Two-stage repair for delayed presentation of anorectal malformation with vestibular and perineal fistulae in females

Background/aim In spite of advances in knowledge and techniques in the management of anorectal malformation (ARM), delayed presentation of female ARM with vestibular and perineal fistulae is quite common especially in developing countries. In this literature, there is no standardized surgical algorithm for late presentation of these female ARM. This study aimed to present the experience of two pediatric surgery tertiary centers regarding delayed presentation of vestibular and perineal fistulae using two-stage procedures. Patients and methods This prospective study was conducted on 28 female patients with late presentation of vestibular and perineal fistulae from November 2014 to April 2019. Lower contrast study was done for all patients. Laxative, frequent rectal wash enemas, and Hegar's dilators were used for 2-3 weeks preoperatively. All patients were repaired by anterior sagittal anorectoplasty (ASARP) with covering high sigmoid loop colostomy, and colostomy closure was performed after 2-3 months. Krickenbeck continence score was used for assessment of anal continence. Results Of 28 patients included, 25 patients completed the study. There were 14 patients with perineal and 11 with vestibular fistulae. The age of patients ranged from 8 months to 18 years. The main reasons for delay were socioeconomic, unawareness, and overlapping causes. Sphincter-saving ASARP was used in 15 patients and the classic ASARP was used in 10 patients. Tapering proctoplasty was needed in one patient. Minor wound infection developed in three patients. Constipation occurred in 11 (44%) patients. Follow-up period ranged from 6 to 50 months (median 30 months). Good continence was obtained. Conclusion Although delayed presentation of vestibular and perineal fistulae in female patients is accompanied by comorbidities, with good surgical management, successful results can be obtained by using two-stage procedure.