Isolated Liver Transplant in Infants With Short Bowel Syndrome: Insights Into Outcomes and Prognostic Factors

• Published in: Journal of pediatric gastroenterology and nutrition Vol. 48; no. 3; pp. 334 - 340
• Main Authors: Dell‐Olio, D, Beath, SV, Goyet, J, Clarke, S, Davies, P, Lloyd, C, Protheroe, S, Millar, AJW, Kelly, DA, Gupte, GL
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins, Inc 01-03-2009; Lippincott Williams & Wilkins
• Subjects: Bacterial translocation; Biological and medical sciences; Body Size; Enteral Nutrition; Feeding. Feeding behavior; Female; Fundamental and applied biological sciences. Psychology; Gastroenterology. Liver. Pancreas. Abdomen; Gastrointestinal Motility; Growth; Humans; Infant; Intestinal adaptation; Intestinal Diseases - etiology; Intestinal Diseases - mortality; Intestinal Diseases - surgery; Intestinal failure associated liver disease; Kaplan-Meier Estimate; Liver Diseases - etiology; Liver Diseases - mortality; Liver Diseases - surgery; Liver transplantation; Liver Transplantation - mortality; Liver, biliary tract, pancreas, portal circulation, spleen; Male; Medical sciences; Modular feeding; Other diseases. Semiology; Parenteral nutrition; Parenteral Nutrition - statistics & numerical data; Prognosis; Retrospective Studies; Risk Factors; Short bowel syndrome; Short Bowel Syndrome - complications; Short Bowel Syndrome - mortality; Short Bowel Syndrome - surgery; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the digestive system; Treatment Outcome; Vertebrates: anatomy and physiology, studies on body, several organs or systems; Human; Translocation; intestinal failure; Prognosis; Metabolic diseases; Infant; Hepatic disease; Parenteral nutrition; Short bowel syndrome; Parenteral administration; Feeding; Isolated organ; Infection; Intestinal malabsorption; Gastroenterology; Bacteriosis; Bacterial translocation-Intestinal adaptation- Intestinal failure associated liver disease-Liver transplantation-Modular feeding-Parenteral nutrition- Short bowel syndrome; Digestive diseases; Intestinal disease; Adaptation; Liver transplantation
• ISSN: 0277-2116; 1536-4801
• DOI: 10.1097/MPG.0b013e31818c6099

ABSTRACT
Objective:
Selected infants with short bowel syndrome (SBS) and progressive intestinal failure associated liver disease (IFALD) may benefit from isolated liver transplantation (iLTx). The aim of the study is to identify risk factors for unfavourable outcome in iLTx.
Patients and Methods:
A retrospective review of medical records from 1998 to 2005 was undertaken. Risk factors were assessed by comparing long‐term survivors with those who died after iLTx.
Results:
Fifteen iLTx were performed in 14 infants with IFALD. All were parenteral nutrition (PN) dependent, but had tolerated enterally 54% (38–100) of energy intake before iLTx. Median residual bowel was 60 cm (30–200). Eight out of 14 had intact ileocaecal valve (ICV). Median bilirubin was 298 μmol/L (87–715) and all had portal hypertension. Eight out of 9 survivors were weaned from PN after median 15 months. In 4 out of 9 children, nontransplant surgery after iLTx facilitated intestinal adaptation. Growth velocity had improved at 3 years after iLTx (P = 0.001). Five children who died had poor enteral tolerance following iLTx (P < 0.002), which correlated with pretransplant dysmotility seen in 4 out of 5 children shown by contrast studies (P = 0.02) and increased frequency of line infections before (>6/year P < 0.04) and after (P < 0.001) iLTx.
Conclusions:
Isolated liver transplantation is a lifesaving option for selected children with SBS and IFALD. Revised criteria are proposed: progressive IFALD; 50 cm functional bowel in absence of ICV or 30 cm with ICV; 50% daily energy intake tolerated enterally for 4 weeks with satisfactory growth; and children with dysmotile bowel should be assessed for combined liver/bowel transplant unless the dysmotility is resolved and associated with minimal line infections.