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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2016; 26(03): 332-336
DOI: 10.4103/0971-3026.190423
Neuroimaging

Magnetic resonance neurographic confirmation of extensive Plexiform neurofibroma in neurofibromatosis-1 presenting as ambiguous genitalia

Ishan Kumar
Department of Radiodiagnosis, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
,
Ashish Verma
Department of Radiodiagnosis, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
,
Ritu Ojha
Department of Radiodiagnosis, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
,
Priyanka Aggarwal
Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
,
Ram C Shukla
Department of Radiodiagnosis, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
,
Arvind Srivastava
Department of Radiodiagnosis, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India
› Author Affiliations Financial support and sponsorship Nil.
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Abstract

Genitourinary involvement of neurofibromatosis is uncommon and genital neurofibromatosis is even rarer. Involvement of clitoris by neurofibroma can lead to clitoromegaly masquerading as a male penis. We report such a case of ambiguous genitalia in a 7-year-old female child presenting with clitoromegaly since birth, in which magnetic resonance imaging (MRI) revealed the presence of extensive neurofibromatosis in the clitoris and lumbosacral regions. We emphasize the central role of MRI in evaluation of hormonal and non-hormonal causes of ambiguous genitalia. We further discuss the merits of including MR neurography in the imaging protocol for comprehensive delineation of neurofibromatosis.

Keywords

Clitoromegaly - genital neurofibromatosis - MR neurography


Publication History

Article published online:
30 July 2021

© 2016. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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