Congenital vaginal atresia is a rare abnormality of the female reproductive tract which occurs as a result of failure of canalisation of the urogenital sinus^[1].The prevalence of female genital tract anomalies is 4-7% amongst general population and  as common as 8-10% in women with recurrent pregnancy loss ands approximately 3% in patients with infertility. Vaginal atresia, a rare Müllerian anomaly whose incidence is 1/4000 to 1/10000 people, is frequently associated with either normal lower vagina or the absent vagina^[4].

We report an uncommon case of a 13yearold girl with a primary amenorrhea associated with cyclical pelvic pain. Ultrasound examination revealed hematometrium and Magnetic Resonance Imaging (MRI) revealed hematometrocolpos secondary to imperforate hymen and left ovarian hemorrhagic cyst. The diagnosis of partial vaginal aplasia with normal uterus was diagnosed intraoperatively and the patient underwent diagnostic vaginolaparoscopy with laparoscopy guided vaginoplasty. The evolution was satisfactory with regular cycles and improvement of pelvic pain.

Congenital vaginal atresia is a rare malformation which presents as primary amenorrhea with chronic cyclic pelvic pain. Diagnosis is based on clinical examination and imaging. The MRI is designed to assess the atresia and guide surgical management while the surgical technique aims to restore the integrity of the utero-vaginal tract.

Surgical reconstruction is possible in these cases. Extirpative surgery may be preserved for surgical failures.Uterovaginal anastomosis should be considered in difficult cases, especially those prone for stenosis. Early surgery is essential for favourable results. Clinical judgement of the surgeon is most important. It is important to determine the malformation during surgery. Also it is of crucial importance for us to follow up the patient well into reproductive and obstetric outcomes in future. The postoperative results are usually satisfactory: menstruation and sexual activity are established in the majority of the patients.

Keywords: Congenital vaginal atresia, hematometrocolpos, vaginoplasty, Mullerian anomaly.

1. Slaoui A, Benzina I, Talib S, Etber A, Zeraidi N, Lakhdar A, Kharbach A, Baydada A. Congenital vaginal atresia: about an uncommon case. Pan Afr Med J. 2020 Sep 17;37:69. doi: 10.11604/pamj.2020.37.69.21682. PMID: 33244332; PMCID: PMC7680234.
2. Okoro PE, EliSS. Congenital vaginal obstruction in young girls: need for standard post-operative dilatation protocol. Int Surg J2019;6:207-10.
3. Mei L, Zhang H, Chen Y, Niu X. Clinical features of congenital complete vaginal atresia combined with cervical aplasia: A retrospective study of 19 patients and literature review. Congenit Anom (Kyoto). 2021 Jul;61(4):127-132. doi: 10.1111/cga.12417. Epub 2021 May 4. PMID: 33829535; PMCID: PMC8360086.
4. Regmi, D. R., Dangal, G., Karki, A., Pradhan, H. K., Shrestha, R., Bhattachan, K., Tiwari, K. D., Bharati, S., Manandhar, P., & Maharjan, S. (2021). Vaginal Atresia with Cervical Agenesis, a Rare Congenital Malformation. Kathmandu University Medical Journal, 19(3), 390–392. https://doi.org/10.3126/kumj.v19i3.49746.

Dr R. S. Ramya

MBBS, DGO, DNB (Obg) (Endogynaecology Fellowship Resident, Siva Hospital)