111: Surgical Techniques for non-Short Bowel Syndrome Children with Intestinal Failure: Reducing Length and Improving Outcome.

Introduction: Patients with pediatric intestinal pseudo-obstruction (PIPO) and congenital diarrheas and enteropathies (CODE), are unique intestinal failure patient populations. Bowel resection is not a routine and integral part of their treatment options. Herein, we describe extensive intestinal resection as a treatment modality in two distinct pathologies of IF - PIPO and microvillus inclusion disease (MVID). Case reports: 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. In an attempt to better control the patient’s fluid and electrolyte needs we hypothesized that a reduction in hypersecretory bowel mass with an extensive enterectomy would decrease the amount of electrolyte transport diarrhea. 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 electrolytes and volume needs were noted. Specifically, sodium, potassium, phosphate, and total fluid intake (TFI) requirements dropped from 37.7 mmol/kg, 6.9 mmol/kg, 2.4 mmol/kg, and 202.5 ml/kg/day respectively, to 10.5 mmol/kg (Na), 2.5 mmol/kg (K), 0.7 mmol/kg (P), and 123.3 ml/kg/day (TFI) 18 months after the surgery (Figure 1). 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 PIPO, TPN dependent, and with a terminal ileostomy performed at 3 years of age. Her surgical course was complicated by recurrent episodes of stoma prolapse. Numerous operations were performed including revision, resiting and pexing. In addition, the patient also presented with frequent episodes of mechanical bowel obstruction caused by the dilated, atonic, fluid-filled small bowel kinking at the level of the fascia of the ileostomy site. An enterectomy was performed leaving the patient with 135cm of small bowel to a jejunostomy. A tapering enteroplasty was also performed decreasing the caliber from 8cm to 3cm. Although the patient was primarily PN dependent she enjoyed eating for comfort; therefore, 135cm was chosen to permit some absorptive capacity. Two years after the procedure, the patient has had no episodes of stoma complications or bowel obstruction and has not required further surgical intervention. Conclusions: These two cases, illustrate less common forms of autologous bowel surgery to improve functional status. The surgical approach was focused on reducing complications related to the intestinal failure’s etiology. Consideration of extensive enterectomy in patients with no prospect of TPN weaning, can improve quality of life, reduce morbidity, and optimize PN and IF management.

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