Congenital Absence of the Cervix - Its Place in the Present Classification of Female Genital Tract Malformations

ABSTRACT

Aims and Objective: To outline and group cases of cervical agenesis / dysgenesis according to the associated uterine and vaginal abnormalities, as per the proposed ESHRE/ESGE CONUTA classification. Methods: The study was conducted in a tertiary care hospital and involved a total of 93 young girls diagnosed as having an absent cervix. Patients with utero-vaginal anomalies alone, with a patent cervix - such as uterine septae, uterus didelphys, bicornuate uterus; or transverse vaginal septae, imperforate hymen and complete or partial vaginal atresia, were excluded. All cases were subjected to specific clinical and local examination. Detailed 2 D Ultrasound was done to study the uterus. Identification of the cervix, its presence or absence, length of the cervical canal, hemato-cervix or hematocolpos was done by trans-abdominal route. All the cases were operated by the same team of surgeons, with the first author as the chief surgeon, to confirm and make a final diagnosis. They were then tabulated as per the CONUTA classification. Result: There was absence of the cervix (aplasia / dysplasia) in 93 cases as per the clinical examination, ultrasound, MRI and operative findings. There was complete or partial vaginal atresia (aplasia) in 69 (74.19%) cases and a normal vagina in 22(23.65%) cases. There were 33 cases (35.4%) with a normal uterus, with no cases of T-shaped uterus. There were 29/33 cases (87.89%) without vagina. There was one case with septate uterus and complete cervical and vaginal aplasia. Cases with two Bicornuate uteri with a common aplastic/dysgenetic cervix was seen in 4 cases. In 3 cases the cervix was partially patent in the upper side and obstruction was present only in the lower 4-5mm, causing ‘hemato-cervix’. There were 14 cases (15.1%) with unilaterally formed uterine horn, with the non-communicating rudimentary horn. There were 13 cases (13.9%) of cervical aplasia of the unilateral formed uterine horn. In 11 out of 93 cases (11.8%) of cervical aplasia, uterine aplasia / dysplasia and rudimentary horns with cavity were present. Conclusion: Our findings underscore the fact the patients with congenital absence of the cervix present a diagnostic challenge and that this entity should be thoroughly evaluated. The new ESHRE/ESGE classification system has the potential to overcome the limitations of the previous classification systems as it provides an effective and comprehensive categorization of almost all the currently known anomalies of the female genital tract.