Massive bowel resection for children with non-short bowel syndrome intestinal failure

Pediatric patients with chronic intestinal pseudo-obstruction (CIPO) and congenital diarrheas and enteropathies (CODE) are unique intestinal failure (IF) patient populations. Intestinal transplantation is often considered due to complexity of medical management. Herein, we describe extensive intestinal resection as a treatment modality for symptom control in these two distinct categories of IF - CIPO and microvillus inclusion disease (MVID).

Patient 1: A 2-year-old female with MVID (MYO5B mutation) with difficult to manage fluid and electrolyte requirements due to high volume diarrhea refractory to medical management including antisecretory medications. A resection of 178 cm of jejunoileum, ileocecal valve, and a subtotal colectomy was performed. The remaining 40 cm of jejunum was anastomosed to the sigmoid colon. Following this intervention, a significant reduction in parenteral nutrition (PN) electrolytes and volume needs was noted. In addition, improved quality of life and easier daily management at home were reported by the parents.Patient 2: A 15-year-old female diagnosed with CIPO, PN dependent, and with a terminal ileostomy performed at 3 years of age. Her surgical course was complicated by recurrent episodes of stoma prolapse and frequent episodes of mechanical bowel obstruction at the level of the stoma. A partial enterectomy with tapering was performed leaving the patient with 135 cm of small bowel to a jejunostomy. Four years after the procedure, the patient has had no recurrent episodes of stoma complications or bowel obstruction and has not required further surgical intervention.

Consideration of extensive enterectomy as a non-transplant surgical option for symptom control in patients with intestinal pseudoobstruction or mucosal enteropathies where PN weaning is not possible, can improve quality of life, reduce morbidity, and ease PN management.