Brain Herniation in Neurofibromatosis with Dysplasia of Occipital Bone and Posterior Skull Base

A 22-year-old female, a known case of neurofibromatosis 1 (NF1), presented with a congenital swelling in the left occipital region. She had developed recent onset dysphagia and localized occipital headache. Neuroradiology revealed a left occipital meningoencephalocele and a left parapharyngeal menin...

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Bibliographic Details
Main Authors: Vithal Rangarajan, Amit Mahore, Manoj Patil, Prashant Sathe, Amol Kaswa, Sandeep Gore, Pralhad Dharurkar, Juhi Kawale
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2015/816079
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Summary:A 22-year-old female, a known case of neurofibromatosis 1 (NF1), presented with a congenital swelling in the left occipital region. She had developed recent onset dysphagia and localized occipital headache. Neuroradiology revealed a left occipital meningoencephalocele and a left parapharyngeal meningocele. This was associated with ventriculomegaly. She was advised on cranioplasty along with duraplasty which she denied. She agreed to a lumbar-peritoneal shunt. She described a dramatic improvement in her symptoms following the lumbar-peritoneal shunt. Occipital dysplasias, though uncommon, have been reported in the literature. We review this case and its management and discuss relevant literature on occipital dysplasias in NF1.
ISSN:2090-6668
2090-6676