| Intracranial hypertension in 2 cases of craniometaphyseal dysplasia: differing surgical options. | |
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MedLine Citation:
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PMID: 21806345 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Craniometaphyseal dysplasia (CMD) is a very rare bone disorder characterized by abnormally developed metaphyses in long bones and sclerosis of the craniofacial bones. In this paper, the authors report 2 cases of children diagnosed with CMD and chronic intracranial hypertension with deletion in exon 9 of the human ANK gene (ANKH). After intracranial monitoring, a different treatment was chosen for each patient. One of the patients was treated using CSF shunting because ventriculomegaly in the absence of a Chiari malformation was also observed on cerebral MR imaging. The other patient underwent cranial expansion and decompressive craniotomy of the posterior fossa, because ventriculomegaly was excluded after cerebral MR imaging and cervical MR imaging showed a Chiari malformation Type I. The origin of intracranial hypertension in CMD is multifactorial. Previous intracranial pressure monitoring and a thorough understanding of neuroimaging studies are essential to achieve an accurate diagnosis and effective treatment. |
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Authors:
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Mónica Rivero-Garvía; F Javier Márquez-Rivas; Antonia García-Iglesias; Raquel Gutiérrez-González |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Neurosurgical focus Volume: 31 ISSN: 1092-0684 ISO Abbreviation: Neurosurg Focus Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-08-02 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 100896471 Medline TA: Neurosurg Focus Country: United States |
Other Details:
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Languages: eng Pagination: E6 Citation Subset: IM |
Affiliation:
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Pediatric Neurosurgical Unit, Department of Neurosurgery, Hospital Universitario Virgen del Rocío, Seville, Spain. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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