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321 16 Outcomes of surgical management Defecation occurred after bisacodyl infusion in all five children. The remaining child had no HAPCs and did not defecate during the test (patient 25, Supplemental file 2). After colonic manometry, five patients without a history of abdominal surgery received an ileostomy, with two patients subsequently undergoing a subtotal colectomy with ileo-rectal anastomosis (patient 33, 35, Supplemental file 2). In one patient, colonic motility testing was performed to evaluate the possibility of ileostomy closure, but results showed abnormal colonic motility, which resulted in a subtotal colectomy (patient 19, Supplemental file 2). Surgical interventions The initial surgical interventions are presented in Table 1 and Supplemental file 2. Twenty patients underwent a second surgical procedure and seven patients required ≥3 surgical procedures. At the end of the study period, 11 patients (30%) had an ileostomy and seven (19%) a colostomy. Stoma closure with anastomosis was performed in 19 patients (51%). Two senior and experienced pediatric surgeons in colorectal surgery were involved in all surgical procedures. TABLE 2. Preoperative testing Study n (%) Abnormal/positive results, n (%) Findings Colonic transit time (CTT) 32 (87) 25 (78) 25, delayed CTT (>62 hours) 7, normal CTT (<62 hours) a Anorectal manometry 18 (49) 0 (0) 18, RAIR present and normal resting pressure Rectal biopsy 19 (51) 0 (0) 19, ganglion cells present Barium enema 19 (51) 13 (68) 10, dolichocolon 8, megarectum or –sigmoid Defecography 12 (32) 7 (58) Functional findings 5, normal 3, failed to defecate 1, incomplete relaxation of the puborectalis muscle Anatomic findings 3, rectocele MRI lumbar spine 10 (27) 0 (0) 10, normal Colonic manometry 6 (19) 6 (86) 6, abnormal colonic motility b a Two patients with normal CTT used laxatives during the measurement. b High-resolution colonic manometry recordings are analyzed for the presence of different colonic motor patterns, including HAPCs (as described previously 21 ).

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