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Congenital cervical atresia is a rare abnormality of the Mullerian System. Up until 1997, only 58 cases had been reported in the literature; of these, 48% were not associated with other abnormality in the genital tract.1
Congenital cervical atresia presents as cyclical abdominal pain, haematometra, and pelvic endometriosis including endometriotic cyst. With the recent advent of imaging techniques such as ultrasonography and magnetic resonance imaging (MRI), diagnosis of the condition has become much easier and less invasive. Usually a distended uterus, adnexal masses, and fluid in the Pouch of Douglas can be seen. In addition, with MRI, the atretic cervix without a canal can be demonstrated.2
Treatment of the condition has largely been by hysterectomy due to difficulty in the creation of a patent cervical canal or a cervicovaginal fistula.3 The laparatomy approach has recently been adopted whereby the endometrial cavity is exposed to enable a trocar needle to puncture the solid cervical tissue from the vaginal end. The tract is then dilated and a stent is inserted, but there is difficulty in keeping the stent in place. Under ultrasound guidance, however, it is possible to insert an infant chest drain through the cervical tissue to reach the endometrial cavity. The flow of dark blood from the chest drain denotes that it is in the endometrial cavity. The drain can be put in place for 18 months without causing infection.
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Cases of congenital cervical atresia should, therefore, be managed using a conservative surgical approach with the assistance of modern imaging techniques. With this approach, there is a possibility of normal menstruation and pregnancy for the individual concerned.4
The ultrasound scan images are of a 14-year-old girl who presented with primary amenorrhoea. She experienced abdominal pain after being given cyclical oestrogen/progestogen therapy. Ultrasound revealed some fluid in the Pouch of Douglas but no other findings of note (figure 1). The endometrial lining was normal. The cervix appeared small.
Examination under anaesthesia showed an atretic cervix and an infant chest drain was inserted (figure 2). The patient remained on hormone replacement therapy, but no longer complained of abdominal pain.
References
1. Fugimoto VY, Miller JH, Klein NA, et al. Congenital cervical atresia: report of seven cases and review of the literature. Am J Obstet Gynecol 1997;177:1419-1425.
2. Hovsepian DM, Auyeung A, Ratts VS. A combined surgical and radiologic technique for creating a functional neo-endocervical canal in a case of partial congenital cervical atresia. Fertil Steril 1999;10:158-162.
3. Rock JA, Schlaff WD, Zacur HA, et al. The clinical management of congenital absence of the uterine cervix. Int J Gynaecol Obstet 1984;22:231-235.
4. Fluker MR, Bebbington MW, Munro MG. Successful pregnancy following zygote intrafallopian transfer for congenital cervical hypoplasia. Obstet Gynecol 1994;84: 659-661.
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