title: Isolated liver transplantation for liver failure in patients with short bowel syndrome.
abstract: The ultimate prognosis for patients with short bowel syndrome (SBS) has become progressively more favorable over the past decade. Advances in long-term total parenteral nutrition (TPN) have allowed this group of patients to meet nutritional needs while the process of intestinal adaptation occurs. Unfortunately, a subgroup of patients with SBS have hepatic failure (HF), most often secondary to TPN-induced cholestasis. Combined small bowel and liver transplantation (LT) offers a sound anatomic solution for cases of HF with SGS, but it remains experimental at this time. We propose that an isolated LT is a viable alternative mode of therapy for the patient with HF and SBS. The following characteristics were reviewed for five patients with SBS and HF who underwent LT: age at transplantation, weight, liver function, survival, intestinal length, volume of feeding before surgery, and current feeding tolerance and liver function. Four boys and one girl, aged 5.5 to 15 months (average, 11.9), had LT. The total bilirubin level at the time of transplantation was 14.4 to 37 mg/dL (average, 24.7). The patients weighed between 3.8 and 12 kg (average, 8.0), and feeding tolerance ranged from no enteric to complete enteric feeding (average, < 33% of calories by enteric feeding). Bowel loss was attributed to necrotizing enterocolitis in two cases, volvulus in two, and birth hypoxia in one. Bowel length ranged from 60 to 120 cm (average, 88.6). Four children (80%) survived LT, and the average follow-up period was 9.3 months. Three (75%) are home; one is on combined hyperalimentation and enteral feeding, and two are on full enteric feeding. One remains in a chronic care facility, on combined enteral and intravenous feeding. The average daily enteral feeding now comprises more than 70% of caloric requirements. The total bilirubin level is .6 to .8 mg/dL (average, .71). Isolated LT for HF in the patient with SBS effectively restores liver function, allowing time for further intestinal adaptation.
authors.affiliation: Department of Surgery, St Christopher's Hospital for Children, Philadelphia, PA 19134-1095.
authors.forename: J P
authors.initials: JP
authors.lastname: Lawrence
authors.forename: S P
authors.initials: SP
authors.lastname: Dunn
authors.forename: D F
authors.initials: DF
authors.lastname: Billmire
authors.forename: K
authors.initials: K
authors.lastname: Falkenstein
authors.forename: C D
authors.initials: CD
authors.lastname: Vinocur
authors.forename: W H
authors.initials: WH
authors.lastname: Weintraub
mesh_terms: D005260:Female; D006801:Humans; D007223:Infant; D017093:Liver Failure / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D011182:Postoperative Care; D012778:Short Bowel Syndrome / Q000150:complications*