Purpose: Low-type anorectal malformation (ARM) in female patients is considered to be associated with a low risk of postoperative complications and favorable bowel dysfunction; however, it can still lead to various issues requiring long-term follow-up and management. This study aimed to investigate the changes over time in the postoperative defecation function of female patients with low-type ARM.
Method: Patient data were collected between 1984 and 2021. Eighty-eight female patients with low-type ARM were enrolled. One patient with an undetermined ARM type was excluded. The patient characteristics, associated anomalies, and long-term defecation function were retrospectively reviewed and analyzed.
Results: Of the 87 patients with confirmed ARM, 46 (52.9%) had anovestibular fistula (AVF), 9 (10.3%) had anovulvar fistula, 19 (21.8%) had anocutaneous fistula, 10 (11.5%) had covered anal stenosis, and 3 (3.4%) had complete anal coverage. The total evacuation score, except for AVF, reached "excellent" at around six years of age, while that in AVF reached "excellent" at nine years of age. The mean treatment period with glycerin enema after anorectoplasty was 4.1±3.9 years for AVF, which was the longest among the 5 types of ARM. The follow-up period for covered anal complete was the longest among the five types at 94.5±98.3 months. Notable associated anomalies related to female ARM included Mayer-Rokitansky-Küster-Häuser Syndrome (MRKH) in AVF (2.2%) and perineal groove in anocutaneous fistula (10.5%). All cases of completely covered anus had trisomy 21.
Conclusion: The postoperative defecation function in female patients with low-type ARM improved over time. However, patients with AVF tended to reach "excellent" defecation function more slowly in comparison to other low-type ARMs, leading to the longest requirement for glycerin enema usage and follow-up period among all types of ARM. Long-term defecation management that considers the characteristics of functional improvement is important, particularly in patients with AVF.
Keywords: low-type anorectal malformation, defecation function, female, associated anomaly.
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