Isolated liver & multivisceral tx TPN-related ESLD.

[Guest guest]

J Pediatr Surg.

2007 Jan;42(1):143-7.

Isolated liver and multivisceral

transplantation for total parenteral

nutrition-related end-stage liver disease.

JD, Rudolph JA, Kocoshis SA, Alonso MH, Ryckman FC, Tiao GM.

Division of Pediatric & Thoracic Surgery, Cincinnati Children's Hospital,

OH 45229

PURPOSE: Total parenteral nutrition (TPN) has

prolonged survival in children with intestinal failure; however, end-stage

liver disease owing to TPN-induced cholestasis

(ESLD-TPN) may preclude its use. ESLD-TPN is an

indication for isolated liver transplantation (ILT) or multivisceral

transplantation (MVT). Isolated liver transplantation

for ESLD-TPN should only be considered in patients who have the potential for enteral autonomy. METHODS: We

retrospectively reviewed the records of patients with ESLD-TPN who underwent

ILT (n = 7) or MVT (n = 5) between 1994 and 2005. The

median age at the time of transplantation was 10.0 months. Intestinal

failure followed necrotizing enterocolitis (n = 3), gastroschisis (n = 3), gastroschisis

with volvulus (n = 3), gastroschisis

with atresia (n = 1), malrotation

(n = 1), and megacystis microcolon

intestinal hypoperistalsis syndrome (n = 1). RESULTS: Isolated liver transplant patients had a median

length of small bowel of 70 cm and tolerated a median of 50% of enteral calories. The median

length of small bowel in patients who underwent MVT was 29 cm, and none

tolerated more than 30% of goal enteral feeds. Reduced-size (n = 5) and whole-liver (n = 2) allografts were used for patients undergoing ILT. Patients undergoing MVT received liver-small

bowel-pancreas (n = 4) or liver-small bowel-pancreas-colon (n = 1). Overall patient survival was 57% in ILT (median follow-up

= 25.1 months); 3 survivors are TPN independent, and the fourth patient

requires TPN 3 days/wk. Patient survival was 40% after MVT (median follow-up =

13.0 months); 1 MVT patient died of abuse 16.9 months after transplant and was

TPN independent at the time of death. Both survivors

are TPN independent. Bilirubin levels are within

normal range in all survivors. CONCLUSION: Isolated

liver transplantation for ESLD-TPN in the setting of intestinal failure is a

viable option in patients who have the potential for enteral

autonomy. Multivisceral

transplantation is the only alternative in patients without the potential for

intestinal recovery. Survival can be achieved in

patients with ESLD-TPN, but mortality remains high for both procedures. PMID: 17208555 [PubMed

- indexed for MEDLINE]

Barb in Texas - Together in the Fight, Whatever it Takes!

Son Ken (32) UC 91 - PSC 99 Listed 7/21 @ Baylor Dallas