RECTOVAGINOPLASTY FOR VAGINAL ATRESIA WITH ANORECTAL MALFORMATION

Abstract

Rectovestibular fistula with vaginal atresia is a rare anorectal malformation, with only 12 cases found in the world to date.13 We report on a girl who had a rectovestibular fistula, and complete absence of the vagina, uterus and both fallopian tubes (complete miillerian agenesis). A preliminary colostomy was performed followed by staged reconstruction, involving rectovaginoplasty and anorectoplasty using a combined abdominal and posterior sagittal approach. CASE REPORT A 10-month-old girl was hospitalized with a recent onset of constipation. She had been passing stools through the vestibule with an absent anal opening since birth. On examination the child was of average health with no other obvious congenital anomaly. The perineum was well developed and the anal opening was absent at the normal site. The labia, clitoris and urethral opening were normal. A wide opening similar to a vagina was present just below the urethral meatus. Clinical diagnosis was rectovaginal fistula. Investigations did not reveal any cardiac, renal or spinal anomalies. Karyotype was 46XX. Left sigmoid colostomy was done to be followed 6 months later by posterior sagittal anorectoplasty. No cologram was done. A detailed examination with the patient under anesthesia and panendoscopy preoperatively revealed a normal urethra and bladder. he opening presumed to be the vagina was actually a wide rectovestibular fistula and the vagina was absent. Exploratory laparotomy at the same time showed complete absence of the vagina, uterus and both fallopian tubes but both ovaries were normal (part A of figure). The distal 7 cm. of the fistula was divided and closed over as neovagina, whereas the remaining rectum and colon were brought down through the sphincter muscle complex by posterior sagittal anorectoplasty (part B of figure). Convalescence was uneventful. The colostomy was closed after 3 months following rectovaginoplasty and posterior sagittal