Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I

Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I

Neurofibromatosis type 1 (NF1) is a clinically and genetically distinct disease involving both neuroectodermal and mesenchymal derivatives. Orofacial manifestations in NF1 have been documented before but occurrence of multifocal intraosseous (IO) and extraosseous (EO) neurofibromas is rare. The pres...

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Main Authors: Rachna Rath, Sheetal Kaur, Shadab Ali Baig, Punyashlok Pati, Sonalisa Sahoo
Format: Article
Language:English
Published: Hindawi Limited 2016-01-01
Series:Case Reports in Radiology
Online Access:http://dx.doi.org/10.1155/2016/3980270
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spelling doaj-45b5ffcfc0104b3d8659b4c2b86efd5f2020-11-24T22:22:33ZengHindawi LimitedCase Reports in Radiology2090-68622090-68702016-01-01201610.1155/2016/39802703980270Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type IRachna Rath0Sheetal Kaur1Shadab Ali Baig2Punyashlok Pati3Sonalisa Sahoo4Department of Oral & Maxillofacial Pathology, SCB Government Dental College & Hospital, Cuttack 753007, IndiaDepartment of Radio Diagnosis, Acharya Harihar Regional Cancer Centre, Cuttack 753007, IndiaDepartment of Oral & Maxillofacial Surgery, SCB Government Dental College & Hospital, Cuttack 753007, IndiaDepartment of Oral & Maxillofacial Pathology, SCB Government Dental College & Hospital, Cuttack 753007, IndiaDepartment of Oral & Maxillofacial Pathology, SCB Government Dental College & Hospital, Cuttack 753007, IndiaNeurofibromatosis type 1 (NF1) is a clinically and genetically distinct disease involving both neuroectodermal and mesenchymal derivatives. Orofacial manifestations in NF1 have been documented before but occurrence of multifocal intraosseous (IO) and extraosseous (EO) neurofibromas is rare. The present case highlights the importance of imaging findings in the diagnosis and management of multifocal jaw, infratemporal, and parotid neurofibromas with muscular hypoplasia in an eight-year-old girl with NF1. Apart from orthopantomograms (OPG), three-dimensional computed tomography (3D CT) and cross-sectional reformations were valuable in delineating the extent of the lytic lesion and identifying additional bony deformities of the mandible. Magnetic resonance imaging (MRI) helped to identify the solid nature of the lesion and true extent of the soft tissue mass.http://dx.doi.org/10.1155/2016/3980270
collection DOAJ
language English
format Article
sources DOAJ
author Rachna Rath
Sheetal Kaur
Shadab Ali Baig
Punyashlok Pati
Sonalisa Sahoo
spellingShingle Rachna Rath
Sheetal Kaur
Shadab Ali Baig
Punyashlok Pati
Sonalisa Sahoo
Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I
Case Reports in Radiology
author_facet Rachna Rath
Sheetal Kaur
Shadab Ali Baig
Punyashlok Pati
Sonalisa Sahoo
author_sort Rachna Rath
title Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I
title_short Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I
title_full Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I
title_fullStr Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I
title_full_unstemmed Multifocal Head and Neck Neurofibromas with Osseous Abnormalities and Muscular Hypoplasia in a Child with Neurofibromatosis: Type I
title_sort multifocal head and neck neurofibromas with osseous abnormalities and muscular hypoplasia in a child with neurofibromatosis: type i
publisher Hindawi Limited
series Case Reports in Radiology
issn 2090-6862
2090-6870
publishDate 2016-01-01
description Neurofibromatosis type 1 (NF1) is a clinically and genetically distinct disease involving both neuroectodermal and mesenchymal derivatives. Orofacial manifestations in NF1 have been documented before but occurrence of multifocal intraosseous (IO) and extraosseous (EO) neurofibromas is rare. The present case highlights the importance of imaging findings in the diagnosis and management of multifocal jaw, infratemporal, and parotid neurofibromas with muscular hypoplasia in an eight-year-old girl with NF1. Apart from orthopantomograms (OPG), three-dimensional computed tomography (3D CT) and cross-sectional reformations were valuable in delineating the extent of the lytic lesion and identifying additional bony deformities of the mandible. Magnetic resonance imaging (MRI) helped to identify the solid nature of the lesion and true extent of the soft tissue mass.
url http://dx.doi.org/10.1155/2016/3980270
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AT shadabalibaig multifocalheadandneckneurofibromaswithosseousabnormalitiesandmuscularhypoplasiainachildwithneurofibromatosistypei
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