SURGICAL CORRECTION OF NONHORMONAL CLITOROMEGALY IN NEUROFIBROMATOSIS TYPE 1

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Abstract

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. 

About the authors

D. A. Morozov

I.M. Sechenov First Moscow State University

Author for correspondence.
Email: damorozov@list.ru
Доктор медицинских наук, профессор, заведующий кафедрой детской хирургии и урологии-андрологии Russian Federation

N. Yu. Raigorodskaya

V. I. Razumovsky Saratov State Medical University

Email: nraygorodskaya@gmail.com
Доктор медицинских наук, доцент кафедры пропедевтики детских болезней, детской эндокринологии и диабетологии Russian Federation

E. S. Pimenova

I.M. Sechenov First Moscow State University

Email: fake@neicon.ru
Кандидат медицинских наук, доцент кафедры детской хирургии и урологии- андрологии Russian Federation

N. V. Bolotova

V. I. Razumovsky Saratov State Medical University

Email: fake@neicon.ru
Доктор медицинских наук, профессор, заведующая кафедрой пропедевтики детских болезней, детской эндокринологии и диабетологии Russian Federation

E. K. Airyan

I.M. Sechenov First Moscow State University

Email: fake@neicon.ru
детский хирург, аспирант кафедры детской хирургии и урологии-андрологии Russian Federation

References

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Copyright (c) 2016 Morozov D.A., Raigorodskaya N.Y., Pimenova E.S., Bolotova N.V., Airyan E.K.

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This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

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