Adrenogenital Syndrome: A Case Report

Lee, Hee Yong; Choi, Hi Suck

Abstract

A 5 year-old girl was admitted to the hospital on April 18, 1967 to be investigated for genital anomalies such as greatly enlarged clitoris, sexual hair in axillae and on pubic region, acne on the face, and excessive somatic growth which were first noticed by her parents at the age of two(Figures 1 and 2). She measured 118 cm in height (normal, 96 cm), and 20kg in weight (normal, 11kg) and was very muscular (Fig. 2 and table 1). The enlarged clitoris measured 3 cm long and showed a groove on its ventral surface communicating with a small opening from the urogenital sinus. The urogenital sinus was persitted and the labia minora are small, but the labia majora are large and resemble an open scrotum. the two labio-scrotal folds were separated and contained no exteriorized gonads. Pubic and axillary hair were scanty but acne on the face was moderately developed (Fig. 3). Nuclear sexing was female type. Her blood pressure and strum electrolytes were normal. Her bone age was around 9 years of a girl. Cystogram and vaginogram were found to be normal (Figure 4,5 and 6). Three consecutive urinary 17-ketosteroid outputs per day were 9.0, 14.0 and 11.4 mg prior to treatment. On cortisone suppression test, the amount of it dropped from 14.0 to 3.7 mg, but it was not significantly suppressed on the third to seventh days of the test to a value below two-third of the pretest level (Fig. 7). Constructive operation of feminine external genitalia including vaginoplasty and clitoridectomy was performed following a vaginogram being taken under general anesthesia on April 28. 1967 (Fig. 8). Both adrenal glands were explored on May 16, 1967 by Young's bilateral approach under general anesthesia and they were found to be hyperplastic almost three times bigger than the normal size but no tumor man was seen. After having decided that both adrenals were in a condition of marked hyperplasia, outer halves of both adrenals were excised. The removed specimens measured 4.0X1.5X 0.5 cm in the right side and 3.5X2.0X0.4 cm in the left. Histological examination showed simple cortical hyperplasia of both adrenals, but the severity was more marked in left than in right (Fig. 9-A, B). Acne subsided and hirsutism decreased one month after the initiation of cortisone therapy in addition to the subtotal adrenal resection. Although it is too early to evaluate the ultimate value of long-term suppressive therapy, clinical results at this time are encouraging.