Hum. Reprod. Advance Access originally published online on January 12, 2008
Human Reproduction 2008 23(3):548-553; doi:10.1093/humrep/dem420
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Cervicovaginal agenesis: spontaneous gestation at term after previous reimplantation of the uterine corpus in a neovagina: Case Report
1 Service of Obstetrics and Gynecology, University Hospital of San Juan, Campus of San Juan, Alicante, Spain 2 Department/Division of Gynecology, Miguel Hernández University, Campus of San Juan, Alicante, Spain
3Correspondence address. E-mail: acien{at}umh.es
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We present an exceptional case of a patient with complete cervical atresia and total vaginal aplasia. After McIndoe vaginoplasty, cervicoitsmic resection with implantation and reimplantation of the uterine corpus in the neovagina, and conservative surgery for endometriosis, the patient had normal menstruations and became pregnant spontaneously. A Caesarean section was performed at week 36. This is the third published case report of a successful spontaneous pregnancy and Caesarean section at term in a patient with complete cervicovaginal aplasia, and the first published case study of a patient becoming pregnant after McIndoe vaginoplasty, cervicoistmic resection and utero-neovaginal anastomosis. Gestation developed successfully without cerclage. We recommend conservative surgery in patients with congenital cervical atresia. McIndoe vaginoplasty should be performed as soon as possible in adolescence if there is associated vaginal aplasia. Nevertheless, fibrotic stenosis can occur, even after several years and, therefore, additional operations and uterine reimplantation may be required.
Key words: cervicovaginal agenesis/vaginal aplasia/Müllerian anomalies/McIndoe vaginoplasty/utero-neovaginal anastomosis
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Cervicovaginal agenesis is a complex Müllerian malformation that generally presents with a normal functional uterus and endometrium and apparently normal external genitals and tubes. It is an uncommon and rare anomaly that causes serious complications. In our opinion, this malformation should be grouped with Müllerian anomalies that exhibit failure or absence of the Müller tubercle (vagina) and of the diverging portion of the Müller ducts (cervical atresia) (Acién et al., 2004
; Sánchez-Ferrer et al., 2006). Class Ia of the ASRM Classification for uterine anomalies (American Fertility Society, 1988) includes cases with vaginal agenesis coupled with a functional uterus and endometrium; the uterus may be normal or present fusion or resorption defects and the cervix may be present, absent or hypoplastic (Grimbizis et al., 2004; Sparac et al., 2004). Cases with cervical agenesis are in Class Ib. Therefore, the true incidence of congenital cervical atresia is somewhat difficult to determine. Partial or complete cervical atresia has been described in the presence or absence of vaginal atresia. A relatively common Müllerian anomaly, such as congenital Müllerian agenesis corresponding to Mayer–Rokitansky–Kuster–Hauser syndrome, may occur with variable degrees of upper reproductive tract development that range from either one or both functioning cavitated hemiuteri to a rudimentary uterus to the complete absence of Müllerian tissue (Bryan et al., 1949; Jones and Wheeless, 1969; Griffin et al., 1976). The frequency of congenital vaginal atresia is reportedly 1 in 4000 to 1 in 10 500 female births (Griffin et al., 1976), with normal development of the uterine corpus observed in <10% of these patients. However, the reported cases with congenital cervical atresia are frequently associated with partial or minimal vaginal aplasia of the upper part and cases with complete vaginal aplasia usually present partial or minimal low cervical atresia. Complete cervical and vaginal atresia is very rare. To that end, very few reported cases have been identified. After puberty, primary amenorrhoea may be the only symptom of cervicovaginal atresia, but there is usually cyclic pelvic pain that worsens with time. Physical examination generally reveals normal external genitals, the absence of a vagina and, in the rectal examination (and transrectal ultrasound), a normal or thickened uterus, with or without pelvic tumours corresponding to hematometra, hematosalpinx or endometriosis. There may be important post-surgery complications and, as the reproductive capacity is low despite successful creation of a neovagina, a hysterectomy is recommended as an elective procedure, even in young patients (Niver et al., 1980; Rock et al., 1984). Despite this recommendation, conservative surgery is now becoming more frequently recommended in patients with congenital cervical atresia and with total or partial vaginal aplasia. In this communication, we present an exceptional case of a patient with complete congenital cervical atresia and vaginal aplasia who, after neovagina creation and implantation and reimplantation of the uterine corpus in this neovagina, had normal menstruations and became pregnant spontaneously. A Caesarean section was successfully performed at week 36. |
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A fourteen-year-old girl who experienced primary amenorrhoea and cyclic pelvic pain was sent to our hospital in 1992 with a diagnosis of vaginal agenesis. The clinical examination showed appropriate general feminization, normal external genitals and the absence of a vagina. The rectal exam revealed thickening of the uterus and left adnex. The ultrasound evidenced the presence of uterine and left adnexal cysts. Urography indicated that both kidneys were normal. MRI (Fig. 1A) revealed that the uterus and endometrial cavity were normal with complete cervicovaginal agenesis. As dictated by the above results, the following surgeries were planned:
- Diagnostic laparoscopy to confirm the presence of a normal uterus and adnexes, and showing adhesions and endometriomas on the left side. Through laparoscopic puncture of the uterus, a contrast agent was introduced and an hysterosalpingography image was obtained (Fig. 1B). Hematometra was not observed. The uterine cervical portion and the right tube were not filled, but the left tube was filled and indicated permeability and normal peritoneal spillage, although it was distorted by the adhesions and endometriomas.
- Creation of a neovagina, according to the McIndoe technique, using a Dexon prosthesis and skin graft taken from the right buttock.
- Laparotomy, bladder separation, traction with Pozzi tenacula of the atretic cervical tract, opening of the neoformed vaginal fundus, exposing the upper side of the prosthesis and resection of the cervical fibrous tract without injuring the uterine vessels (see diagram in Fig. 2). Cervical drilling was performed on the upper side until the endometrial cavity was penetrated. A vesical sound was introduced into the uterus and vaginal extraction was performed via the light of the Dexon prosthesis. Implantation of the uterine corpus in the neovagina required suturing of the myometrium and perimetrium to the edges of the buttonhole opened on the neovaginal fundus. Adhesiolysis and conservative surgery for endometriosis were performed.
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The post-operative period was normal. Antibiotic treatment, endouterine sound and the prosthesis were maintained for nine days. Following this period, the prosthesis and sound were removed, the neovagina was made aseptic and the prosthesis was replaced. The patient was discharged from the Hospital with the indication of placing and keeping the prosthesis in the neovagina every night. The neovagina developed proper squamous epithelium. However, the scar on the utero-vaginal fundus soon became stenous and, after 2–3 menstruations, amenorrhoea and cyclic dysmenorrhea reappeared. Therefore, some months later (1993), the laparotomic surgery, a higher cervical resection and reimplantation of the uterine corpus on the neoformed vaginal fundus, now properly epithelized and consolidated, were repeated. Afterwards, the patient had cyclic menstrual bleeding and normal ultrasound check-ups for 3–4 years; however, in 1998, after 2 years without check-ups, she had strong post-menstrual abdominal pain and was admitted to the Emergency Unit of another hospital where she was diagnosed with breakage of endometriomas. A laparotomy and "partial resection of both ovaries with endometriomas" were performed. A posterior check-up in our Hospital in November 1998 was normal, with a normal transvaginal ultrasound, normal levels of CA-125 and normal cyclic menstrual bleeding.
In 2001, she presented with scarce menstrual bleeding, intermenstrual spotting and dysmenorrhea. In the transvaginal ultrasound, post-menstrual hematometra and some cysts suggestive of adhesions on the left adnex were observed. As the menstruations were becoming scarcer and the dysmenorrhea stronger, a new laparotomic surgery was performed. This surgery, which was performed in January 2002, involved lysis of the adhesions, wide opening of the vaginal fundus, cone-shape resection higher in the uterus (Fig. 3) with progressive sections of the low uterine portion up to the endometrium and uterine cavity and reimplantation or utero-neovaginal anastomosis. An endometrial biopsy was normal (secreting endometrium) and a conservative surgery for endometriosis was performed.
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After this last operation, normal cyclic menstrual bleeding returned, dysmenorrhea disappeared and the yearly ultrasound check-ups were normal. She had normal intercourse and became pregnant spontaneously in May of 2006. She was constantly observed during the pregnancy. Cervical cerclage was not done because of the absence of a cervix. The gestation reached week 36 and, in January 2007, after a normal gestational ultrasound and documentation of a fetal weight of 3000 g, we performed a Caesarean section (Fig. 4). The normal newborn was born with normal posterior evolution. The transvaginal ultrasound check-up 2 months after the Caesarean section showed a normal uterus and endometrium and absence of the uterine cervical portion.
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As mentioned above, complete atresia of the vagina and cervical uterine portion with a functioning endometrial cavity is a rare and complex anomaly. Sometimes, there is also uterine or associated tubal malformation and, naturally, endometriosis, depending on the time with respect to retrograde menstrual bleeding and cryptomenorrhoea. In our case, we could not confirm the permeability of the right tube, which may have segmentary atresia. We noticed and resected endometriomas and adhesions on the left side each time that we performed a laparotomy. In addition, the report provided when she was laparotomized in a different hospital (in 1998) due to pain and possible breakage of endometriomas states that "partial resection of both ovaries with endometriomas" was performed. Given these frequent alterations associated with the cervicovaginal agenesis, the risk of ascending infections and the low reproductive capacity despite successful creation of a neovagina, hysterectomy is recommended as an elective procedure, even in young patients. In cases of cervical agenesis, attempts to make the cervix permeable usually fail, patients generally have serious complications and normal menstruations and posterior pregnancy are rarely achieved (Niver et al., 1980
; Rock et al., 1984). However, in our case, despite the need of several operations (reimplantation of uterine corpus in the neovagina three times), menstrual bleeding remained normal for several years, there have not been any ascending infections, and the woman became spontaneously pregnant. We did not think that gestation could be maintained for longer than 3 or 4 months due to the absence of the cervix and uterine closing of a cervical cerclage; however, gestation continued normally up to week 36. At this point, with a proper fetal weight, we performed a Caesarean section at the lower edge of the present uterine corpus.
The creation of a neovagina and uterine opening or anastomosis into the neovagina should be done as soon as possible (at 12–14 years) to avoid endometriosis. It may be preferable to perform these procedures in two stages (Bugmann et al., 2002). That is, the utero-vaginal anastomosis should be performed after the neovagina is epithelized and consolidated. For vaginoplasty, we believe that the McIndoe technique is optimal, using an inert material prosthesis and skin graft. Other techniques (such as Vecchietti's, or using sigmoid bowel, bladder mucous, peritoneum, etc.) are currently recommended, but our experience in limited to the McIndoe technique. We now use this technique with Interceed between the skin and prosthesis and have good results in all cases. Nevertheless, if a hysterectomy is required due to associated pathology, creation of the neovagina must be postponed until the age of 18–20 years, at which time, surgery is technically easier, the patient is more mature and the surgery can be followed by sexual intercourse.
After serious complications in their cases, including death, Geary and Weed (1973) realized that the patients with cervical agenesis (and those with cervical atresia) required definitive surgery (hysterectomy). Further, they suggested that creation of an artificial duct would allow for, rather than inhibit, an ascending infection and that the absence of cervical mucus would make conception highly improbable. For these reasons, heroic surgical efforts to preserve fertility would not be justified in most patients, especially if cervical and vaginal agenesis co-exist (Butram and Reiter, 1987). However, as observed in our case report, cervicovaginal atresia can be treated with surgery, with respect to menstrual bleeding and posterior pregnancy. Sing and Devi (1983) communicated a case in which they had performed surgical reconstruction of the uterus and vagina. Despite the occurrence of fibrotic stenosis in this case, a utero-vaginal fistula remained and allowed for a successful posterior pregnancy. Also, successful pregnancies were reported by Welker et al. (1988) after utero-vaginal anastomosis and by Hampton et al. (1990) after vaginoplasty and cervical drilling; there was atresia of the upper part of the vagina in the former case and vaginal and partial agenesis of the cervical portion in the latter. Fujimoto et al. (1997) revised the 58 cases of congenital cervical atresia reported in the literature, including their own 7 patients. Of these patients, 48% had isolated congenital cervical atresia with a normal vagina, whereas the remainder had either complete or partial vaginal atresia. Normal menstrual bleeding was achieved in 59% of the patients who underwent utero-vaginal canalization procedures (23/39). Four of these patients subsequently became pregnant and were delivered at term. They conclude that surgical canalization in selected patients with congenital cervical atresia can be successfully performed to provide an opportunity for conservative management, resulting in normal menstrual bleeding, resolution of cyclic pelvic pain, and some potential for fertility. However, posterior fibrotic stenosis usually occurs and further operations should be anticipated.
Deffarges et al. (2001) performed utero-vaginal or utero-vestibular anastomosis in 18 patients with uterine cervix atresia of whom 7 had associated high vaginal aplasia. A secondary stenosis of the anastomosis occurred in one case, and two out of the seven cases with vaginal aplasia had successful pregnancies. Likewise, Chakravarty et al. (2000) operated on 18 women with congenital cervicovaginal atresia using a new technique of surgical canalization (vertical utero-cervical incision by laparotomy, IUD as a cervical stent, anastomosis to vault the vagina and vaginoplasty with a plastic mold and amniotic membrane). Two of the cases had posterior pregnancies, but only one of them had complete cervicovaginal atresia. More recently, laparoscopically-assisted utero-vestibular anastomosis was reported by Creighton et al. (2006) for 1 case and by Fedele et al. (2007) for 12 cases with uterine cervix atresia and vaginal aplasia. So far, these patients have not become pregnant. We believe that utero-vaginal anastomosis is an appropriate method in cases of cervical atresia or atresia in the upper vagina, but, in case of vaginal aplasia, it seems preferable to perform a vaginoplasty first.
Nine published cases of successful pregnancies in women with cervicovaginal atresia are found in the literature; they are summarized in Table I. There are only three cases (apart from ours) with complete cervicovaginal atresia. Nargund and Parsons (1996) reported one of these cases. Pregnancy was achieved by IVF through the utero-vaginal fistula. Sing and Devi's case (1983) presented fibrotic stenosis, but a utero-vaginal fistula remained, which allowed for a successful pregnancy. The case reported by Chakravarty et al. (2000) became pregnant after vertical utero-cervical incision, anastomosis and vaginoplasty with a plastic mold and amniotic membrane. Therefore, our case is the third published case with a spontaneous successful pregnancy and Caesarean section at term in a patient treated for complete cervicovaginal atresia. It is the first case of a successful pregnancy after McIndoe vaginoplasty, cervicoisthmic resection and reimplantation of the uterine corpus in neovagina (utero-neovaginal anastomosis). She has required repeated operations, but has had menstrual bleeding for 15 years and a spontaneous successful gestation without cerclage or complications.
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Therefore, we recommend conservative surgery in patients with congenital cervical atresia, including the performance of McIndoe vaginoplasty as soon as possible. This should be done in adolescence if there is associated vaginal aplasia, with resection of the atretic cervix and implantation and anastomosis of the uterine corpus to the neovagina once the neovagina is properly consolidated and epithelized. Despite these measures, stenosis can occur in later years and, therefore, further operations and uterine reimplantation should be anticipated.
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Submitted on September 6, 2007; resubmitted on November 30, 2007; accepted on December 13, 2007.
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