Chiari 1 Malformation and Raised Intracranial Pressure

Overview

Journal Childs Nerv Syst

Specialty Pediatrics

Date 2019 Jun 15

PMID 31197535

Citations 4

Authors

Rory J Piper

Shailendra A Magdum

Affiliations

Soon will be listed here.

Abstract

Background: The pathophysiology of Chiari 1 malformation (CM1) is inextricably related to intracranial pressure (ICP). The characteristic cerebellar tonsil herniation at the foramen magnum may either cause raised ICP by disturbing CSF flow (as observed in idiopathic CM1) or may itself be the effect of raised ICP (as observed in acquired CM1). Distinguishing between these two phenomena, therefore, is of paramount importance in successfully alleviating the symptoms of the condition and preventing serious complications.

Objectives: In this article, we discuss the pathophysiology of raised ICP in CM1 and review the current evidence for its investigation and treatment. We also share our own clinical experience which investigates the utility of ICP monitoring in a series of 26 children with CM1.

Citing Articles

Endoscopic third ventriculostomy for the management of children with cerebrospinal fluid disorders, ventriculomegaly, and associated Chiari I malformation.

Saenz A, Piper R, Thompson D, Tahir M World Neurosurg X. 2023; 19:100200.

PMID: 37181585 PMC: 10172834. DOI: 10.1016/j.wnsx.2023.100200.

UK Chiari 1 Study: protocol for a prospective, observational, multicentre study.

Piper R, Afshari F, Soon W, Kolias A, Dyson E, Watkins L BMJ Open. 2021; 11(4):e043712.

PMID: 33846149 PMC: 8048021. DOI: 10.1136/bmjopen-2020-043712.

Rare and de novo coding variants in chromodomain genes in Chiari I malformation.

Sadler B, Wilborn J, Antunes L, Kuensting T, Hale A, Gannon S Am J Hum Genet. 2020; 108(1):100-114.

PMID: 33352116 PMC: 7820723. DOI: 10.1016/j.ajhg.2020.12.001.

Chiari 1 malformation and exome sequencing in 51 trios: the emerging role of rare missense variants in chromatin-remodeling genes.

Provenzano A, La Barbera A, Scagnet M, Pagliazzi A, Traficante G, Pantaleo M Hum Genet. 2020; 140(4):625-647.

PMID: 33337535 PMC: 7981314. DOI: 10.1007/s00439-020-02231-6.

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