Skipped aganglionic lengthening transposition (SALT) for short bowel syndrome in patients with total intestinal aganglio
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TECHNICAL INNOVATION
Skipped aganglionic lengthening transposition (SALT) for short bowel syndrome in patients with total intestinal aganglionosis: technical report and feasibility A. Pini Prato1,2 · M. G. Faticato1 · I. Falconi1 · E. Felici1 · G. Casaccia1 · M. Caraccia1 · P. Nozza1 Accepted: 8 October 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
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. Keywods Total intestinal aganglionosis · Short bowel syndrome · Transposition · Lengthening · Malabsorption
Introduction Total intestinal aganglionosis (TIA) can lead to short bowel syndrome (SBS) depending on the proximal jejunal involvement of aganglionosis [1]. TIA leads to a particularly demanding form of SBS as ileocecal valve or colon are not preserved neither functional (both aganglionic by definition). On the ground of these considerations, newborn with TIA and less than 40 cm of ganglionated jejunum at Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00383-020-04763-4) contains supplementary material, which is available to authorized users. * A. Pini Prato [email protected] 1
Umberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, EU, Italy
Unit of Minimally Invasive and Robotic Surgery, Department of Surgery, Ospedale Pediatrico Bambino Gesù, IRCCS, Piazza di Sant’Onofrio, 4, 00165 Rome, Italy
2
birth are considered at high risk of intestinal failure (IF) and subsequent parenteral nutrition (PN) dependence with high stoma output making PN weaning unlikely [2]. A peculiarity of patients with TIA is the presence of defunctionalized but dysmotile (aganglionic) gut distal to the norm
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