A Surgical Perspective of the Outcome of a Multidisciplinary Intestinal Rehabilitation Program for Children With Short Bowel Syndrome in the Netherlands

Aim. In 2001, a multidisciplinary intestinal rehabilitation program, prompted by a nationwide collaboration on intestinal failure (Dutch Registry for Intestinal Failure and Intestinal Transplantation), was started for children who have short bowel syndrome (SBS). This study evaluates this program, focusing on children who have SBS after extensive bowel resection. Design. This is a retrospective cohort study. Method. Demographic data, general information on disease status, and outcome of intestinal rehabilitation of patients treated between 2001 and 2009 were collected. Outcome measures were intestinal autonomy, intestinal and/or liver transplantation, and survival. Results. Ten boys and 9 girls, median gestational age 36 weeks, were treated. Eight were referred, 3 times as many as in the period 1991-2000. Causes of SBS were intestinal atresia (3), gastroschisis (2), volvulus (9), necrotising enterocolitis (3), and strangulation (2). The median remaining small-intestinal length was 35 cm (range, 10 to 70 cm). In 14 patients the ileocecal valve was still present. In all patients at least 25% of colon was still present. The median follow-up was 25 months (range, 50 days to 9 years). After a median of 138 days (range, 41 days to 11 years) on total parenteral nutrition, 16 patients (84%) reached intestinal autonomy. Central venous catheter related complications occurred in all; there were liver function disorders in 68%, and a failure to thrive in 26%. One patient underwent intestinal lengthening. No patient needed intestinal transplantation, but one underwent liver transplantation for intestinal failure associated liver disease. Overall mortality was 11%: those 2 patients died of abdominal sepsis. Conclusion. This specialized intestinal rehabilitation program led to intestinal autonomy in 84% of the patients who had SBS. None of the patients underwent an intestinal transplantation