Ambiguous Genitalia in a 22-year-old Nigerian: A Case Report of Successful Perineoplasty and Clitoroplasty

Abstract

External genitalia are considered to be ambiguous whenever there is difficulty assigning gender to a child based on the appearance of the external genitalia. It is a common presentation of Disorders of Sexual Development (DSD). This is a case of a 22-year-old single undergraduate who had a disorder of sexual development with ambiguous genitalia resulting from congenital adrenal hyperplasia. Although the appearance of her genitalia in childhood could not be ascertained, she presented with an inability to achieve penetrative vagina intercourse, enlarged clitoris, poor urinary stream, and terminal dribbling.

She had clitoromegaly with fused labia minora at the midline which formed anterior to the urethral meatus with a normal capacious vagina behind the fused labia. She also had acne and hirsutism. Findings of investigations were suggestive of congenital adrenal hyperplasia with no salt wasting. She had successful perineoplasty and clitoroplasty and reported satisfactory appearance of her genitalia, she has been able to achieve penetrative vagina intercourse with satisfactory sexual function and urinary streams have improved with terminal dribbling resolved. She was counseled about the need for fertility support when ready.

Disorders of sexual development though rare may have significant psychosocial effects on its victims and understanding the pathology with surgical intervention as indicated in this case may help improve the quality of life of the affected persons.