[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address Please contact the publisher to request reinstatement.
[Skip to Content Landing]
April 1958

Female Pseudohermaphroditism with Penile Urethra: Report of an Unusual Case of Congenital Adrenal Hyperplasia

AMA Am J Dis Child. 1958;95(4):410-412. doi:10.1001/archpedi.1958.02060050414010

The extent to which masculinization of the external genitalia occurs in the female pseudohermaphrodite with congenital adrenal hyperplasia is variable. The usual patient with this condition shows some clitoral enlargement with chordee, a urethra and vagina opening into a urogenital sinus which has its external aperture at the base of the clitoris, and some degree of corrugation and fusion of the labia majora simulating a scrotum. Several cases with the urethra coursing partially or entirely through the phallus have been reported. The most extreme degree of masculinization is represented by the development of the phallus into a penile structure with the urethra pursuing a normal course and opening at the tip of the glans and with fusion of the labia to resemble a scrotal structure. Patients with this configuration of the external genitalia resemble cryptorchid males with macrogenitosomia praecox due to congenital adrenal hyperplasia. Only three such cases previously have