2. Surgical Treatment of a Rare Case of 46,XY Disorder of Sexual Development

We present the surgical management of a 21-year-old patient with 46,XY PGD,who has predominantly female external genitalia with clitoral enlargement and complete vaginal agenesis. Our patient presented with primary amenorrhea and lack of breast development. Clitoral enlarge ment was present with a single urethral opening and absence of an external vaginal opening (Fig.1)Laboratory evaluation revealed low testosterone concentrations (0.3 ng/ml) with elevated FSH and LH leves.Total testosterone/dihydrotestosterone and androstenedione ratios were normal.

Source: Journal of Pediatric and Adolescent Gynecology - April 1, 2024 Category: OBGYN Authors: Ipek Evruke, Ozlem Dural, Funda Gungor Ugurlucan, Hevra Ekin Ulusoy, Inci Sema Tas, Cenk Yasa, Suleyman Engin Akhan Source Type: research