
Journal Basic Info
- Impact Factor: 5.253*
- H-Index: 6
- ISSN: 2474-1655
- DOI: 10.25107/2474-1655
Major Scope
- Pneumonia
- Oncology Cases
- Geriatric Medicine
- Family Medicine and Public Health
- Sleep Medicine and Disorders
- Nutrition and Food Science
- ENT
- Gastric Cancer
Abstract
Citation: Ann Clin Case Rep. 2021;6(1):1904.DOI: 10.25107/2474-1655.1904
A Child Presented with Clitoromegaly in Context with Neurofibromatosis Type 1
Zeindine Sirena and Al Ebrahem Asad
Department of Pediatrics, General Authority for Pediatric Hospital, Syria
*Correspondance to: Zeindine Sirena
PDF Full Text Case Report | Open Access
Abstract:
Neurofibromatosis type 1, also called Von Recklinghausen disease is a genetic disorder characterized by the development of multiple benign tumors affecting the skin and nervous system. It is a genetic disease with a prevalence of one case in 3,000 births. The cause of VRD is a genetic mutation. In half of all cases of NF1, the faulty gene is passed from a parent to their child. Clitoromegaly presenting in childhood can be congenital or acquired. Non-hormonal causes like NF1, epidermoid cysts, tumor syndromes have been reported. Clitoris enlargement is a frequently seen congenital malformation, but acquired clitoral enlargement is rarely detected. While most cases of VRD are the result of a genetic mutation in the neurofibromin gene, there are also acquired cases due to spontaneous mutations. The pathological alterations behind it begin in the embryonic period, prior to differentiation of the neural crest. We report a case of 6.5 year-old-female admitted for clitoromegaly and an apparent deformity of the genital organs. The girl has been diagnosed with clitoromegaly in the context of NF1 Disease. This patient was successfully treated by clitoroplasty. The biopsied clitoral tissue was sent to pathology and revealed plexiform neurofibroma.
Keywords:
NF1; Von Recklinghausen disease; Clitoromegaly; Clitoroplasty; Child
Cite the Article:
Sirena Z, Asad AE. A Child Presented with Clitoromegaly in Context with Neurofibromatosis Type 1. Ann Clin Case Rep. 2021; 6: 1904.