患者有三种移植类型可供选择——单独小肠移植、肝加小肠移植和多脏器移植(包括其他腹内器官)。如果存在腹壁缺损或腹内无游离空间,则需要进行腹壁移植。目前,孤立小肠ITx术后5年生存率为54%,似乎比TPN相关的生存率更差。然而,由于手术和技术进步以及药物治疗的进展,结果正在大幅改善。另一方面,ITx存在并发症(如排斥反应、感染和移植后淋巴细胞增生性疾病)以及与免疫抑制相关的不良事件的风险。少数患者报告了CD复发,但这种主要为组织学上的复发可能在临床上并不十分重要。
ITx已成为TPN治疗失败且出现危及生命并发症患者的成熟治疗方法。幸运的是,它仅涉及一小部分CD患者,但确实能提供合理的生存率和生活质量。小肠衰竭患者的初始管理应由在医学肠道康复、营养和其他实体器官移植方面经验丰富的中心提供。