Outcomes Of Children With Intestinal Failure Secondary To Ultrashort Bowel Syndrome

Introduction: In the current era of management for paediatric intestinal failure, mortality has decreased significantly. Improved survival rates in patients with short bowel syndrome have allowed patients increased time to achieve enteral autonomy. Ultrashort bowel syndrome (USBS) has been defined as <25% of predicted small bowel length for age. The specific incidence and mortality of paediatric patients with USBS is not known. Traditionally, children with USBS have higher mortality and less chance of achieving enteral autonomy. Our objective was to determine characteristics and outcomes of paediatric patients with USBS in the modern era of intestinal rehabilitation. Methods: A retrospective cohort study of patients managed by a multidisciplinary intestinal rehabilitation program with a diagnosis of USBS (residual bowel length of <25% of that predicted for age) between January 2006 and December 2017 with a minimum one-year follow up. Student’s t test, and Chi Square were used where appropriate. Results: There were 61 patients with USBS with 42 males (69%) and a mean gestational age of 34 (±4.3) weeks. The most common aetiologies included abdominal wall defects (37.7%) and necrotizing enterocolitis (21.3%). Mean follow-up of the cohort was 1644 days (¨± 1318.4). 37 patients (60.7%) required a stoma, with 18 of these being colostomies. Twenty-two (36%) patients achieved enteral autonomy with a mean time of 591.9 (± 524.5) days. These patients had significantly greater percentage small bowel length, 18.9% vs.12.45% (p=0.001). 73% of the cohort experienced at least one septic event. The mean rate of central line associated bloodstream infections was 1.4/1000 (±5.74) catheter days. 27 (44.3%) patients reached a conjugated bilirubin (CB) level of 50umol/L after 80 (±67) days, 12 (19.7%) patients reached a CB of 100umol/L at 136 (±65.86) days, and 4 (6.5%) patients received liver transplantation, 3 of these included small bowel transplantation The case fatality rate was 9.8%. Conclusions: This study presents the long-term outcomes of paediatric patients with USBS. Despite the medical complexity of these patients, in the current era of management, overall survival was 90%. Furthermore, one third of patients achieved enteral autonomy with a low rate of sepsis and transplantation.