Clitoromegaly: principles of diagnostics and treatment
https://doi.org/10.17749/2313-7347/ob.gyn.rep.2023.400
Abstract
The literature review is dedicated to the topical subject in current endocrinology and gynecology – clitoromegaly. At present, clitoromegaly is considered as a symptom of an endocrinological disease associated with impaired testosterone or androgen production. However, in most cases, the cause of the disease cannot be determined. A condition described in the clitoromegaly classification as an "idiopathic clitoromegaly" holds a leading place. Despite an opportunity to perform a successful clitoroplasty in childhood, adult patients with clitoromegaly quite often seek specialists` help. Such girls and women often suffer from anxietydepressive disorders related to social maladjustment associated due to sexological and psychological problems, which requires a sexologist or psychotherapist` supervision and treatment. Here, we provide the data on genetic syndromes accompanied by clitoromegaly. Differential diagnostics algorithms for neonates, young and adult women are proposed.
About the Authors
N. V. Ivanov
Mechnikov North-Western State Medical University
Russian Federation
Russian Federation
Nikita V. Ivanov – MD, PhD, Associate Professor, Academician Baranov Department of Endocrinology
Author ID: 647940
41 Kirochnaya Str., Saint Petersburg 199034
I. P. Serebryakova
Mechnikov North-Western State Medical University
Russian Federation
Russian Federation
Inna P. Serebryakova – MD, PhD, Associate Professor, Academician Baranov Department of Endocrinology
Author ID: 433944
41 Kirochnaya Str., Saint Petersburg 199034
N. V. Khudyakova
Saint Petersburg State University
Russian Federation
Russian Federation
Natalia V. Khudyakova – MD, PhD, Associate Professor, Department of Internal Diseases
Author ID: 81693
7/9 Universitetskaya Embankment, Saint Petersburg 199034
A. I. Fedorova
Mechnikov North-Western State Medical University
Russian Federation
Russian Federation
Anna I. Fedorova – MD, Dr Sci Med, Professor, Department of Psychotherapy, Medical Psychology and Sexology
Author ID: 460196
41 Kirochnaya Str., Saint Petersburg 199034
References
1. Mikhaylichenko V.V., Fesenko V.N., Novikov A.I., Murtazaaliev O.A. Klitoroplastika in congenital adrenal hyperplasia. [Klitoroplastika pri vrozhdennoj giperplazii kory nadpochechnikov]. Andrologiya i genital'naya hirurgiya. 2013;14(2):31–4. (In Russ.). https://doi.org/10.17650/2070-9781-2013-2-31-341.
2. Morozov D.A., Raigorodskaya N.Yu., Pimenova E.S. et al. Surgical correction of nonhormonal clitoromegaly in neurofibromatosis type I. [Hirurgicheskaya korrekciya negormonal'noj klitoromegalii pri nejrofibromatoze I tipa]. Rossijskij vestnik detskoj hirurgii, anesteziologii i reanimatologii. 2016;6(4):92–6. (In Russ.). https://doi.org/10.17816/psaic298.
3. Morozov D.A., Airyan E.K., Tsmokalyuk E.N. et al. Clitoroplasty with morphology of the glans sensitive zones in children with disorders of sex development. [Klitoroplastika s morfologiej sensitivnyh zon golovki u detej s narusheniem formirovaniya pola]. Novosti hirurgii. 2019;27(5):541–52. https://doi.org/10.18484/2305-0047.2019.5.541.
4. Airyan E.K., Morozov D.A., Tsmokalyuk E.N. Morphology of sensitive clitoris zones during virilization of external genitalia in patients with disorders of sex development. [Morfologiya sensitivnyh zon klitora pri virilizacii naruzhnyh genitalij u pacientov s narusheniem formirovaniya pola]. Detskaya hirurgiya. 2017;21(2):64–6. (In Russ.). https://doi.org/10.18821/1560-9510-2017-21-2-64-66.
5. Adamyan L.V., Nikolaev V.V., Bizhanova D.A. et al. Feminizig genitoplasty in congenital adrenal hyperplasia: an overview. [Feminiziruyushchaya plastika pri vrozhdennoj disfunkcii kory nadpochechnikov (obzor literatury)]. Problemy reprodukcii. 2021;27(6):56–65. (In Russ.). https://doi.org/10.17116/repro20212706156.
6. O’Connell H., Sanjeevan K., Hutson J. Anatomy of the clitoris. J Urol. 2005;174(4 Pt 1):1189–95. https://doi.org/10.1097/01.ju.0000173639.38898.cd.
7. Andronik D.I., Bashnina E.B., Burtsev E.A. et al. Lilith principle. On the issue of sex formation in humans: a practical guide for doctors. Eds. N.V. Ivanova, E.B. Tower. [Princip Lilit. K voprosu formirovaniya pola u cheloveka: prakticheskoe rukovodstvo dlya vrachej. Pod red. N.V. Ivanova, E.B. Bashninoj]. SPb.: POLITEH-PRESS, 2021. 382 p. (In Russ.).
8. Copcu E., Aktas A., Sivrioglu N. et al. Idiopathic isolated clitoromegaly: a report of two cases. Reprod Health. 2004;1(1):4. https://doi.org/10.1186/1742-4755-1-4.
9. Hughes I., Houk C., Ahmed S., Lee P.; LWPES/ESPE Consensus Group. Consensus statement on management of intersex disorders. Arch Dis Child. 2006;91:554–63. https://doi.org/10.1136/adc.2006.098319.
10. Serebryakova I.P., Velikanova L.I., Vorokhobina N.V. et al. The specific features of adrenal steroidogenesis in patients with congenital adrenal cortical dysfunction due to 21-hydroxylase deficiency. [Osobennosti adrenalovogo steroidogeneza u bol'nyh s vrozhdennoj disfunkciej kory nadpochechnikov vsledstvie defekta 21-gidroksilazy]. Problemy endokrinologii. 2005;51(4):22–6. (In Russ.). https://doi.org/10.14341/probl200551422-26.
11. Bachelot A., Grouthier V., Courtillot C. et al. MANAGEMENT OF ENDOCRINE DISEASE: Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: update in management of adult patients and prenatal treatment. Eur J Endocrinol. 2017;176(4):R167–R181. https://doi.org/10.1530/EJE-16-0888.
12. Siddiqui S., Soomro N., Ganatra A. Classic congenital adrenal hyperplasia: a delayed presentation. Pak J Med Sci. 2013;29(1):220–3. https://doi.org/10.12669/pjms.291.2830.
13. Bergendi E., Plöchl E., Vlasak I., et al. A Turner-like phenotype in a girl with an isodicentric fluorescent Y chromosome mosaicism. Klin Padiatr. 1997;209(3):133–6. https://doi.org/10.1055/s-2008-1043943.
14. Wilkins L., Jones H., Holman G., Stempfel R. Masculinization of the female fetus associated with administration of oral and intramuscular progestins during gestation: non-adrenal female pseudohermaphrodism. J Clin Endocrinol Metab. 1958;18(6):559–85. https://doi.org/10.1210/jcem-18-6-559.
15. Voorhess M. Masculinization of the female fetus associated with norethindrone-mestranol therapy during pregnancy. J Pediatr. 1967;71(1):128–31. https://doi.org/10.1016/s0022-3476(67)80244-0.
16. Parappil H., Rahman S., Al Khalaf F. Virilization in a female neonate due to increased maternal androgens. Qatar Med J. 2009;18(2):74–6. https://doi. org/10.1016/j.fertnstert.2005.02.029.
17. Sandrini R., Ribeiro R., Delacerda L. Childhood adrenocortical tumors. J Clin Endocrinol Metab. 1997;82(7):2027–31. https://doi.org/10.1210/jcem.82.7.4057.
18. Latronico A., Pinto E., Domenice S. et al. An inherited mutation outside the highly DNA-binding domain of the P53 tumor suppressor protein in children and adults with sporadic adrenocortical tumors. J Clin Endocrinol Metab. 2001;86(10):4970–3. https://doi.org/10.1210/jcem.86.10.7957.
19. Lim Y., Lee S., Shin J. et al. Virilizing adrenocortical oncocytoma in a child: a case report. J Korean Med Sci. 2010;25(7):1077–9. https://doi.org/10.3346/jkms.2010.25.7.1077.
20. Schulin-Zeuthen P., Yamamoto C., Pires N. et al. Precocious puberty in a six years old girl caused by a juvenile granulosa cell ovaric tumor. Report of one case. Rev Med Chil. 2003;131(1):71–6.
21. Park S.M., Kim Y.N., Woo Y.J. et al. A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl. Korean J Pediatr. 2011;54(5):224–7. https://doi.org/10.3345/kjp.2011.54.5.224.
22. Azziz R., Carmina E., Dewailly D. et al. The androgen excess and PCOS society criteria for the polycystic ovary syndrome: the complete task force report. Fertil Steril. 2009;91(2):456–88. https://doi.org/10.1016/j.fertnstert.2008.06.035.
23. Köşüş A., Kamalak Z., Köşüş N. et al. Clitoral and labial sizes in women with PCOS. J Obstet Gynaecol. 2016;36(1):97–101. https://doi.org/10.3109/01443615.2015.1036399.
24. Slavotinek A., Tifft C. Fraser syndrome and cryptophthalmos: review of the diagnostic criteria and evidence for phenotypic modules in complex malformation syndromes. J Med Genet. 2002;39(98):623–33. https://doi.org/10.1136/jmg.39.9.623.
25. Ramalingam K., Kaliyamurthy S., Govindarajan M., Swathi S. Seckel syndrome: a report of a case. J Indian Soc Pedod Prev Dent. 2012;30(3):258–61. https://doi.org/10.4103/0970-4388.105021.
26. Cohen M., Kreiborg S. Visceral anomalies in the Apert syndrome. Am J Med Genet. 1993;45(6):758–60. https://doi.org/10.1002/ajmg.1320450618.
27. Wong C., Cuda S., Kirsch A. A review of the urologic manifestations of Beckwith-Wiedemann syndrome. J Pediatr Urol. 2011;7(2):140–4. https://doi.org/10.1016/j.jpurol.2010.06.003.
28. Galli-Tsinopoulou A., Emmanouilidou E., Karagianni P. et al. A female infant with Silver Russell Syndrome, mesocardia and enlargement of the clitoris. Hormones (Athens). 2008;7(1):77–81. https://doi.org/10.14310/horm.2002.1111040.
29. Khandpur S., Kumar A., Khadgawat R. Congenital generalized lipodystrophy of Berardinelli-Seip type: a rare case. Indian J Dermatol Venereol Leprol. 2011;77(3):402. https://doi.org/10.4103/0378-6323.79740.
30. Cost N.G., Sanchez F.S., Weinberg A.G. et al. Neurofibromatosis presenting as painless clitoromegaly. Urol J. 2009;6(3):220–2. https://doi.org/10.22037/uj.v6i3.409.
31. Karabouta Z., Rousso I., Athanassiadou-Piperopoulou F. Clitoromegaly as first presentation of a neurocutaneous syndrome in a 3-year-old girl. Clin Case Rep. 2015;3(9):767–8. https://doi.org/10.1002/ccr3.320.
32. Nayyar S., Liaqat N., Sultan N., Dar S.H. Cavernous haemangioma mimicking as clitoral hypertrophy. Afr J Paediatr Surg. 2014;11(1):65–6. https://doi.org/10.4103/0189-6725.129238.
33. Rouzi A.A., Sindi O., Radhan B., Ba’aqeel H. Epidermal clitoral inclusion cyst after type I female genital mutilation. Am J Obstet Gynecol. 2001;185(3):569–71. https://doi.org/10.1067/mob.2001.117660.
34. Schober M.S., Hendrickson B.W., Alpert S.A. Spontaneous clitoral hood epidermal inclusion cyst mimicking clitoromegaly in a pediatric patient. Urology. 2014;84(1):207–8. https://doi.org/10.1016/j.urology.2014.03.008.
35. Gomes A.L., Onofre L.S., de Souza Leão J.Q. et al. Clitoral anomalies not associated with disorders of sex development. J Ped Surg Case Reports. 2013;1(11):403–5. https://doi.org/10.1016/j.epsc.2013.11.003.
36. Llaneza P., Fresno F., Ferrer J. Schwannoma of the clitoris. Acta Obstet Gynecol Scand. 2002;81(5):471–2. https://doi.org/10.1034/j.1600-0412.2002.810517.x.
37. Maor-Sagie E., Arbell D., Prus D. et al. Pilonidal cyst involving the clitoris in an 8-year-old girl: a case report and literature review. J Pediatr Surg. 2010;45(11):e27–9. https://doi.org/10.1016/j.jpedsurg.2010.07.022.
38. Kawamura M., Matsumoto F., Matsui F. et al. Aggressive angiomyxoma of the vulva mimicking clitoromegaly in a young child. Urology. 2017;101:142–4. https://doi.org/10.1016/j.urology.2016.10.034.
39. Kujur A., Joseph V., Chandra P. Nerve sparing clitoroplasty in a rare case of idiopathic clitoromegaly. Indian J Plast Surg. 2016;49(1):86–90. https://doi.org/10.4103/0970-0358.182241.
40. Prader A. Genital findings in the female pseudo-hermaphroditism of the congenital adrenogenital syndrome; morphology, frequency, development and heredity of the different genital forms. Helv Paediatr Acta.1954;9(3):231–48. (In German).
41. Puppo V. Anatomy of the clitoris: revision and clarifications about the anatomical terms for the clitoris proposed (without scientific bases) by Helen O’Connell, Emmanuele Jannini, and Odile Buisson. ISRN Obstet Gynecol. 2011;2011:261464. https://doi.org/10.5402/2011/261464.
42. Kessler S.J. Lessons from the Intersexed. New Brunswick: Rutgers University Press, 1998. 208 р.
43. Oberfield S.E., Mondok A., Shahrivar F. et al. Clitoral size in full-term infants. Am J Perinatol. 1989;6(4):453–4. https://doi.org/10.1055/s-2007-999638.
44. Sane K., Pescovitz O.H. The clitoral index: a determination of clitoral size in normal girls and in girls with abnormal sexual development. J Pediatr. 1992;120(2 Pt 1):264–6. https://doi.org/10.1016/s0022-3476(05)80439-1.
45. Ivanov N.V., Petrunichev A.Yu. Genetic syndromes in andrology and gynecology. [Geneticheskie sindromy v andrologii i ginekologii]. SPb.: Izd-vo Politekhnicheskogo universiteta, 2010. 316 p. (In Russ.).
46. Akramov N.R., Zakirov A.K. Sexual development disorder in girls: evolution of views on surgical treatment. [Narushenie formirovaniya pola u devochek: evolyuciya vzglyadov na hirurgicheskoe lechenie]. Reproduktivnoe zdorov'e detej i podrostkov. 2012;(5):50–63. (In Russ.).
47. Krysiak R., Drosdzol-Cop A., Skrzypulec-Plinta V., Okopien B. Sexual function and depressive symptoms in young women with nonclassic congenital adrenal hyperplasia. J Sex Med. 2016;13(1):34–9. https://doi.org/10.1016/j.jsxm.2015.11.002.
48. Andreeva E.N., Sheremet'eva E.V., Grigorian O.R., Dedov I.I. Psychological characteristics of patients with different types of pcos. [Psihologicheskie osobennosti bol'nyh pri razlichnyh fenotipah sindroma polikistoznyh yaichnikov]. Problemy reprodukcii. 2013;(3): 25–9. (In Russ.).
49. Nordenstrom A., Frisen L., Falhammar H. et al. Sexual function and surgical outcome in women with congenital adrenal hyperplasia Due to CYP21A2 deficiency: clinical perspective and the patients’ perception. J Clin Endocrinol Metab. 2010;95(8):3633–40. https://doi.org/10.1210/jc.2009-2639.
50. Kanhere M., Fuqua J., Rink R. et al. Psychosexual development and quality of life outcomes in females with congenital adrenal hyperplasia. Int J Pediatr Endocrinol. 2015;2015:21. https://doi.org/10.1186/s13633-015-0017-z.
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For citations:
Ivanov N.V., Serebryakova I.P., Khudyakova N.V., Fedorova A.I. Clitoromegaly: principles of diagnostics and treatment. Obstetrics, Gynecology and Reproduction. 2023;17(4):462-475. (In Russ.) https://doi.org/10.17749/2313-7347/ob.gyn.rep.2023.400
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