A Two-year-old female child from South Sudan presented to us with ARM and a huge perineal mass covering the whole perineum with unusual female external genitalia. The child had previous simple loop sigmoid colostomy which was done in Sudan at day two after birth. She had an average weight percentile for age, fully active with no special features. Formal examination of the perineum was performed under general anesthesia revealed a large perineal Lipoma extending from tip of coccyx posteriorly to a mildly separated pubic bone anteriorly. There was duplication of the introitus with two openings, each flanked by the single laterally located labia majora which are separated by the perineal mass. The clitoris was duplicated with one is seen on either side. The two orifices on either side were communicating with each other as a catheter introduced from one orifice exited through the other. The vaginal and urethral orifices could not be identified from the outside; however, there was intermittent discharge of urine from both introitus openings. The anal opening was absent. The muscle complex was examined by muscle stimulator and the point of maximal contraction was more on right side of midline. Abdominal US showed absence of associated renal abnormalities. Genitography and distal loopogram examinations were performed in the same setting. A catheter was introduced through the right introitus, with opacification of uterine cavity and vagina, faint opacification of the rectum is noted, suggesting recto-vestibular fistula which was confirmed in distal loopogram examination. Pelvic MRI examination showed a large fat signal intensity lesion at the perineal region with separation of the symphysis pubis. There were two vaginal introitus, with the left one reached to the uterus, the two vaginal introitus were communicating with each other through a transverse tract. Examined portion of the spine revealed dysplastic sacrum with paramidline orientation of the visualized malformed sacral segments, together with tethered cord & small intraspinal sacral lipoma (caudal regression syndrome). Surgery for reconstruction of perineal body, external genitalia, excision of the perineal mass performed in the same sitting with anorectoplasty. Histopathological examination of the mass revealed mature fat cells. Inconclusion ARMs may be associated with complex anomalies, proper preoperative assessment and imaging are essential before any attempt of surgical intervention.
SPR 2022 Annual Meeting & Postgraduate Course