Liver and intestinal transplantation in a child with cystic fibrosis: A case report Cystic fibrosis (CF) is an inherited disorder that affects epithelial chloride transport and presents as a multisystem disease. Meconium ileus is the presenting symptom in 20% of patients with CF (1), and is characterized by extremely viscid, protein-rich inspissated meconium causing ter- minal ileal obstruction (2). Approximately half of these patients present with complicated meconi- um ileus, including volvulus, atresia, gangrene, perforation and meconium peritonitis, manda- ting early surgical intervention (3, 4). With current approaches, morbidity and mortality have decreased remarkably (5, 6), with long-term prognosis dependent mostly on the patient’s pulmonary status (7, 8). Due to the improvements in pulmonary ther- apy, the predicted median survival in patients with CF is now 32 yr (1). Despite initial concerns regarding exacerbation of underlying pulmonary disease and the potential effect of immunosup- pression on respiratory infections, liver trans- plantation in recipients with CF-related hepatic failure has been accomplished successfully (9–16). Because of the multisystem nature of this disease, some patients with irreversible pulmon- ary and hepatic failure have received multiorgan allografts, with dismal results (16–19). In this report we review the first combined liver and small bowel multiorgan transplantation in a recipient with CF and short bowel syndrome secondary to complicated meconium ileus. Case report This boy initially presented to our center at the age of 5 months with short bowel syndrome resulting from extensive small bowel resection for meconium ileus complicated by midgut volvulus. The diagnosis of CF was made by a positive sweat chloride test, and confirmed by DNA analysis. He had a small bowel remnant of Fridell JA, Mazariegos GV, Orenstein D, Sindhi R, Reyes J. Liver and intestinal transplantation in a child with cystic fibrosis: A case report. PediatrTransplantation2003:7:240–242. Ó 2003BlackwellMunksgaard Abstract: Cystic fibrosis (CF) is an inherited disorder that presents as a multisystem disease with meconium ileus being the presenting symptom in 20% of patients. Approximately half of these patients present with complicated meconium ileus mandating early surgical intervention, potentially resulting in short gut syndrome. Although liver transplan- tation in children with CF has been described, this is the first report of a combined liver and small bowel transplant in a recipient with CF. A 7-month-old boy with CF presented with short bowel syndrome following extensive small bowel resection for meconium ileus and pro- gressive cholestatic liver failure from intravenous hyperalimentation. He underwent combined liver and small intestinal transplant. He was dis- charged home three weeks post-transplant on enteral feeds with sup- plemental intravenous fluid. He has had routine protocol small bowel allograft biopsies with no documented rejection episodes. He has been treated for minor respiratory infections without major sequelae. Improvements in pulmonary therapy have impacted on the survival in the CF population to the point where the need for multiorgan trans- plantation will be increased in the future. Extrapolating from the excellent experience of liver transplantation in children with CF, early liver and small intestinal multivisceral transplantation, if indicated, can be performed safely in children with CF. Jonathan A. Fridell 3 , George V. Mazariegos 1 , David Orenstein 2 , Rakesh Sindhi 1 and Jorge Reyes 1 1 Children's Hospital of Pittsburgh, Departments of Pediatric Transplantation, Thomas E. Starzl Transplantation Institute, and 2 Department of Pediatrics, Antonio J. and Janet Palumbo Cystic Fibrosis Center, University of Pittsburgh Medical Center, Pittsburgh, PA, 3 Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, USA Key words: pediatric – liver transplantation – intestinal transplantation – cystic fibrosis Jorge Reyes, MD, Thomas E. Starzl Transplantation Institute, Children's Hospital of Pittsburgh, Room 7 North, 7950, 3705 Fifth Avenue, Pittsburgh, PA 15213, USA Tel.: 412-692-7867 Fax: 412-692-6116 E-mail: jorge.reyes@chp.edu Accepted for publication 16 January 2003 Abbreviations: CF, cystic fibrosis; DNA, deoxyribonucleic acid; DNAse, deoxyribonuclease; TPN, total parenteral nutrition. Pediatr Transplantation 2003: 7: 240–242 Printed in UK. All rights reserved Copyright Ó 2003 Blackwell Munksgaard Pediatric Transplantation ISSN 1397-3142 240