Short Bowel Syndrome

Abstract

Objective: Short Bowel Syndrome (SBS) is the leading cause of Intestinal Failure (IF) in children requiring Home Parenteral Nutrition (HPN). This 16-year study evaluates the complications and prognosis factors of SBS patients managed by a single expert center. Study design: SBS pediatric patients referred to HPN between January 1st 2000 and December 31st 2015 were classified into 3 groups: type 1: end jejunostomy, type 2: jejuno-colic anastomosis and type 3: jejuno-ileal anastomosis. The level of PN dependency [by using the index Non Protein Energy Intake (NPEI)/Resting Energy Expenditure (REE) by Schofield formula], outcomes, complications, growth, pubertal onset were analyzed. Results: For the 156 children, the underlying diseases were necrotizing enterocolitis (NEC) (22.40%), midgut volvulus (21.80%), gastroschisis (20.50%), extensive Hirschsprung’s disease (EHD) (16.00%), intestinal atresia (15.40%), or other events (3.90%). Weaning off was achieved in 79 patients (50.70%), after 2.20 ± 1.70 years of PN. Intestinal Transplantation (ITx) was performed in 13 patients (8.30%) after 4.20 ± 1.30 years of PN. At the end of the study period, 63 patients (40.40%) were still on HPN for 5.3 ± 3.7 years. Only one patient with cystic fibrosis and liver cirrhosis deceased. By Cox regression analysis, the following anatomical characteristics are independent prognosis factors of PN duration: length of remnant bowel, presence of an ostomy, ileo-caecal valve (ICV) resection, colon resection. Citrulline plasma levels correlate with the ratio NPEI/REE. Overall rate of catheter related sepsis was 1.2 CRS/1000 days of PN. Bilirubin > 30 μmole/l was observed in 16% of patients still dependent on PN after 5.30 ± 3.70 years. No statistical differences on growth were found between the groups, proving that sufficient nutritional intake was provided to achieve optimal growth. The puberty occurred in 8 girls at 12.1 ± 1.0 years and 8 boys at 13.8 ± 0.7 years. Conclusion: SBS has a very good outcome despite long term PN. HPN is a safe and efficient therapeutic option for long term management of SBS pediatric patients. Such data might be very helpful in addressing criteria for GLP-2 (teduglutide).