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Autologous Intestinal Reconstructive Surgery in the Management of Total Intestinal Aganglionosis

Fusaro, Fabio*; Morini, Francesco*; Mutanen, Annika; De Angelis, Paola*; Tambucci, Roberto; Capriati, Teresa*; Hermans, Dominique; Candusso, Manila*; Diamanti, Antonella*; Bagolan, Pietro*; Pakarinen, Mikko

Journal of Pediatric Gastroenterology and Nutrition: May 2019 - Volume 68 - Issue 5 - p 635–641
doi: 10.1097/MPG.0000000000002260
Original Articles: Gastroenterology
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Objectives: Total/near total intestinal aganglionosis (TIA/NTIA) is the most uncommon and life-threatening form of Hirschsprung disease (HD). The management of TIA/NTIA is challenging and the role of autologous intestinal reconstructive (AIR) surgery is controversial. The objective is to evaluate the effectiveness of AIR in patients with TIA/NTIA.

Methods: Records from children affected by TIA and enrolled in the multicenter international Pediatric Intestinal Rehabilitation and Transplantation Registry were retrospectively reviewed.

Results: Fourteen patients with TIA were identified. TIA diagnosis was confirmed histologically at the median age of 14 days of life. All received a proximal decompressive jejunostomy. Two patients died, 4 patients had satisfactory stoma output with enteral tolerance without additional procedures, 8 underwent 10 AIR procedures (4 Ziegler myotomy-myectomy, 3 transposition of aganglionic ileum with or without myotomy, 2 simple tapering, 1 longitudinal lengthening and tailoring procedure with associated myotomy). AIR significantly reduced median stoma output, from 197 to 31 mL · kg−1 · day−1 (P = 0.0001). The reduction was seen in all patients. In addition, AIR improved enteral tolerance in the long term in 5 of 8 patients (63%), and temporarily in 1, leading to a reduction of parenteral nutrition requirement from 100% to 70% (P = 0.0231).

Conclusions: AIR surgery in carefully selected patients may be useful and effective way to enhance residual bowel absorptive function and to reduce parenteral nutrition requirements. AIR and intestinal transplantation are complementary surgical tools in the complex treatment algorithm of TIA/NTIA.

*Department of Medical and Surgical Neonatology, Newborn Surgery Unit, Digestive and Endoscopic Surgery, Gastroenterology and Nutrition, Intestinal Failure Rehabilitation Research Group, Bambino Gesù Children's Hospital Research Institute, Rome, Italy

Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

Department of Pediatrics and Pediatric Surgery Unit, Saint Luc Hospital-Université Catholique De Louvain, Brussels, Belgium.

Address correspondence and reprint requests to Fabio Fusaro, MD, Department of Medical and Surgical Neonatology, Newborn Surgery Unit, Bambino Gesù Children's Hospital Research Institute, Piazza S. Onofrio 4, 00165 Rome, Italy (e-mail: fabio.fusaro@opbg.net).

Received 28 October, 2018

Accepted 25 December, 2018

The authors report no conflicts of interest.

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,