INTESTINAL TRANSPLANTATION FOR SHORT GUT SYNDROME SECONDARY TO GASTROSCHISIS

Abstract

P605 Aims: Gastraschisis is the most frequent cause of pediatric intestinal transplant. The outcome for infants with gastroschisis has improved with current advantages in prenatal diagnosis, neonatal care and surgical techniques. However, a subset of these patients requires extensive bowel resections and in addition small portion of them develops long-term dependence on total parental nutrition (TPN) as a status of short-bowel syndrome. This study reviews our experience with intestinal transplantation for children who had sustained small intestinal loss as a result of gastroschisis in order to elucidate those factors affecting the eventual outcome of children with short-bowel syndrome secondary to gastroschisis. Methods: A retrospective chart review was performed for children who underwent small bowel transplantation for gastroschisis at the University of Miami between August 1994 and June 2003. Results: Thirty-two transplants were performed in twenty-eight children with gastroschisis during the study period. There were 14 boys and 14 girls with a median age of 23 months (range, 6 months to 8.5 years) at transplant. 14 patients (50%) were less than one year old at the time of transplant. Most patients were born prematurely and the average estimated gestation age was 34.5 weeks (range; 30 to 38 weeks). Mean birth weight was 2192 g. (range; 1235 g to 3012 g). Gastroschisis was prenatally diagnosed in 14 cases. Associated mid-gut anomalies were present in 22 infants (Bowel atresia was seen in 14 cases, Volvulus 3 cases, and/or Bowel ischemia 16 cases). Spontaneous prenatal closure of gastroschisis, which was considered to be a rare anomaly usually associated with bowel atresia and ischemia due to very small abdominal defect, was seen in 9 patients. In 3 patients, necrotizing enterocolitis (NEC) was developed after gastroschisis repair. Nineteen infants needed bowel resection within a week after birth. Most of the patients had complicated course and required multiple abdominal surgery before transplant. Transplantation types included isolated small intestine (n=8), liver and small intestine (n=8) and multivisceral transplants (n=16). Eighteen patients (64.3 %) currently are alive at a median follow-up of 15.7 months (range, 0.5 to 72 months). Short-term survival rate has significantly improved in recent years. Patient survival at 6 months, one year, and 2 years was: 50.0 %, 40.0 %, and 40.0% in the period between 1994 and 2000; 94.4 %, 77.9 %, and 77.9 % in 2001 through present. Conclusions: The outcome for most of the children with simple gastroschisis is quite good without being short bowel syndrome or intestinal failure. However, patients with complex gastroschisis, especially those with associated intestinal anomalies, likely to need extra surgeries in their early days of life and have a significant risk to become short bowel syndrome. Intestinal transplantation can be a life-saving option and provides a satisfactory outcome for children with gastroschisis associated short bowel syndrome who already developed serious TPN-related complications.