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Case Rep Neurol Med. 2015;2015:816079. doi: 10.1155/2015/816079. Epub 2015 Oct 27.

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

Case reports in neurological medicine

Vithal Rangarajan, Amit Mahore, Manoj Patil, Prashant Sathe, Amol Kaswa, Sandeep Gore, Pralhad Dharurkar, Juhi Kawale

Affiliations

  1. Department of Neurosurgery, Seth G.S. Medical College and K.E.M. Hospital, Parel, Mumbai 400012, India.
  2. Department of Medicine, Seth G.S. Medical College and K.E.M. Hospital, Parel, Mumbai 400012, India.

PMID: 26600957 PMCID: PMC4639658 DOI: 10.1155/2015/816079

Abstract

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.

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