- Techniques and Instrumentation
- Open Access
Uterovaginal anastomosis for the management of congenital atresia of the uterine cervix
© Springer-Verlag 2010
Received: 15 September 2010
Accepted: 3 November 2010
Published: 16 December 2010
Congenital atresia of the cervix is a rare mullerian anomaly. Hysterectomy has been advocated as the management of choice in the early days as the reproductive performance is thought to be low despite successful neo-canal creation. In recent years, conservative surgery is being recommended more frequently in patients with congenital cervical atresia and with total or partial vaginal aplasia and is shown to have a better reproductive performance. The treatment strategy should be tailored to relieve retrograde menstrual symptoms and restore fertility. Here, we report a young girl with congenital cervical atresia with upper vaginal atresia managed with uterovaginal anastomosis and review the management options and reproductive performance in such cases.
Cervical atresia is an extremely rare and complex mullerian malformation. Unlike most other mullerian anomalies, the initial surgical management of congenital cervical atresia remains controversial. Total hysterectomy eliminates the symptoms related to hematometra, but loss of reproductive function is irreversible. Hysterectomy should be avoided as first choice and reserved for cases where canalization attempts fail or are impossible. However, canalization procedures, mainly cervical drilling, may be followed by recurrent obstruction of the uterovaginal neo-canal, and persistent infertility. Hence, a majority of clinicians view hysterectomy as the optimal primary surgical management in these patients [1, 2]. Recently, small series have been reported which show an improved reproductive performance after utero-vaginal anastomosis [3, 4]. We report a 16-year-old girl who underwent uterovaginal anastomosis for cervical atresia and review the management options as well as the reproductive performance in such patients.
Atresia or agenesis of the uterine cervix is a rare developmental malformation of the female genital tract. Fujimoto et al.  reported 51 cases in the world literature. According to the Buttram and Gibbons  classification, cervical agenesis represents a Class I B mullerian anomaly whereas the ASRM classification of 1988 places it in Class II b. In approximately 50% of cases, cervical agenesis is associated with partial or complete vaginal agenesis .
Patients usually present with amenorrhea and symptoms of retrograde menstruation plus hematometra. In pubescent girls, pelvic pain in the absence of menstrual bleeding must evoke the suspicion of an obstructive genital syndrome. Clinical examination eliminates vaginal atresia or an imperforate hymen, but it may not always be possible to differentiate cervical atresia from a high vaginal septum. Transabdominal or transperineal sonography may help to identify the level of obstruction but is not so reliable in the diagnosis of cervical atresia [7, 8]. The MRI is a more reliable imaging technique, and may diagnose associated upper genital tract anomalies or problems like hematosalpinx and endometriosis [9, 10]. Laparoscopy allows an exploration of the pelvis with assessment of the internal genital organs. An intravenous pyelography (IVP) can be performed because of the frequency of urinary tract malformations associated with Mullerian ducts anomalies . MRI may be sufficient to obtain a preoperative diagnosis of cervical atresia, as in the present patient. MRI may also diagnose urinary tract malformations; hence an IVP may be omitted.
Unlike most of the other mullerian anomalies, management of congenital cervical atresia is challenging. The earliest reported case of congenital cervical atresia managed by hysterotomy and cervical canalization was described in 1900 . The most successful surgical methods employed involved a transvaginal or transabdominal approach to create a neo-ostium through the dense fibrous cervix and communicate it to the endometrial cavity and vagina. Patency was maintained by application of stents (which were retained postoperatively for varying periods of time), with or without a surrounding full or split-thickness skin graft. However, re-stenosis of fibrous tissue, post-operative severe infection, or septicemia in occasional cases resulted in the recommendation of hysterectomy for this problem [4, 11–13]. However, in recent years, there has been resurgence towards conservative surgery which is now being frequently attempted in congenital cervical atresia associated with total or partial vaginal aplasia, with an aim to preserve the reproductive capability and relieve the menstrual symptoms. A procedure that sustains cyclic menses and does not allow re-stenosis would be optimal. Canalization techniques such drilling are easier to perform, but these are associated with re-stenosis of the cervix and its complications in nearly 40–60%. Hence, the technique of utero-vaginal anastomosis described by Deffarges et al.  is now preferred as it has a lower risk of re-stenosis and can be performed even in the presence of associated vaginal aplasia. It reduces the risk of damage to the bladder and rectum and obtains a reliable canalization of uterovaginal canal. They reported only one patient out of 18 who developed re-stenosis of the uterovaginal canal leading to multiple canalization procedures, and ultimately needed salpingo-oophorectomy for pyosalpinx. Susbequently techniques using laproscopy or with laproscopic assistance have been reported. Novel techniques have been described by El Saman using laproscopic assistance (i.e., endoscopic monitored canaisation under vaginoscopic monitoring and retropubic balloon vagnioplasty). It has the advantages of requiring less laproscopic expertise and with less perineal dissection or extensive [14, 15]. However, the long-term outcome and the reproductive performances after the laparoscopic techniques are yet to be reported.
Due to the risk of retrograde menstruation and subsequent development of endometriosis, creating an outflow tract should be done as early as possible (12–16 years). In cases where hysterectomy needs to be performed for any associated pathology, creation of the neovagina may be delayed up to the age of 18–20 years. In complete aplasia of the vagina, such a two-stage procedure has been carried out successfully [16, 17].
The success of uterovaginal anastomosis depends upon the size of the created ostium, the length of the new endocervical canal, the presence of vaginal mucosa adjacent to the end of the neo-ostium, and the duration of stenting . Laparoscopically assisted utero-vestibular anastomosis is also reported, but data about post procedure reproductive performance is yet not available . The duration of the stenting has been reported to range from 3 weeks to 3 months, but in the series reported by Deffarges et al. , it was kept for 2 weeks only, and re-stenosis occurred in only 1/18. In our case, we chose uterovaginal anastomosis, as the long-term outcome and the reproductive performance after this method were reported in the literature. She is currently relieved of her menstrual symptoms. Her stent (Foleys catheter) was expelled spontaneously after 3 weeks, and she is symptom-free 9 months post-procedure.
The likelihood of spontaneous pregnancy occurring as a result of canalization appears to be low. This low fertility rate may be due to the presence of associated endometriosis and pelvis adhesions, plus the lack of normal endocervical canal glandular function. Very few spontaneous pregnancies have been reported after canalization of a completely or partially atretic cervix . Assisted reproductive techniques are usually required to help these patients to achieve a pregnancy. [20, 21]. The need of cervical cerclage is described but is controversial. In series by Deffarges et al. , 40% of the women who attempted to conceive had a successful pregnancy, and only one out o ten patients had cerclage. They should be delivered by elective cesarean as the vertical uterine incision may increase the chance of uterine rupture.
In conclusion, a conservative approach may be recommended for women with congenital cervical atresia even in the presence of vaginal agenesis. Uterovaginal anastomosis can be considered as the surgical method of choice in these women. Surgery should be performed as early as possible to avoid complications like endometriosis and its sequelae which may hamper future reproductive performance. In addition, the presence of regular menses has an extremely favorable psychological impact on these young women.
Conflict of interest
The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
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