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"A 4 year boy was admitted to the hospital on Dec. 7, 1971 to be investigated for genital anomalies such as greatly enlarged clitoris like phallus, sexual hair on pubic region, abnormal pigmentation on the pubic region, and excessive somatic growth. He measured 116cm in height and 18kg in weight, .`and was very muscular. The phallus measured 4 cm long and was bent to the urogenital sinus on which a small external urethral orifice showed. The labia majora were large and resemble a bifid scrotum, but which contained no gonads. Nuclear sexing was female type and urinary 17-ketosteroid output per day was 13.44 mg/24 hour urine. Panendoscopy showed a normal bladder and an opening 2.5cm apart from the external urethral orifice was noticed and it was thought to be communicating to the internal sex organs. Urethrovaginocystoram was found to be normal bladder, uterus, and vagina (Fig. 1). ; Presacral air insufflation showed bilateral adrenal hyperplasia (Fig.2). Exploratory laparotomy was performed to confirm the diagnosis of adrenocortical hyperplasia on Dec. 13, 197l. A normal bladder and female internal sex organs such as uterus, vagina, and ovaries were confirmed and both adrenals were exp1ored. Both adrenals were found to be hyperplastic almost three times bigger than the normal size. but no tumor mass was seen. After having decided that both adrenals were in a condition of marked hyperplasia, total adrenalectomy on right side and partial adrenalectomy on left were performed. The removed right adrenal measured 6.5X4.8X1.2 cm and weight 12 gm. The left adrenal specimen was measuring 6.0x 2.5XO.8 cm and weighing 4. 5gm. Histological examination of the adrenals showed bilateral cortical hyperplasia, diffuse type(Fig. 3) and biopsy of the ovaries showed normal ovaries with many premodial follicles."
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