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Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis: A Review

Chiari 1 malformation and hydrocephalus are frequent findings in multi-suture and syndromic craniosynostosis patients. In this article, we review the pathogenesis, clinical significance, and management options for these conditions with comments from our own experience. The role of premature fusion o...

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Autores principales: Vankipuram, Siddharth, Ellenbogen, Jonathan, Sinha, Ajay Kumar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9648655/
https://www.ncbi.nlm.nih.gov/pubmed/36388008
http://dx.doi.org/10.4103/jpn.JPN_49_22
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author Vankipuram, Siddharth
Ellenbogen, Jonathan
Sinha, Ajay Kumar
author_facet Vankipuram, Siddharth
Ellenbogen, Jonathan
Sinha, Ajay Kumar
author_sort Vankipuram, Siddharth
collection PubMed
description Chiari 1 malformation and hydrocephalus are frequent findings in multi-suture and syndromic craniosynostosis patients. In this article, we review the pathogenesis, clinical significance, and management options for these conditions with comments from our own experience. The role of premature fusion of skull base sutures leading to a crowded posterior fossa and venous outflow obstruction resulting in impaired cerebrospinal fluid (CSF) absorption is highlighted. Management options are unique in this group and we advocate early (prior to 6 months of age) posterior vault expansion by distraction osteogenesis (DO) in the management of Chiari 1 malformation. Foramen magnum decompression is recommended for a select few either as part of posterior vault expansion or at a later date. Treatment of hydrocephalus, utilizing a ventriculoperitoneal (VP) shunt with preferably a programmable high-pressure valve and anti-siphon device, is required in a small percentage of cases despite successful posterior vault expansion. Patients need to be carefully selected and managed as hydrocephalus often serves as an important cranial vault growth stimulus. Further, they require careful monitoring and thought to ensure the management of these conditions and the timing of any intervention provides the optimal long-term outcome for the patient.
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spelling pubmed-96486552022-11-15 Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis: A Review Vankipuram, Siddharth Ellenbogen, Jonathan Sinha, Ajay Kumar J Pediatr Neurosci Review Article Chiari 1 malformation and hydrocephalus are frequent findings in multi-suture and syndromic craniosynostosis patients. In this article, we review the pathogenesis, clinical significance, and management options for these conditions with comments from our own experience. The role of premature fusion of skull base sutures leading to a crowded posterior fossa and venous outflow obstruction resulting in impaired cerebrospinal fluid (CSF) absorption is highlighted. Management options are unique in this group and we advocate early (prior to 6 months of age) posterior vault expansion by distraction osteogenesis (DO) in the management of Chiari 1 malformation. Foramen magnum decompression is recommended for a select few either as part of posterior vault expansion or at a later date. Treatment of hydrocephalus, utilizing a ventriculoperitoneal (VP) shunt with preferably a programmable high-pressure valve and anti-siphon device, is required in a small percentage of cases despite successful posterior vault expansion. Patients need to be carefully selected and managed as hydrocephalus often serves as an important cranial vault growth stimulus. Further, they require careful monitoring and thought to ensure the management of these conditions and the timing of any intervention provides the optimal long-term outcome for the patient. Wolters Kluwer - Medknow 2022-09 2022-09-19 /pmc/articles/PMC9648655/ /pubmed/36388008 http://dx.doi.org/10.4103/jpn.JPN_49_22 Text en Copyright: © 2022 Journal of Pediatric Neurosciences https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Review Article
Vankipuram, Siddharth
Ellenbogen, Jonathan
Sinha, Ajay Kumar
Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis: A Review
title Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis: A Review
title_full Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis: A Review
title_fullStr Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis: A Review
title_full_unstemmed Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis: A Review
title_short Management of Chiari 1 Malformation and Hydrocephalus in Syndromic Craniosynostosis: A Review
title_sort management of chiari 1 malformation and hydrocephalus in syndromic craniosynostosis: a review
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9648655/
https://www.ncbi.nlm.nih.gov/pubmed/36388008
http://dx.doi.org/10.4103/jpn.JPN_49_22
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