Sigurdsson L, Reyes J, Kocoshis S A, Mazariegos G, Abu-Elmagd K M, Bueno J, Di Lorenzo C
Department of Paediatric Gastroenterology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213-2583, USA.
Gut. 1999 Oct;45(4):570-4. doi: 10.1136/gut.45.4.570.
Children with chronic intestinal pseudo-obstruction (CIPO) often require total parenteral nutrition (TPN) which puts them at risk of liver failure and recurrent line infections. Intestinal transplantation has become a therapeutic option for TPN dependent children with intestinal failure who are failing management with TPN.
To investigate the outcome of children with CIPO referred for intestinal transplantation.
A retrospective review was carried out of records and diagnostic studies from 27 patients with CIPO referred for intestinal transplantation.
Five children were not listed for transplantation: two because of parental decision, two because of suspicion of Munchausen syndrome by proxy, and one because he tolerated enteral nutrition. Six are still TPN dependent and awaiting transplantation. Eight children died awaiting transplantation. Eight children underwent transplantation. Three died (two months, seven months, and four years after transplant). Five children are alive with a median follow up of 2.6 years (range two months to six years). All transplanted children were able to tolerate full enteral feedings. The postoperative course was complicated by dumping syndrome, Munchausen syndrome by proxy, narcotic withdrawal, and uncovering of achalasia. Conclusion-Intestinal transplantation may be a life saving procedure in children with CIPO. Early referral and thorough pretransplant evaluation are keys to successful transplantation.
患有慢性肠假性梗阻（CIPO）的儿童通常需要全胃肠外营养（TPN），这使他们面临肝衰竭和反复发生导管相关感染的风险。对于依赖TPN且TPN治疗失败的肠衰竭儿童，肠移植已成为一种治疗选择。
研究转诊接受肠移植的CIPO儿童的治疗结果。
对27例转诊接受肠移植的CIPO患者的病历和诊断研究进行回顾性分析。
5名儿童未被列入移植名单：2名是因为家长的决定，2名是因为怀疑代理孟乔森综合征，1名是因为他能耐受肠内营养。6名儿童仍依赖TPN并等待移植。8名儿童在等待移植期间死亡。8名儿童接受了移植。3名儿童死亡（分别在移植后2个月、7个月和4年）。5名儿童存活，中位随访时间为2.6年（范围为2个月至6年）。所有接受移植的儿童都能够耐受完全肠内喂养。术后过程出现倾倒综合征、代理孟乔森综合征、麻醉剂戒断反应和贲门失弛缓症暴露等并发症。结论——肠移植对于患有CIPO的儿童可能是一种挽救生命的手术。早期转诊和全面的移植前评估是移植成功的关键。
