SURGICAL CORRECTION OF NONHORMONAL CLITOROMEGALY IN NEUROFIBROMATOSIS TYPE 1

D. A. Morozov; Морозов Дмитрий Анатольевич; N. Yu. Raigorodskaya; Райгородская Надежда Юрьевна; E. S. Pimenova; Пименова Евгения Сергеевна; N. V. Bolotova; Болотова Нина Викторовна; E. K. Airyan; Айрян Эдуард Каренович

doi:10.17816/psaic298

SURGICAL CORRECTION OF NONHORMONAL CLITOROMEGALY IN NEUROFIBROMATOSIS TYPE 1

作者: Morozov D.A.¹, Raigorodskaya N.Y.², Pimenova E.S.¹, Bolotova N.V.², Airyan E.K.¹
隶属关系:
1. I.M. Sechenov First Moscow State University
2. V. I. Razumovsky Saratov State Medical University
期: 卷 6, 编号 4 (2016)
页面: 92-96
栏目: Case reports
##submission.dateSubmitted##: 11.06.2017
##submission.dateAccepted##: 11.06.2017
##submission.datePublished##: 10.12.2016
URL: https://rps-journal.ru/jour/article/view/298
DOI: https://doi.org/10.17816/psaic298
ID: 298

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Neurofibromatosis 1 type (NF1, Recklinghausen disease) is caused by mutations in the NF1 gene (localized to chromosome 17q) and is an autosomal dominant genetic syndrome. The disease occurs in 1 of every 2500–3000 births. In neurobibromatosis the urogenital tract is affected rarely. The article presents the clinical case of examination, diagnosis verification and surgery of a 12‑year-old girl complaining of an increased clitoris with the aggravated genealogical anamnesis (her uncle suffers from neurofibromatosis type 1). The child examined by an endocrinologist, removed diagnosis violation forming the floor. Spend plastic feminized external genitalia with the removal of nodules neurofibromatosis imitating virilization of the external genitalia, resection of the corpora cavernosa, labiaplasty of mucocutaneous grafts. Cosmetic outcome immediately after the operation is regarded as satisfactory.

关键词

neurofibromatosis type 1, clitoromegaly, surgical treatment

参考

Ferner R. E., Huson S. M., Thomas N., Moss C., Willshaw H., Evans D. G., Upadhyaya M., Towers R., Gleeson M., Steiger C., Kirby A. Guidelines for the diagnosis and management of individuals with neurofibromatosis 1 // J Med Genet. 2007;44 (2):81–8.
Sutphen R., Galán-Goméz E., Kousseff B. G. Clitoromegaly in neurofibromatosis //Am J Med Genet. 1995.55 (3):325–30.
Pascual-Castroviejo I., Lopez-Pereira P., Savasta S. Neurofibromatosis type 1 with external genitalia involvement: Presentation of 4 patients // J of Pediatric Surgery. 2008.43:1998–2003.
Болотова Н. В., Морозов Д. А., Райгородская Н. Ю., Жарков Д. А., Григорьева М. М., Цмокалюк Е. Н. Критерии кли- нической диагностики и выбор лечебной тактики у пациентов с неопределенностью пола // Саратовский научно- медицинский журнал. 2010. Т.6. №1. С. 178–182.
Bolotov N.V., Morozov D.A., Raigorodskaya N.Y., Zharkov D.A., Grigoriev M.M., Tsmokalyuk E.N. Criterias for clinical diagnosis and choice of treatment strategy in patients with gender uncertainty // Saratov Journal of Medical Science. 2010 V.6. №1. Pp 178–182.
McDonnell C. Neurofibromatosis of bladder and prostate // Am J Surg 1936; 34: 90–3.
Thomas W. J., Bevan H. E., Hooper D. G., Downey E. J. Malignant schwannoma of the clitoris in a 1-year-old child // Cancer. 1989 Jun 1;63 (11):2216–9.

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SURGICAL CORRECTION OF NONHORMONAL CLITOROMEGALY IN NEUROFIBROMATOSIS TYPE 1

全文:

详细

关键词

作者简介

D. Morozov

N. Raigorodskaya

E. Pimenova

N. Bolotova

E. Airyan

参考

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