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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Etiopathogenesis of the Malformations of the Genital Tract
Duncan PA, Shapiro LR, Stangel JJ, Klein RM, Addonizio JC. The MURCS association: Mullerian ductaplasia, renal aplasia, and cervicothoracic somite dysplasia. J Pediatr. 1979;95:399–402.
Clitoris
Williams PL, Warwick R, Dyson M, Bannister LH. Splanchnology. In: Williams PL, editor. Gray’s anatomy. Oxford: Churchill-Livingstone; 1989.
Schober JM, Meyer-Bahlburg HFL, Ransley PG. Self-assessment of genital anatomy, sexual sensitivity and function in women: implications for genitoplasty. BJU Int. 2004;94:589–94.
O’Connell HE, Eizenberg N, Rahman M, Cleeve J. The anatomy of the distal vagina: towards unity. J Sex Med. 2008;5(8):1883–91.
Phillip M, De Boer C, Pilpel D, Karplus M, Sofer S. Clitoral and penile sizes of full term newborns in two different ethnic groups. J Pediatr Endocrinol Metab JPEM. 1996;9(2):175–9.
Clitoromegaly
Kutlu HA, Akbiyik F. Clitoral length in female newborns: a new approach to the assessment of clitoromegaly. Turk J Med Sci. 2011;41(3):495–9.
Atilla S. Controversies on clitoroplasty. Ther Adv Urol. 2011;3(6):273–7.
Schmidta A, Langb U, Kiessa W. Epidermal cyst of the clitoris: a rare cause of clitoromegaly. Eur J Obstet Gynecol Reprod Biol. 1999;87(2):163–5.
Graya HJ, Rendic MH, Holmes M. Painful clitoromegaly caused by rare epithelioid hemangioma. Gynecol Oncol Case Rep. 2013;4:60–2.
Yüksel H, Odabaşi AR, Kafkas S. Clitoromegaly in type 2 neurofibromatosis: a case report and review of the literature. Eur J Gynaecol Oncol. 2003;24(5):447–51.
Copcu E, Aktas A, Sivrioglu N, Copcu O, Oztan Y. Idiopathic isolated clitoromegaly: a report of two cases. Reprod Health. 2004;1:1–6.
Chada R, Kothary SK, Tanwar US, Gupta S. Female pseudohermaphroditism associated with cloacal anomalies: faulty differentiation in caudal developmental field. J Pediatr Surg. 2001;36:9.
Dumont T, Black AY, Ahmet A, Fleming NA. Isolated transient neonatal clitoromegaly with hyperandrogenism of unknown etiology. J Pediatr Adolesc Gynecol. 2009;22:142–5.
Lattimer JK. Relocation and recession of the enlarged clitoris with preservation of the glans: an alternative to amputation. J Urol. 1961;86:113–6.
Young HH. Genital abnormalities, hermaphroditism and related adrenal disease. Baltimore: Williams & Wilkins; 1937. p. 103–5.
Clitoral Agenesis
Falk HC, Hyman AB. Congenital absence of the clitoris, a case report. Obstet Gynecol. 1971;38:269.
Clitoral Duplication
Hellenthal NJ, Kurzrock EA. Complete bladder duplication presenting as incontinence in an 11-year-old girl. Urology. 2008;70(6):1223–19.
Clitoral Cyst
Merlob P, Bahari C, Liban E, Reisner SH. Cysts of the female external genitalia in the newborn infant. Am J Obstet Gynecol. 1978;132:607–10.
Abudaia J, Habib Z, Ahmed S. Dermoid cyst: a rare cause of clitoromegaly. Pediatr Surg Int. 1999;15:521–2.
Lambert B. Epidermoid cyst of the clitoris: a case report. J Low Genit Tract Dis. 2011;15(2):161–2.
Accessory Phallic Urethra
Bagul AS, Sarathi V, Bokade CM. Urogenital sinus developmental anomaly with phallus and accessory phallic urethra presented as disorder of sex differentiation in female. J Neonatal Surg. 2014;3(1):15.
Reilly AW, Hinman F, Pickering DE, Crane JT. Phallic urethra in female pseudohermaphroditism. Am J Dis Child. 1958;95(1):9–12.
Karlin G, Brock W, Rich M, Peña A. Persistent cloaca and phallic urethra. J Urol. 1989;142(4):1056–9.
Clitoral Hood Anomalies
Cold CJ, Taylor TR. The prepuce. Br J Urol. 1999;83(1):34–44.
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-662-43680-6_8
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-662-43679-0
Online ISBN: 978-3-662-43680-6
eBook Packages: MedicineMedicine (R0)