Utero-vaginal anastomosis in women with uterine cervix atresia: long-term follow-up and reproductive performance. A study of 18 cases

J.V. Deffarges, B. Haddad,1, R. Musset and B.J. Paniel

Service de Gynécologie-Obstétrique, Centre Hospitalier Intercommunal de Créteil, 40, Avenue de Verdun, 94010 Créteil Cedex, France


    Abstract
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 Abstract
 Introduction
 Materials and methods
 Results
 Discussion
 References
 
BACKGROUND: Atresia of the uterine cervix is an uncommon Müllerian anomaly. Total hysterectomy remains the classical treatment of this malformation. The purpose of this study was to evaluate functional results and reproductive performance of women who had conservative surgical procedure. METHODS AND RESULTS: The medical records of 18 patients admitted to our centre between 1969 and 1998 for the treatment of uterine cervix atresia, were reviewed. Seven women had an associated high vaginal aplasia. Fifteen women had a history of abdominal or pelvic surgery before referral, with an unsuccessful attempt at canalization in five cases. Associated pelvic endometriosis or adhesions were observed in 12 cases. The utero-vaginal anastomosis procedure was performed successfully in all cases. A secondary stenosis of the anastomosis occurred in one case and this required canalization. Median follow-up after surgery was 4.5 years. Sexual intercourse was satisfactory for the 12 patients who began sexual activity. Ten patients had a pregnancy, four of which resulted in a total of six successful spontaneous pregnancies. Of the six remaining women, five had an evident cause of infertility. CONCLUSIONS: Utero-vaginal anastomosis should be proposed in women with congenital atresia of the uterine cervix, even when it is associated with vaginal aplasia. Early diagnosis and surgery appear necessary to avoid the development of pelvic associated lesions.

Key words: fertility/pregnancy outcome/uterine cervix atresia/uterine malformation/vagina malformation


    Introduction
 Top
 Abstract
 Introduction
 Materials and methods
 Results
 Discussion
 References
 
Atresia of the uterine cervix is an uncommon Müllerian malformation which may be associated with vaginal aplasia. Its incidence is unknown and the management of women with this malformation remains controversial. Total hysterectomy is recommended by some authors when canalization procedures fail or are impossible (Maciulla et al.1978Go; Niver et al.1980Go; Buttram, 1983; Fliegner and Pepperell, 1994Go). This latter management is certainly successful in relieving symptoms related to cervical atresia, but leads to an irreversible effect on reproductive performance. Our policy since 1969 has been to avoid removal of the uterus in order to preserve reproductive performance. Here, we evaluate long-term functional results and reproductive performance in women who had utero-vaginal anastomosis for cervical atresia.


    Materials and methods
 Top
 Abstract
 Introduction
 Materials and methods
 Results
 Discussion
 References
 
We carried out a retrospective study of women with atresia of the uterine cervix who were treated in our centre between November 1969 and June 1998. A total of 18 patients (mean age: 18.7 years, range: 13–36) were reviewed. Fifteen patients (83%) had a previous history of abdominal or vaginal surgery before referral, with an unsuccessful attempt at canalization in five cases (28%). All patients had a laparoscopy or a laparotomy and vaginal examination before our surgical procedure in order to clarify the uterine malformation and to explore the upper and lower genital tract. Associated upper genital tract malformations and complications were found in four (22%) and 15 (83%) women respectively. Seven (39%) women had associated vaginal aplasia. All patients also underwent ultrasound examination of the kidneys or intravenous pyelography: kidney agenesis, ureteral duplicity, and pelvic kidney were found in three different women. Characteristics of the patients are given in Table IGo.


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Table I. Characteristics of the 18 patients with atresia of uterine cervix (numbers in parentheses are percentages)
 
All women had utero-vaginal anastomosis. The procedure was as follow: a laparotomy was performed under general anaesthesia and in a semilithotomy position allowing both abdominal and perineal approaches. A large dissection of the anterior space (between the bladder and the uterus), and of the posterior space (between the rectum and the uterus) was performed. Then an inverted U-shaped incision was made on the perineum or vaginal tissue. The flap created by this incision, with a posterior base and anterior top, was performed to provide more available tissue and avoid stenosis on the utero-vaginal anastomosis. Through this incision, a channel between the bladder and the rectum was created by shape and blunt dissection. The dissection was continued until the channel was large enough to admit two fingers and to reach the anterior and posterior dissections achieved by laparotomy. An incision of 1 cm diameter was made on the uterine fundus, allowing catheterization of the uterine cavity with a 10-mm dilator. This manoeuvre helped to identify the upper limit of the atretic tissue of the cervix. The atretic tissue was then resected as for a cervical conization until the uterine cavity was reached. The uterus was pulled down through the pelvic channel, and sutured to the high vaginal or vestibular mucosa with separate stitches of 3–0 polyglactine. A 16 CH Foley catheter was inserted in the uterine cavity for15 days. Antibiotic prophylaxis (ampicillin) was maintained until the Foley catheter was removed. No mould was required.

Patients were assessed postoperatively at 1 and 3 months. Morphological results were obtained from medical records. To assess long-term functional results and reproductive performance, the patients were asked to complete a questionnaire; this took place an average of 54 months after surgical procedure. The questionnaire concerned dysmenorrhoea, dyspareunia, and pregnancies, including their number and results (live birth, early termination, early spontaneous abortion, ectopic pregnancy), gestational age at delivery, and mode of delivery.


    Results
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 Abstract
 Introduction
 Materials and methods
 Results
 Discussion
 References
 
The surgical procedure was successful and uneventful in all cases. Associated procedures performed for upper genital tract complications in seven patients (39%) were as follows: resection or coagulation of endometriotic nodes in three cases (17%), adhesiolysis in two cases (11%), unilateral adnexectomy and contralateral salpingectomy in one case, and unilateral adnexectomy in another case. None of the patients required blood transfusion. Histological analysis of the resected atretic tissue of the cervix carried out in seven cases confirmed the presence of conjunctive tissue without epithelium in all cases.

All the patients had a medical visit at 1 month. Median vaginal depth at 1 month was 6 cm (range: 4–9). Menstruation was restored in all patients in a median delay of 1.5 months (range: 1–5). Three patients were lost to follow-up afterwards. Among women who had follow-up, five (33%) complained of dysmenorrhoea. Finally, the 12 patients who had sexual intercourse were satisfied. A secondary low vaginal stenosis occurred in two cases (11%) far from the utero-vaginal anastomosis. This required a simple section under general anaesthesia followed by wearing of a vaginal mould for 1.5 and 5 months respectively, with good morphological and functional results. In another case, surgery was complicated by a secondary cervical stenosis, leading to dysmenorrhoea, and requiring multiple canalization procedures. Finally, a left pyosalpinx complicated these procedures and led to salpingo-oophorectomy. Because the patient had no desire for pregnancy, ovarian function was blocked with medroxyprogesterone acetate, and the patient was lost to follow-up a few months later.

Ten patients attempted to become pregnant during the study period. Their characteristics are shown in Table IIGo. Six spontaneous pregnancies resulted from four patients (40%). Cervical cerclage was performed in only one case. All pregnancies were delivered by Caesarean section between 36 and 38 weeks gestation (median birth weight 2400 g, range 2350–2700). Six women were infertile at the time of sending the questionnaire and five of them had an associated complication (Table IIGo). Two of these patients asked for assisted medical procreation. They were, however, excluded from these procedures because of uterine hypoplasia in one case, and unclear reason in the second case.


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Table II. Characteristics of patients who attempted to become pregnant
 

    Discussion
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 Abstract
 Introduction
 Materials and methods
 Results
 Discussion
 References
 
Atresia of the uterine cervix is a very uncommon Müllerian malformation associated in 50% of the cases with a vaginal aplasia (Fujimoto et al.1997Go). Any abdominal or pelvic, acute or chronic pain, in a pubescent girl must evoke an obstructive genital syndrome. The presence of a mass inside the vagina, discovered on rectal examination, suggests blood retention above an obstacle. Clinical examination easily eliminates hymeneal imperforation or blind hemivagina, but might not differentiate cervical atresia from high vagina diaphragm. Transabdominal or transperineal ultrasonography may specify the level of the obstacle (Graham and Nelson, 1986Go; Scanlan et al.1990Go; Meyer et al.1995Go), but seems not very reliable for the diagnosis of uterine cervix atresia (Fedele et al.1999Go). Conversely, transrectal ultrasonography may help to analyse the cervix, as it provides an accurate sight of pelvic organs (Fedele et al.1999Go), and three-dimensional ultrasound (Raga et al.1996Go) may contribute to analyse the external shape of the uterus. Magnetic resonance imaging (MRI) currently appears to be the most reliable morphological examination for the diagnosis of utero-vaginal malformations with a surgical correlation >80% (Barach et al.1987Go; Reinhold et al.1997Go; Letterie, 1998Go; Lang et al.1999Go). Moreover, MRI may help in the diagnosis of upper genital tract associated complications. This technique, however, is limited in case of previous surgical procedures (Lang et al.1999Go), which is very frequent in these patients. Moreover, MRI does not change radically the management of this malformation. Laparoscopic exploration has the ability to assess the type of uterine malformation, and reveals other complications of the upper genital tract that may require appropriate surgery.

Conservative surgical treatment of uterine cervical atresia, mainly cervical drilling, has been associated in some cases with deadly peritonitis (Maciulla et al.1978Go; Niver et al.1980Go). Moreover, some authors speculated that chance of subsequent pregnancies were few, (Niver, et al.1980Go; Buttram, 1983; Fliegner and Pepperell, 1994Go), particularly when associated to vaginal aplasia (Jacob and Griffin, 1989Go; Fujimoto et al.1997Go). Consequently hysterectomy was recommended as first line treatment by many authors (Niver et al.1980Go; Buttram, 1983; Fliegner and Pepperell, 1994Go). Some rare cases of successful pregnancies, however, have been recently reported after conservative surgical management of cervical aplasia (Table IIIGo).


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Table III. Published cases of successful pregnancies in women with uterine cervix atresia
 
The results of our series confirm that uterine cervix atresia may be successfully treated by utero-vaginal anastomosis even when this malformation is associated to vaginal aplasia. Because canalization techniques are at high risk of secondary stenosis of the cervix, up to 40–60% (Fujimoto et al.1997Go), utero-vaginal anastomosis should be preferred to canalization techniques, even if these latter procedures are easier to perform. In our study only one patient had secondary stenosis of the anastomosis that required multiple canalization procedure complicated by high genital infections.

Preserving reproductive performance was the aim of the surgical conservative management. None of our patients had hysterectomy. A total of 40% of the women who attempted to become pregnant had a successful pregnancy. This relatively low fertility rate should be balanced against the significant rate of associated upper genital tract complications (83% of cases in our series), and fertility rate cannot therefore be expected to be as high as in the general population. Infertility, however, may not be related to all the complications cited in Table IGo. In particular, cervical atresia was complicated by haematometra in 44% of cases, and we have previously demonstrated in women with blind hemivagina, that once the obstruction has been relieved the dilated uterus recovers its normal reproductive function (Haddad et al.1999Go). Conversely, four cases of infertility in our series are related to associated upper genital tract lesions such as tube lesions, adhesions, or endometriosis. Of interest, uterine cervix atresia may induce an abnormal endocervical glandular function as evoked by some authors (Geary and Weed, 1973Go; Jacob and Griffin, 1989Go; Thijssen et al.1990Go; Fujimoto et al.1997Go) and be also a cause of infertility that has not been analysed in the infertile women of our series. It should be noted, however, that four women had six spontaneous, successful, pregnancies. Finally, assisted medical procreation may help the infertile women to obtain a pregnancy (Thijssen et al.1990Go; Fluker et al.1994Go; Nargund and Parsons, 1996Go; Anttila et al.1999Go), provided that the uterus and the ovaries are preserved.

In conclusion, our results show that the diagnosis of uterine cervix atresia should be made as early as possible to avoid genital complications that may lead to aggressive surgery such as adnexectomy or hysterectomy. Utero-vaginal anastomosis appears to be an adequate treatment of uterine cervix atresia with good functional results. By preserving the uterus, this management allows pregnancies that may be obtained spontaneously or by assisted medical procreation procedures.


    Notes
 
1 To whom correspondence should be addressed. E-mail: bhaddad{at}chicreteil.fr Back


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 Abstract
 Introduction
 Materials and methods
 Results
 Discussion
 References
 
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Submitted on December 15, 2000; accepted on April 6, 2001.