TY - JOUR
T1 - Rectal atresia and rectal stenosis
T2 - the ARM-Net Consortium experience
AU - de Beaufort, Cunera M. C.
AU - Gorter, Ramon R.
AU - Iacobelli, Barbara D.
AU - Midrio, Paola
AU - Sloots, Cornelius E. J.
AU - Samuk, Inbal
AU - van Rooij, Iris A. L. M.
AU - ARM-Net consortium
AU - Lisi, Gabriele
N1 - Funding Information: The members of the working group of the ARM-Net Consortium would like to thank Martina Pennese, a medical student at the Department of Pediatric Surgery, Spirito Santo Hospital, Pescara, G. d’Annunzio University, Chieti-Pescara, Italy, for her contribution to the collection of additional follow-up data for this project. Furthermore, we would like to thank all participating pediatric surgeons for their contribution by entering their patients into the ARM-Net registry. Members of the ARM-Net Consortium not mentioned as co-authors by person: Ivo de Blaauw, Department of Surgery-Pediatric Surgery, Radboudumc Amalia Children’s Hospital, Nijmegen, the Netherlands; Francesco Fascetti-Leon, University of Padua, Italy; Araceli García Vázquez, Hospital 12 de Octubre Madrid, Spain; Wilfried Krois, Medical University of Vienna. Vienna, Austria; Martin Lacher, Department of Pediatric Surgery, University of Leipzig, Germany; Ernesto Leva, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milano, Italy; Eberhard Schmiedeke, Klinikum Bremen Mitte, Bremen, Germany; Nagoud Schukfeh, Medical School Hannover, Hannover, Germany; and Michael Stanton, University Hospital Southampton, Southampton, United Kingdom Publisher Copyright: © 2023, The Author(s).
PY - 2023/12
Y1 - 2023/12
N2 - PURPOSE: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry. METHODS: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed. RESULTS: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3-9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema. CONCLUSION: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging.
AB - PURPOSE: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry. METHODS: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed. RESULTS: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3-9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema. CONCLUSION: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging.
KW - ARM-Net Consortium
KW - Anorectal malformations
KW - Constipation
KW - Rectal atresia
KW - Rectal stenosis
UR - http://www.scopus.com/inward/record.url?scp=85166011158&partnerID=8YFLogxK
U2 - https://doi.org/10.1007/s00383-023-05518-7
DO - https://doi.org/10.1007/s00383-023-05518-7
M3 - Article
C2 - 37507508
SN - 0179-0358
VL - 39
SP - 242
JO - Pediatric surgery international
JF - Pediatric surgery international
IS - 1
M1 - 242
ER -