Ventura K, Rekhtman Y, Jan D, Emond J, Lobritto S
Columbia University Medical Center, New-York Presbyterian Hospital, 622 W. 168th Street, New York, NY 10032, USA.
Transplant Proc. 2006 Jul-Aug;38(6):1699-701. doi: 10.1016/j.transproceed.2006.05.040.
Cholestasis progressing to end-stage liver disease (ESLD) is common in intestinal failure (IF) in infants. Isolated liver transplantation (OLT) is performed when eventual enteral sufficiency is expected. We reviewed our experience with OLT for ESLD in patients with residual IF. From 1998 to 2004, four IF patients underwent OLT for ESLD at our institution. Three were performed as UNOS status I for acute decompensation of chronic liver failure; one other with severe cholestasis with a living donor. The living donor recipient died within months after OLT of chronic respiratory failure despite normal liver function. One recipient remains on parenteral nutrition (PN) and continues to receive partial enteral feeds with normal liver function. Two other recipients became enterally sufficient after the OLT. Biliary complications occurred in two patients, one with late hepatic artery thrombosis. Resolution was achieved with serial balloon dilatations and the other by conversion from duct-to-duct anastomosis to a choledochoduodenostomy. The morbidity of OLT for this indication is higher than for others likely because of comorbidities like sepsis and pulmonary insufficiency. OLT is complicated further by the attempt to maximally preserve residual bowel length for the biliary reconstruction. OLT can be an emergent life-saving procedure in IF patients despite higher morbidity. Improved liver function and diminished portal pressures may shorten the time to enteral sufficiency. Management of parenteral nutrition after OLT can avoid ESLD and eliminate small bowel transplantation in selected patients.
胆汁淤积进展为终末期肝病（ESLD）在婴儿肠衰竭（IF）中很常见。当预期最终能实现肠内营养充足时，会进行单纯肝移植（OLT）。我们回顾了我们对残余IF患者进行OLT治疗ESLD的经验。1998年至2004年，我们机构有4例IF患者因ESLD接受了OLT。其中3例作为UNOS I级状态进行手术，用于慢性肝衰竭的急性失代偿；另一例严重胆汁淤积患者接受了活体供肝移植。尽管肝功能正常，但该活体供肝受者在OLT后数月内因慢性呼吸衰竭死亡。一名受者仍依赖肠外营养（PN），肝功能正常并继续接受部分肠内喂养。另外两名受者在OLT后实现了肠内营养充足。两名患者出现了胆道并发症，其中一名发生了晚期肝动脉血栓形成。通过连续球囊扩张解决了该问题，另一名患者则通过将胆管对胆管吻合改为胆总管十二指肠吻合术解决。由于脓毒症和肺功能不全等合并症，该适应证的OLT发病率高于其他情况。为了最大程度保留用于胆道重建的残余肠段长度，OLT的复杂性进一步增加。尽管发病率较高，但OLT对IF患者可能是一种紧急的挽救生命的手术。肝功能改善和门静脉压力降低可能会缩短实现肠内营养充足的时间。OLT后肠外营养的管理可以避免ESLD，并在部分患者中消除小肠移植。
