ROENTGENOLOGY OF THE CONGENITAL ADRENOGENITAL SYNDROME

¹ From the Department of Radiology, Indiana University Medical center, Indianapolis, Indiana

The congenital adrenogenital syndrome is a hereditary form of hyperadrenal corticism present at birth. The basic abnormality is an enzymatic defect in cortisol synthesis, resulting in adrenal hyperplasia and excessive adrenal androgen production. The three basic clinical types are: (1) simple virilism, (2) virilism and adrenal insufficiency, and (3) virilism and hypertension.

Although there are no pathognomonic roentgen findings several features are present which characterize patients with this disease. Six of a series of 43 cases are briefly reported to show some of these roentgen features.

Approximately 6o per cent of patients are of the type with simple virilism. Accelerated maturation is reflected roentgenographically in the skeleton, teeth, and muscular tissue. Five patterns of genital malformations in the female are listed, some of which can be best defined by roentgen analysis.

One-third of the cases have virilism and adrenal insufficiency. These patients present in early infancy with roentgen signs of dehydration such as pulmonary overinflation, reduced soft tissue thickness over the chest wall and sometimes a small transcardaic diameter. Plain abdominal roentgenograms may demonstrate a paucity or, occasionally, absence of intestinal gas.

The type associated with virilism and hypertension represents about 5 per cent of cases of the congenital adrenogenital syndrome. Cardiac enlargement and signs of passive pulmonary congestion may be evident on the chest roentgenogram.

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