Anorectal agenesis with rectovaginal fistula are extremely rare type of anorectal malformations (ARM) characterized by the absence of the anus and abnormal connection of the rectum to the vagina. Uterus didelphys and longitudinal vaginal septum are varieties of mullerian anomalies.
A full-term two-day old baby girl with a birth weight of 2800 g, was referred to hospital for the absence of an anal opening. Upon admission, she passed a small amount of meconium through vagina Physical examining showed a soft abdomen and an absent anal opening, however, normal urethral and vaginal orifices were identified and a small spot of meconium seen through the hymen Echocardiogram showed an atrial septal defect and ventral septal defect. The whole-body skeletal survey and the abdomen ultrasonography were unremarkable and she was then scheduled for surgery.
In the operation room, perineal examination under anesthesia revealed longitudinal vaginal septum . Therefore, a sigmoid loop colostomy was performed as a first management stage. The postoperative course was uneventful and the patient was discharged home in stable condition. On a follow-up visit, a distal loop colostogram was performed showing the rectum is connected to the top of the vaginal vault. Then, this contrast delineated the vagina and drained out of the perineum .This radiological finding was suggestive of the high location of the rectovaginal fistula.
At the age of one year, the baby underwent laparoscopic-assisted pull-through procedure. The operation started by opting for an open method for umbilical port insertion and pneumoperitoneum was created by CO2 pressure maintained at 10 mmHg. The laparoscopic intervention was carried out.laparoscopic exploration revealed that the rectum is connected to the dome of the vagina and along with it, the didelphys uterus is communicating with the vaginal dome on both sides of the rectovaginal fistula . Then, laparoscopic rectal dissection was carried out at the level of peritoneal reflection by using ultra-scissors. The dissection continued until it reached the rectal pouch, where it becomes narrow at the level of the rectovaginal fistula. The fistula was trans-ligated using vicryl 3/0 stitch and divided in close proximity to the vagina
The anal area was mapped on the external surface of the perineum. Using a muscle stimulator, the anterior and posterior limits of the future anus and a 1.5 cm midline vertical incision was made over the perineum at the site of the proposed anal orifice is determined. The intersphincteric plane was dissected bluntly from its caudal end and continued cephalic in the midline under laparoscopic guidance. The tract was then dilated radially and the rectal pouch was grasped and pulled down to the perineum within the sphincter muscle complex. Finally, the anastomosis was completed between the pulled rectum and new anus, using an interrupted 5-0 vicryl suture. At the end of the procedure, the vaginal septum was resected using diathermy.
Oral feeding was resumed on the first postoperative day, then she was discharged home on the fifth postoperative day. An anal dilatation program was started postoperatively. The reverse colostomy was performed after six weeks of the definitive surgery. The cosmetic appearance was also satisfactory. However, a long term follow-up was recommended.
In conclusion ARM with isolated RVF is a rare variant of ARM, it should be differentiated from persistent cloaca by perineal examination in order to establish a proper surgical procedure. Being aware of the genitourinary anomalies in female patients with ARM could eliminate serious future complications during pregnancy and delivery.
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