Congenital urogenital sinus anomaly without Adrenal Hyperplasia

Urogenital sinus anomaly is a mal-development of female urogenital system, usually associated with Congenital Adrenal Hyperplasia and may varied in presentation according to embryologic separation and length of sharing of common channel between vagina and urethra. We report a case of pure urogenital sinus anomaly of 2.5 years female child without any feature of Congenital Adrenal Hyperplasia or any other associated anomalies which is quite uncommon. The patient was diagnosed with thorough physical, biochemical, radiological and endoscopic evaluation. She had clitiromegaly, very poorly developed fused Labia minoras with a central hole, the length of common channel was about 2.5 cm and the level of vaginal confluence was at the mid portion of common channel. After in general counseling to parents, according to their desire she was operated. We performed Labialo-clitoro-vagino-urothroplasty and from post operative period to till now (after 3 months of operation) she is uneventful. Both functionally and cosmetically she is sound and her parents are also happy.