Vaginal Agenesis and Atresia of the Uterine Cervix Associated to Vestibular Fistula: Successful Staged Combined Laparoscopic and Perineal Approach Preserving the Uterus

Aim:

Show a successful staged laparoscopic treatment of a patient previously operated for an ARM with a nondiagnosed associated vaginal agenesis and atresia of the uterine cervix.

Case:

A 13-year-old female presented with severe acute pelvic pain. She had undergone an anorectoplasty through a posterior sagittal approach at the age of 2 with the diagnosis of a vestibular fistula. Although presenting normal puberal parameters, she had never had menses. No vaginal opening was found at a perineal examination. Ultrasonography showed a hematometra and a left complex adnexal mass. An initial laparoscopic approach showed a single uterus with a normal external appearance but distended with blood and left ovarian endometrioma and hematosalpinx that were removed and drained, respectively. As there was no evidence of associated hematocolpos, a cervical atresia associated to vaginal agenesis was suspected and a drain was placed in the fundus. Menses were inhibited using analogs while planning future reconstruction allowing psychological support. An MRI confirmed total vaginal agenesis associated to atresia of the cervix. A combined laparoscopic and perineal approach to enable sigmoid vaginal replacement, cervical canalization, and a uterovaginal anastomosis followed.

Technique:

For the initial procedure, we used a 5-mm lens and two working ports placed at the right lower quadrant and suprapubic space. The fundus was incised perpendicularly using monopolar cautery and a probe was inserted and exteriorized through the suprapubic port entry. Three working ports were used for definitive reconstruction. Bowel adhesions secondary to colostomy take down and endometrosis were freed. A 15-cm-long distal sigmoid was isolated. Dissection between the urethra and rectum followed. Linear staplers were inserted from this approach to transect the colon. The uterine cervix area was dissected preserving its vascular supply. Recanalization of its lumen and resection of fibrosis were achieved using both approaches. Enlarging the suprapubic port entry was used to facilitate suturing of the proximal end of the neovagina around the cervix.

Results:

The operative time was 210 minutes. The patient presents irregular menses without clinical and ultrasonographic evidence of infection or obstruction after a 38-month follow-up period.

Discussion:

(1) Agenesis or atresia of the cervix uteri is an uncommon entity. It is often associated with the absence of the vagina. The diagnosis is difficult to make before surgery. Ultrasonography and MRI show hematometra, but sometimes it may be difficult to distinguish between cervical atresia and a high tranverse vaginal septum. Although there is general agreement that if the cervix is absent, without any cervical stroma left, hysterectomy is advisable to prevent ovarian endometriosis and pelvic infections, preservation of the uterus may be intended in selected patients. We have previously treated four patients with combined vaginal and cervix agenesis using a combined laparoscopic and perineal approach. Laparoscopy was useful to define the anomaly and to complete hysterectomy after the evidence of total cervix aplasia and to perform a sigmoid vaginal replacement. This is our first patient undergoing a long-term successful laparoscopic-assisted sigmoid vaginal replacement, cervical canalization, and uterovaginal anastomosis even after previous abdominal and perineal surgery (sigmoid colostomy and posterior sagittal anorectoplasty). (2) The diagnosis of a uterovaginal anomaly is a common misleading finding in patients with vestibular fistula. A meticulous perineal examination is mandatory in newborns with this anomaly to plan combined vaginal and anorectal reconstruction avoiding redo surgery and sequela related to obstructive functional mullerian ducts. We have previously reported a combined endoscopic and laparoscopic initial assessment as a less invasive and time-consuming approach for atypical ARM like the one presented.

No competing financial interests exist.

Runtime of video: 5 mins