SURGICAL CORRECTION OF NONHORMONAL CLITOROMEGALY IN NEUROFIBROMATOSIS TYPE 1
D. A. Morozov , N. Yu. Raigorodskaya , E. S. Pimenova , N. V. Bolotova , E. K. Airyan
Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care ›› 2016, Vol. 6 ›› Issue (4) : 92 -96.
SURGICAL CORRECTION OF NONHORMONAL CLITOROMEGALY IN NEUROFIBROMATOSIS TYPE 1
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.
neurofibromatosis type 1 / clitoromegaly / surgical treatment
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Morozov D.A., Raigorodskaya N.Y., Pimenova E.S., Bolotova N.V., Airyan E.K.
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