Isolated liver transplantation in infants with short gut syndrome: Is less better?

✍ Scribed by George V. Mazariegos; Kyle A. Soltys; Geoffrey J. Bond; Robert H. Squires; Rakesh Sindhi

• Publisher: John Wiley and Sons
• Year: 2006
• Tongue: English
• Weight: 56 KB
• Volume: 12
• Category: Article
• ISSN: 1527-6465
• DOI: 10.1002/lt.20771

✦ Synopsis

Children awaiting intestinal transplantation for short gut syndrome (SGS) face daunting challenges. Especially for those with concomitant liver disease, the combination of long waiting time, scarcity of size-matched suitable donors, and systemic comorbidities of sepsis, gastrointestinal bleeding, and coagulopathy result in a national median wait list time of greater than 9 months and an annual national mortality of 35% (http:// www.ustransplant.org). Clearly, SBS patients with endstage liver disease account for the majority of this risk, comprising nearly 90% of the reported deaths on the waiting list since 1994. 1 Due to the known detrimental effects of liver failure on gut adaptation 2 and because a minority of these patients might be considered candidates for weaning from parenteral nutrition in the absence of end-stage liver disease, attempts have been made to apply isolated liver transplantation (LT) in selected patients with SGS. Sporadic single case reports 3-5 and small series 6-8 have reported varying degrees of success with isolated LT in this patient population. A review of the literature (Table 1) reveals that, 24 SGS patients had previously undergone isolated LT, with 18 of 24 (75%) surviving and 15 of 24 (62.5%) achieving freedom from parenteral nutrition at follow-up periods that range from 9 to 27 months in the case series and 3.5 to 8 yr in the single case reports.

In this issue of Liver Transplantation, Botha and colleagues build on their initial experience with isolated LT for SGS 8 and present their retrospective experience with LT in 23 patients with SGS and end-stage liver disease. The series reports patient and graft survival of 82% and 75% at 1 yr and 72% and 60% at 5 yr, respectively. Of the survivors, 14 (82%) achieved enteral autonomy. As this issue is analyzed critically, several cautionary