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May 1961

Adrenogenital Syndrome with Phallic Urethra

Author Affiliations

From the Departments of Pediatrics and Pathology, Long Island Jewish Hospital.

Am J Dis Child. 1961;101(5):645-649. doi:10.1001/archpedi.1961.04020060103015

Many case reports and articles have been published in recent years describing the adrenogenital syndrome in detail. The clinical picture and physiochemical explanation have become well known. There is, however, one minor anatomical variation which we believe has not been sufficiently stressed. This variation is one in which the urethra opens at the tip of a hypertrophied clitoris, giving the appearance of a normally formed penis with the labia completely fused. Admittedly, this is a rare occurrence, but can be misleading when it does exist if the possibility is ignored. We recently had occasion to see such a patient, and, although the adrenogenital syndrome was considered in the diagnosis, it was discarded because it was thought that the urethra in this condition always opened at the base of the enlarged clitoris or more posteriorly in the perineum.

Report of Case  The patient was born by uneventful delivery to a 25-year-old