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Radiological Cases of the Month

Osamu Arisaka, MD; Madoka Arisaka, MD; Atsuto Hosaka, MD; Naoto Shimura, MD; Keijiro Yabuta, MD; Takeshi Miyano, MD; Beverly P. Wood, MD
Am J Dis Child. 1992;146(8):949-950. doi:10.1001/archpedi.1992.02160200071029.
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A girl aged 2 years 7 months was seen because of malodorous, yellow discharge from a perineal urogenital sinus opening that had persisted for 1 month. At birth, she demonstrated ambiguous external genitalia (female pseudohermaphroditism), and a diagnosis of virilizing congenital adrenal hyperplasia due to 21-hydroxylase deficiency was made. Clitoroplasty was performed at age 1 year 6 months, but division of the labioscrotal fold and vaginoplasty were delayed. A pelvic ultrasonogram (Fig 1) and a vaginogram/cystourethrogram (Fig 2) were obtained at this presentation.

Denouement and Discussion 

Hydrocolpos in Virilizing Congenital Adrenal Hyperplasia  On catheterization of the urogenital sinus, 100 mL of yellow fluid and stagnant urine was drained, and the previously identified retrovesical cystic mass disappeared. Excretory urography showed no hydroureter or hydronephrosis. Endoscopie examination of the urogenital sinus revealed that the distended vagina entered a urogenital sinus near the external sphincter of the urethra. The examination also showed the urethra to be elongated. Vaginoplasty was performed.

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