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 - for the ARM-Net Consortium
AU - Iacobelli, Barbara D.
AU - Midrio, Paola
AU - Sloots, Cornelius E.J.
AU - Samuk, Inbal
AU - van Rooij, Iris A.L.M.
AU - Lisi, Gabriele
AU - de Blaauw, Ivo
AU - Fascetti-Leon, Francesco
AU - Vázquez, Araceli García
AU - Krois, Wilfried
AU - Lacher, Martin
AU - Leva, Ernesto
AU - Schmiedeke, Eberhard
AU - Schukfeh, Nagoud
AU - Stanton, Michael
N1 - Publisher Copyright: © 2023, The Author(s).
PY - 2023/7/28
Y1 - 2023/7/28
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.
UR - http://www.scopus.com/inward/record.url?scp=85166011158&partnerID=8YFLogxK
U2 - 10.1007/s00383-023-05518-7
DO - 10.1007/s00383-023-05518-7
M3 - Article
C2 - 37507508
AN - SCOPUS:85166011158
SN - 0179-0358
VL - 39
JO - Pediatric Surgery International
JF - Pediatric Surgery International
IS - 1
M1 - 242
ER -