LETTER TO THE EDITOR—BRIEF COMMUNICATION

Uterovaginal anastomosis for complete cervical agenesis and partial vaginal agenesis: a case report

Report of an extremely rare case of triple Mullerian anomaly consisting of cervical agenesis, partial vaginal agenesis and complete bicorporeal uterus with functioning endometrium associated with adenomyosis and pelvic endometriosis in a young girl managed with hysterectomy of both uterine horns and excision of pelvic endometriosis.

A 20-year-old young woman presented with primary amenorrhoea and severe cyclical pain abdomen. She was diagnosed with a rare triple Mullerian anomaly consisting of cervical agenesis, partial vaginal agenesis and complete bicorporeal uterus with functioning endometrium associated with adenomyosis and pelvic endometriosis. She had undergone laparoscopic hematosalpinx drainage in an outside setting which provided her a temporary relief from symptoms. After an accurate pre-operative diagnosis of her condition, she underwent hysterectomy of both uterine horns as both the horns were grossly adenomyotic with hematometra.

This case is unique as two different developmental anomalies agenesis and lateral fusion defect were found together leading to a triple Mullerian anomaly with co-existing adenomyosis and endometriosis. Conservative surgery in this particular case had high probability for developing obstruction, sepsis or pelvic abscess later or repeat procedures/surgeries leading to increased morbidity. She was given the option for oocyte freezing along with gestational surrogacy if she desired fertility in future.

Early diagnosis and tailored intervention of Mullerian anomalies is essential to improve patients' quality of life. Definitive surgery in the form of hysterectomy should be considered if there are associated pathologies such as adenomyosis and endometriosis and findings such as hematometra.

The purpose of this systematic review was to evaluate surgical treatment for malformations of the cervix in terms of short- and long-term postoperative function and relevant reproductive outcomes.

International Prospective Register of Systematic Reviews (ID No CRD42019128899). Electronic databases were searched for eligible studies up to October 2019 on Medline/PubMed (1966–2019), Scopus/Elsevier (1950–2019), and Google Scholar (up to 2019). Search terms included “congenital cervical malformations/anomalies,” “uterocervical aplasia/dysgenesis/agenesis,” “cervical aplasia/dysgenesis/ agenesis,” “müllerian anomalies/dysgenesis/agenesis,” “utero-vaginal anastomosis,” “cervical reconstruction,” “uterocervical reconstruction/canalization,” “cervical canalization,” “reproductive/pregnancy/functional outcome,” “menstruation,” “pregnancy,” and “regular periods.”

Studies were chosen and included with clear description of preoperative diagnosis via sonography, magnetic resonance imaging, and surgical confirmation; assessment and clear description of gynecologic anatomy and any concomitant anomalies; meticulous description of the operative technique; follow-up of at least 6 weeks postoperatively; and postoperative end points including menstrual and reproductive outcomes. We included randomized controlled trials, case-control studies (both prospective and retrospective), and case reports. Data registries, studies without clearly described primary or secondary outcomes, and studies not in the English language were excluded from the analyses.

The literature search returned 745 studies; 546 records were initially excluded (397 not related to the topic, 15 not related to humans, 134 non-English language); 111 full-text articles were further excluded (patients underwent hysterectomy or no surgery); 88 studies with a total of 249 patients were suitable for analysis. Almost all patients had preoperative amenorrhea (248/249, 99.6%). Postoperatively, of 249 patients, resolution of menstruation occurred in 228 patients (91.6%), and hysterectomy was performed in 22 patients (8.8%); overall, there were 30 (12.0%) conceptions that resulted in 27 (10.8%) viable and 24 (9.6%) term pregnancies. Indications for hysterectomy were stenosis of the genital tract and sepsis. Coexisting vaginal agenesis and use of full thickness skin graft for creation of neocervix was associated with negative surgical outcomes.

This review suggests that conservative surgical approaches result in better clinical and reproductive outcomes than more aggressive reconstructive surgeries for patients with malformations of the cervix.

The prevalence of congenital cervical agenesis or dysgenesis ranges from 1/80,000 to 1/100,000, and in about 50% of these cases it coexists with congenital vaginal agenesis. This narrative review summarizes the contemporary knowledge in the field of conservative surgical restoration of the reproductive tract. The management of congenital cervical malformations aims to [1] provide relief from the obstructive symptoms, [2] establish normal sexual function, and [3] preserve the uterus for future fertility. In cases of cervical agenesis and vaginal aplasia, the surgical approach involves the creation of neovagina, the creation of neocervix, and then subsequent restoration of the continuity of the genital tract. In cases where vagina is not congenitally absent, the surgical approach involves either a direct uterovaginal anastomosis or initial creation of neocervix and then subsequent restoration of the continuity of the genital tract. The neocervix can be surgically created with small intestinal submucosa, split-thickness skin graft, full-thickness skin graft, peritoneal flap, or vaginal mucosa lined with a polytetrafluoroethylene graft. Most of the published cases report long-term menstruation and sporadic pregnancies. Conservative surgery of cervical congenital malformations could serve as a first-line treatment. Sexual function and menstruation are established in the majority of patients. Extirpatory surgery may be preserved for surgical failures after initial restoration of the continuity of uterus-cervix-vagina or in cases with more complex anatomy.

Background: The main objectives of treatment of cervical agenesis were symptom relief, achievement of regular menstruation and restoring fertility and management of these cases represents a challenge as in many cases treatment fails and hysterectomy may be needed. In this case report a simple method was used and proved effective. Case report: A size 16 Silicone Foley’s catheter was used to create a uterovaginal tract through combined abdominal and vaginal approaches in 18 years old patient with the diagnosis of the uncommon cervical agenesis and hematometra of 24 weeks pregnant uterus size. Postoperatively three courses of cyclic estrogen/progestagen therapy were given for 20 days every month. Three days after the first course, the first menstruation occurred through the uterine catheter. The Foley’s catheter was removed five weeks postoperatively. The patient was followed up for a year, and menstruation occurred at regular monthly intervals. Conclusion: The procedure promises to be a simple and reasonably effective method for the creation of a menstrual outflow tract for cases of cervical agenesis with functioning uterus.