CONGENITAL BILATERAL ANORCHIA IN CHILDHOOD: A CLINICAL, ENDOCRINE AND THERAPEUTIC EVALUATION OF TWENTY-ONE CASES

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Abstract
An evaluation of 21 boys, including a discordant pair of identical twins, is presented in whom bilateral anorchia was found with a negative family history and without history of breech presentation or of postnatal testicular trauma, torsion or orchitis. The most likely cause is prenatal testicular torsion. The incidence of the condition in the hospital is 1 in 177 cases of cryptorchidism. Prepubertal growth was normal before treatment, and testosterone replacement therapy allowed a normal pubertal growth spurt and skeletal maturation. Although demonstrable basal urinary testosterone was found in the subjects with a postpubertal bone age, most patients tested showed no increase after stimulation with human chorionic gonadotropin. In the presence of a normal penis and scrotum, such findings, together with a high basal FSH [follicle stimulating hormone] and an increased response of plasma LH [luteinizing hormone] to LHRH [luteinizing hormone-releasing hormone], make surgical exploration unnecessary. In the rare patient who shows a positive but subnormal response of testosterone to HCG [human chorionic gonadotropin], Leydig cells are presumed to be present either ectopically or in rudimentary testes, and further surgical exploration is indicated.

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