Clitoral hypertrophy

from Wikipedia, the free encyclopedia
Classification according to ICD-10
Q52.6 Malformations of the clitoris
ICD-10 online (WHO version 2019)
Clitoral hypertrophy in a 22 year old woman

A clitoromegaly even Klitoromegalie or Megaloklitoris called, is the medical term for an anatomically unusually large penis-like clitoris .

etiology

Clitoral hypertrophy is usually a congenital malformation . In addition, clitoromegaly can also be acquired later in life, which is less common in comparison to a congenital malformation.

Aetiologically , the malformations can be divided into four classes:

  • hormonally related
  • non-hormonal
  • Pseudo-clitoromegaly
  • idiopathic clitoromegaly (with no known cause)

Hormonal causes

Hormonally induced clitoral hypertrophy can be broken down into four possible causes:

Endocrinopathies

The most common cause of clitoromegaly is the female pseudohermaphroditism due to a congenital adrenal hyperplasia ( congenital adrenal hyperplasia (CAH)) or a congenital adrenal hyperplasia , which by an enzyme -Defekt the steroid - biosynthesis significantly interfere.

In more than 95% of cases, there is a defect in the enzyme CYP21 ( cytochrome P450c 21; 21- hydroxylase ). CPY21 catalyzes the conversion of 17-α-hydroxyprogesterone (17-OHP) to cortisol and of progesterone to aldosterone . Due to the reduced 17-OHP breakdown, the androgens androstenedione and testosterone are increasingly formed. This causes the virilization (masculinization) of the female patient.

Less often, a defect in the enzyme CYP11B1 (cytochrome P450c11, 11-β-hydroxylase) is the starting point for clitoral hypertrophy. CYP11B1 is also produced in the adrenal cortex . It usually catalyzes the conversion of 11-deoxycortisol to cortisol and of 11-deoxycorticosterone (DOC) to corticosterone . A malfunction of this enzyme also leads to increased androgen production and leads to intrauterine virilization in girls .

If the hormonal disorders occur up to the 14th week of pregnancy , pronounced masculinization of the external genitalia can occur. In extreme cases, this leads to the transformation of the large labia (labia) into the scrotum (scrotum) and the closure of the vagina or the urogenital canal ( urogenital sinus ). In the case of hormone disorders in the advanced stage of pregnancy, virilization is essentially limited to the symptom of clitoral hypertrophy.

Masculinizing tumors

Masculinizing tumors include:

Exogenous androgens

The steroid danazol has been identified as an exogenous androgen capable of causing clitoral hypertrophy . In a retrospective study with 129 women who were given the testosterone derivative danazol during their pregnancy , it was found that of the 94 live births, 34 normal and 23 virilized girls were born. The masculinization manifested itself among other things in the symptom of a clitoral hypertrophy.

A case is reported in the literature in which multiple blood transfusions of a man's blood to an infant apparently induced clitoral hypertrophy.

Non-hormonal causes

Neurofibromatosis type 1 and - although much less frequently - neurofibromatosis type 2 have been described as non-hormonal causes of clitoral hypertrophy . These rare hereditary diseases develop a benign tumor. One possible symptom of the disease is clitoral hypertrophy.

Clitoral cysts can form from the epidermis (upper skin) and penetrate into the dermis (sclera) or even into the subcutaneous tissue (tissue below the skin layers). The cysts can manifest either before birth ( prenatal ) or after an injury ( trauma ).

A number of syndromes of non-hormonal origin can cause clitoral hypertrophy. For example, the congenital generalized lipodystrophy (CGL), a special form of lipodystrophy , the autosomal - recessive inherited. Clitoral hypertrophy has also been described in Turner syndrome , a chromosomal disease in which the affected women only have one functional X chromosome . The extremely rare Fraser syndrome , which is also inherited in an autosomal recessive manner, is also a possible cause of clitoral hypertrophy.

In the case of complete androgen resistance (CAIS = Complete Androgen Insensitivity Syndrome ), an x-linked recessive hereditary disease, the androgen receptor is defective . The clinical picture is very complex and can also include clitoral hypertrophy.

The nevus lipomatosus cutaneus superficialis (NLCS; Hoffmann- Zurhelle ) is a congenital benign malformation in which locally mature lipocytes accumulate in the skin like a birthmark. NLCS in the area of ​​the clitoris can cause the same to enlarge.

Pseudo-clitoromegaly

Pseudo-hypertrophy of the clitoris can be caused by masturbation in little girls . Cases are known in which the manipulation of the skin of the prepuce by repeated mechanical stress resulted in injuries which enlarged the prepuce and the inner labia minora , so that they simulated a clitoromegaly.

Idiopathic clitoromegaly

In idiopathic clitoromegaly, the causes of the malformation are not known.

therapy

The first surgical interventions were performed by HH Young in 1934. He reduced the size of the clitoris of a child who had congenital adrenal hyperplasia (CAH). Until the 1960s, clitoral hypertrophy was treated with an amputation ( clitoridectomy ).

In the meantime, several techniques of clitoral reduction plastic have been established. In addition to cosmetic indications, all of them have the goal of maintaining sexual excitability and sensation as fully as possible. This is made possible by preserving the neurovascular bundle of the clitoris.

See also

Individual evidence

  1. ↑ a b c d e E. Copcu, A. Aktas, N. Sivrioglu, O. Copcu, Y. Oztan: Idiopathic isolated clitoromegaly: A report of two cases. In: Reproductive health. Volume 1, number 1, October 2004, p. 4, ISSN  1742-4755 , doi : 10.1186 / 1742-4755-1-4 , PMC 523860 (free full text).
  2. ↑ J. Horejsi: Acquired clitoral enlargement. Diagnosis and treatment. In: Annals of the New York Academy of Sciences. (Ann NY Acad Sci ') No. 816, June 1997, pp. 369-372.
  3. ↑ M. Wonisch, R. Pokan: Doping and heart: What the specialist needs to know. In: Journal für Kardiologie - Austrian Journal of Cardiology. (J Kardiol) 2014, vol. 21, no. 5-6, p. 141, section side effects. ( Full text as PDF file ).
  4. ↑ Ulrich Abendroth: Doping - the never-ending horror story ( Memento of the original from July 25, 2014 in the Internet Archive ) Info: The @1@ 2Template: Webachiv / IABot / www.medical-tribune.de archive link was inserted automatically and not yet checked. Please check the original and archive link according to the instructions and then remove this notice. . On: medical-tribune.de from June 12, 2014.
  5. ^ JD Wilson: Formation of sexual phenotypes. In: The Endocrinologist. No. 13, 2003, pp. 205-207.
  6. ^ A b D. Michalk, E. Schönau: Differentialdiagnose Pädiatrie. Elsevier, Munich 2004, ISBN 3-437-22530-8 , p. 77.
  7. ↑ V. Pelzer, U. Holthusen: Vulvar changes in childhood and adolescence. In: Korasion. No. 1, 2001.
  8. ^ RL Rosenfield et al.: The diagnosis and management of intersex. Current problems in pediatrics (= Current problems in pediatrics. Vol. 10, No. 7). Yearbook Medical Publishers, Chicago 1980.
  9. ↑ TA Baramki et al: Bilateral hilus cell tumors of the ovary. In: Obstetrics and gynecology. (Obstet Gynecol) Vol. 62, No. 1, 1983, pp. 128-131, PMID 6856214 .
  10. ↑ L. Castelazo-Ayala et al .: Steroid production by gonadal tumors in male pseudo-hermaphroditism with isolated clitoromegaly. Biochemical studies in vivo. In: Steroidologia. No. 2, 1971, pp. 138-142, PMID 4336062 .
  11. ↑ L. Falsetti et al.: Adrenal androgen-secreting carcinoma in a fertile woman. In: Acta Eur Fertil No. 26, 1995, pp. 117-121, PMID 9098472
  12. ↑ R. Ichinohasama et al: Leydig cell tumor of the ovary associated with endometrial carcinoma and containing 17 beta-hydroxysteroid dehydrogenase. In: Int J Gynecol Pathol 8/1989, pp. 64-71. PMID 2707954
  13. ^ PJ Brunskill: The effects of fetal exposure to danazol. In: Br J Obstet Gynaecol 99/1992, pp. 212-215. PMID 1606119
  14. ↑ M. Akcam, A. Topaloglu: Extremely immature infant who developed clitoromegaly during the second month of her postnatal life probably due to frequent whole blood transfusion from an adult male. In: Pediatr Int 45/2003, pp. 347-348. PMID 12828595
  15. ↑ H. Yüksel et al .: Clitoromegaly in type 2 neurofibromatosis: a case report and review of the literature. In: Eur J Gynaecol Oncol 24/2003, pp. 447-451, PMID 14584669 .
  16. ↑ M. Haraoka et al .: Clitoral involvement by neurofibromatosis: a case report and review of the literature. In: The Journal of Urology . Jan. 1988, Vol. 139, No. 1, pp. 95-96, PMID 3121869 .
  17. ^ JAM Massie, J. Lacy: Plexiform Neurofibroma Presenting as Clitoromegaly: Case Report and Review of the Literature. In: Journal of Pediatric and Adolescent Gynecology. 21/2008, p. 97.
  18. ↑ D. Linck, MF Hayes: Clitoral cyst as a cause of ambiguous genitalia. In: Obstet Gynecol 99/2002, pp. 963-966. PMID 11975977
  19. ^ R. Kazlauskaite et al .: A case of congenital generalized lipodystrophy: metabolic effects of four dietary regimens. Lack of association of CGL with polymorphism in the lamin A / C genes. In: Clin Endocrinol (Oxf) 54/2001, pp. 412-414. PMID 11298098
  20. ^ NG Haddad, GH Vance et al .: Turner syndrome (45x) with clitoromegaly. In: The Journal of Urology. Oct. 2003, Vol. 170, No. 4, Part 1, pp. 1355-1356, PMID 14501769 .
  21. ↑ A. Chattopadhyay et al: Fraser syndrome. In: J Postgrad Med. 1993, Vol. 39, pp. 228-230, PMID 7996504 .
  22. ↑ A. Yalinkaya, M. Yayla: Laparoscopy-assisted transinguinal extracorporeal gonadectomy in six patients with androgen insensitivity syndrome. In: Fertility and Sterility . 2003, Vol. 80, pp. 429-433.
  23. ↑ R. Hattori include: Nevus lipomatosus cutaneous superficial of the clitoris. In: Dermatologic Surgery 29/2003, pp. 1071-1072. PMID 12974709
  24. ↑ January Hořejší: Acquired clitoral enlargement. Diagnosis and treatment. In: Annals of the New York Academy of Sciences. (Ann NY Acad Sci) Volume 816, June 17, 1997, pp. 369-372, doi: 10.1111 / j.1749-6632.1997.tb52163.x , PMID 9238289 .
  25. ^ Hugh Hampton Young: Genital abnormalities, hermaphroditism and related adrenal disease. Williams & Wilkins, Baltimore 1937, pp. 103-105.
  26. ↑ MF Bellinger: feminizing genitoplasty and vaginoplasty. In: Reconstructive and Plastic Surgery of the External Genitalia. WB Saunders, Philadelphia, 1999, ISBN 0-7216-6328-1 , p. 263.
  27. ↑ G. Bartsch et al.: Preservation of the vascular nerve bundle during clitoral reduction plastic. In: Current Urology. 1987, Volume 18, No. 2, pp. 96-98, doi: 10.1055 / s-2008-1061429 .

literature

Web links

Commons : Clitoral Hypertrophy  - Collection of images, videos and audio files