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Female Genital Anomalies, Clitoris

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Rare Congenital Genitourinary Anomalies
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Abstract

Most of the female genital tract malformations affect the uterus, and therefore they are often referred to as uterine or Mullerian (paramesonephric) malformations; however, many of the anomalies that affect the Mullerian ducts could originate as a mesonephric (Wolffian) anomaly or in the female gubernaculum. Female genital tract malformations are frequent but are not always detected; therefore, their true prevalence in the general population is unknown because many are asymptomatic and not noticed.

The clitoris is a highly neurovascular erectile part of the external genitalia; embryologically, it is derived from undifferentiated phallus; the major difference between female external genitalia and male genitalia is that female genitalia are separate from the urethra and it consists of the paired corpora, vestibular bulbs, and the glans. Clitoromegaly is defined as a measure of the clitoral index (width × length in mm) more than 15 mm2 in the newborn. Clitoromegaly could be either congenital or acquired; the congenital forms are caused by hormonal disturbances or intersex states; usually, they are obvious at birth, but when the clitoromegaly develops later on in life, the underlying etiology should be explored, and acquired causes should also be considered.

Only few cases of clitoral agenesis had been reported without other genitourinary anomalies, and in two cases there was a manifestation of midline defect like absence of pubic hair or patulous urethra; in this anomaly, may be quite likely that there are more unreported cases.

Clitoral duplication should be differentiated from bifid clitoris; it usually develops in association with bladder exstrophy, with urethral duplication, or with epispadias. This disorder is rare, and the incidence approximates 1 in 480,000 females.

An overall incidence of clitoral cyst in infancy was reported to be of 0.6 %, but clitoral cysts without genital tract mutilation are rare, and only very few cases are reported in literature, but acquired cyst like inclusion cyst is common in areas with high prevalence of female circumcision.

It is not rare to see a dimple or false meatus in the female clitoris, but the presence of an accessory phallic urethra in the female patient is extremely rare; several cases with the urethra coursing partially or entirely through the phallus had been reported.

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Fahmy, M.A.B. (2015). Female Genital Anomalies, Clitoris. In: Rare Congenital Genitourinary Anomalies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43680-6_8

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  • DOI: https://doi.org/10.1007/978-3-662-43680-6_8

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