Vaginal Atresia is a birth defect or congenital anomality of the female Genitourinary System that manifests itself in the absence of a vagina (vaginal agenesis), or a deformed and nonfunctional vagina (Vaginal Atresia). It may occur as an isolated developmental defect or as part of a complex of anomalies.

It is frequently associated with Rokitansky-Mayer-Küster-Hauser (RMKH) syndrome, in which the most common result is an absent uterus in conjunction with a deformed or missing vagina, despite the presence of normal ovaries and normal external genitalia. Other examples include Bardet-Biedl syndrome, Kaufman-McKusick syndrome, Fraser syndrome, and Winters syndrome.

The situation is most urgent where the normal uterovaginal outflow is obstructed. In this case prompt medical action is required. Delayed detection is common and can result in major risk to the female patient because of associated urinary tract anomalies. Isolated vaginal atresia is an extremely rare finding. Most cases occur as müllerian agenesis or are associated with the more common RMKH syndrome

Prevalence

Vaginal atresia is estimated to occur in 1 in 4000–5000 live female births. It is often unnoticed until adolescence, when pain and a lack of menstrual flow indicates the condition. When a doctor diagnoses Vaginal Atresia, there are numerous remedies based on the exact details of the condition. In some cases, surgery can repair the defect or a new vagina can be fabricated using an intestinal graft.

Treatment

• Medical Therapy - The role of medical management of vaginal atresia is limited. In patients with functioning endometrium and an obstructed outflow tract, temporary hormonal manipulation of the menstrual cycle may be necessary until a patent genital tract can be created.
• Surgical Therapy - multiple reconstructive options available for vaginal reconstruction indicate that no single technique has gained uniform approval. Pediatric surgeons, gynecologists, and plastic surgeons have traditionally contributed to the literature on the subject, and specialists in each field continue to be involved in the treatment of patients with vaginal atresia. A multidisciplinary approach is recommended to best meet the challenge of providing good cosmetic results with optimal functional and reproductive outcomes.