Testicular adrenal rest tissue in a patient with classical congenital adrenal hyperplasia: color Doppler findings

Abstract

Congenital adrenal hyperplasia (also known as congenital adrenogenital syndromes) refers to a group of autosomal recessive diseases characterized by altered cortisol production, which may be associated with aldosterone deficiency. The absence of cortisol synthesis stimulates corticotropin production by the adrenal cells and the accumulation of cortisol precursors, which will be diverted for the production of sex hormones. In affected males, ectopic adrenal tissue frequently develops, usually at the testicular level. This tissue is absolutely identical to that of the adrenal gland itself, and its functionality can be stimulated by ACTH and suppressed with glucocorticoid therapy. The authors report the case of a male patient with classic congenital adrenal hyperplasia, who was referred to our staff for evaluation of bilateral testicular tenderness and enlargement. Color Doppler sonography revealed mild enlargement of both gonads, widespread disruption of the testicular echostructure, and hypervascularization. Two months later, when the underlying disease had been controlled, repeat color Doppler ultrasonography revealed markedly decreased vascularity, although no change was noted on the B-mode examination. The color Doppler findings thus represent an early indicator of response to treatment.