Skipped aganglionic lengthening transposition (SALT) for short bowel syndrome in patients with total intestinal aganglionosis: technical report and feasibility.

ABSTRACT

Extended aganglionosis (TIA) is the presence of some viable aganglionic gut distal to the levelling jejunostomy. Different surgical procedures (including transplantation) have been proposed with inconclusive results. We conceived a new procedure named skipped aganglionic lengthening transposition (SALT) consisting of multiple pedicled isoperistaltic transpositions of aganglionic ileal loops interposed to normoganglionic jejunum. The innovative aspect consists of taking advantage of the propulsive effect of normoganglionated bowel to progress enteric content throughout interposed aganglionic loops down to the stoma. The procedure was adopted in a male patient who was born with 30 cm of normoganglionated jejunum. SALT was performed when the baby was 18 months. Three 5-cm pedicled isoperistaltic aganglionic loops of small bowel were interposed each 10 cm of normoganglionic jejunum with an overall 36% length gain (from 42 to 57 cm). Postoperative course was uneventful. 6 months postoperatively, an upper gastrointestinal series showed normal progression without dilatations. A laparoscopic gastrostomy was performed due to food aversion 6 months postoperatively, demonstrating impressive anatomic and functional postoperative results. The procedure provides promising and unique opportunity for patients with TIA with encouraging outlook for the near future.