DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20200914

A rare case report on complete cervical agenesis with vaginal atresia and suspended didelphys uterus with hematometra and left haematosalpinx

Divya Dwivedi, Madhu Jain, Shuchi Jain, Shivi Jain

Abstract


Congenital uterine malformations are deviations from normal anatomy resulting due to defective fusion of Mullerian ducts or the paramesonephric ducts in the developing embryo. These anomalies may be isolated or in combination with urological abnormalities. The mean prevalence of female congenital malformations in general population is up to ⁓ 7%. Patients with these anomalies usually present during pubertal age due to absence of onset of menses, cyclical abdominal pain, or in reproductive age group as infertility or recurrent pregnancy loss depending upon the degree of malformation. Cervical agenesis is a rare Mullerian anomaly with an incidence of 1 in 80,000 females. It represents 3% of all uterine anomalies. It is rarely associated with a functioning uterus (4.8%). Cervical agenesis is often associated with vaginal atresia (less than 50%). It is important to classify these anomalies for easy diagnosis and plan appropriate preoperative treatment.


Keywords


Cervical agenesis, CONUTA classifications, Suspensory uterus, Vaginal atresia

Full Text:

PDF

References


Stray‑Pedersen B, Stray‑Pedersen S. Etiologic factors and subsequent reproductive performance in 195 couples with a prior history of habitual abortion. Am J Obstet Gynecol. 1984;148:140‑6.

Raga F, Bauset C, Remohi J, Bonilla‑Musoles F, Simón C, Pellicer A. Reproductive impact of congenital Müllerian anomalies. Hum Reprod. 1997;12:2277‑81.

Carrington BM, Hricak H, Nuruddin RN, Secaf E, Laros RK, Hill EC. Müllerian duct anomalies: MR imaging evaluation. Radiol. 1990;176:715‑20.

Pellerito JS, McCarthy SM, Doyle MB, Glickman MG, DeCherney AH. Diagnosis of uterine anomalies: Relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology 1992;183:795‑800.

Fedele L, Dorta M, Brioschi D, Massari C, Candiani GB. Magnetic resonance evaluation of double uteri. Obstet Gynecol. 1989;74:844‑7.

Grimbizis GF, Gordts S, Di Spiezio Sardo A, Brucker S, De Angelis C, Gergolet M, et al. The ESHRE/ESGE consensus on the classification of female genital tract congenital anomalies. Hum Reprod (Oxford, England). 2013;28:2032-44.

Deffarges JV, Haddad B, Musset R, Paniel BJ. Utero-vaginal anastomosis in women with uterine cervix atresia: long-term follow-up and reproductive performance. A study of 18 cases. Hum Reprod. 2001;16:1722-5.

Olive DL, Henderson DY. Endometriosis and mullerian anomalies. Obstet Gynecol. 1987;69(3):412-5.

Acién P, Acién M, Ferrer MS. Complex malformations of the female genital tract. New types and revision of classification. Hum Reprod. 2004;19:2377-84.

Xie Z, Zhang X, Liu J, Zhang N, Xiao H, Liu Y. Clinical characteristics of congenital cervical atresia based on anatomy and ultrasound: a retrospective study of 32 cases. Europ J Med Res. 2014;19(1):1.

Farber M, Marchant DJ. Congenital absence of the uterine cervix. Am J Obstet Gynecol. 1975;121(3):414-7.

Creighton SM, Davies MC, Cutner A. Laparoscopic management of cervical agenesis. Fertil Steril. 2006;85:1510.e13-5.

Al-Jaroudi D, Saleh A, Al-Obaid S, Agdi M, Salih A, Khan F. Pregnancy with cervical dysgenesis. Fertil Steril. 2011;96:1355-6.