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Chiari type I and hydrocephalus.
Childs Nerv Syst. 2019 10; 35(10):1701-1709.CN

Abstract

PURPOSE

The association between Chiari type I malformation (CIM) and hydrocephalus raises a great interest because of the still unclear pathogenesis and the management implications. The goal of this paper is to review the theories on the cause-effect mechanisms of such a relationship and to analyze the results of the management of this condition.

METHODS

A review of the literature has been performed, focusing on the articles specifically addressing the problem of CIM and hydrocephalus and on the series reporting about its treatment. Also, the personal authors' experience is briefly discussed.

RESULTS

As far as the pathogenesis is concerned, it seems clear that raised intracranial pressure due to hydrocephalus can cause a transient and reversible tonsillar caudal ectopia ("pressure from above" hypothesis), which is something different from CIM. A "complex" hypothesis, on the other hand, can explain the occurrence of hydrocephalus and CIM because of the venous engorgement resulting from the hypoplasia of the posterior cranial fossa (PCF) and the occlusion of the jugular foramina, leading to cerebellar edema (CIM) and CSF hypo-resorption (hydrocephalus). Nevertheless, such a mechanism can be advocated only in a minority of cases (syndromic craniosynostosis). In non-syndromic CIM subjects, the presence of hydrocephalus could be explained by an occlusion of the basal CSF pathways, which would occur completely in a minority of cases (only 7-10% of CIM patients show hydrocephalus) while it would be partial in the remaining cases (no hydrocephalus). This hypothesis still needs to be demonstrated. As far as the management is concerned, the strategy to treat the hydrocephalus first is commonly accepted. Because of the "obstructive" origin of CIM-related hydrocephalus, the use of endoscopic third ventriculostomy (ETV) is straightforward. Actually, the analysis of the literature, concerning 63 cases reported so far, reveals very high success rates of ETV in treating hydrocephalus (90.5%), CIM (78.5%), and syringomyelia symptoms (76%) as well as in giving a radiological improvement of both CIM (74%) and syringomyelia (89%). The failures of ETV were not attributable to CIM or syringomyelia. Only 11% of cases required PCF decompression after ETV.

CONCLUSIONS

The association between CIM and hydrocephalus probably results from different, multifactorial, and not yet completely understood mechanisms, which place the affected patients in a peculiar subgroup among those constituting the heterogeneous CIM population. ETV is confirmed as the best first approach for this subset of patients.

Authors+Show Affiliations

Fondazione Policlinico Gemelli IRCCS, Neurochirurgia Infantile, Roma, Italy. lmassmi@email.it. Istituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Roma, Italy. lmassmi@email.it. International Neuroscience Institute, Hannover, Germany. lmassmi@email.it.Istituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Roma, Italy.Istituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Roma, Italy.Fondazione Policlinico Gemelli IRCCS, Neurochirurgia Infantile, Roma, Italy. Istituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Roma, Italy.International Neuroscience Institute, Hannover, Germany.Fondazione Policlinico Gemelli IRCCS, Neurochirurgia Infantile, Roma, Italy. Istituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Roma, Italy.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

31227858

Citation

Massimi, Luca, et al. "Chiari Type I and Hydrocephalus." Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery, vol. 35, no. 10, 2019, pp. 1701-1709.
Massimi L, Pennisi G, Frassanito P, et al. Chiari type I and hydrocephalus. Childs Nerv Syst. 2019;35(10):1701-1709.
Massimi, L., Pennisi, G., Frassanito, P., Tamburrini, G., Di Rocco, C., & Caldarelli, M. (2019). Chiari type I and hydrocephalus. Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery, 35(10), 1701-1709. https://doi.org/10.1007/s00381-019-04245-6
Massimi L, et al. Chiari Type I and Hydrocephalus. Childs Nerv Syst. 2019;35(10):1701-1709. PubMed PMID: 31227858.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Chiari type I and hydrocephalus. AU - Massimi,Luca, AU - Pennisi,Giovanni, AU - Frassanito,Paolo, AU - Tamburrini,Gianpiero, AU - Di Rocco,Concezio, AU - Caldarelli,Massimo, Y1 - 2019/06/21/ PY - 2019/04/22/received PY - 2019/05/30/accepted PY - 2019/6/23/pubmed PY - 2020/10/24/medline PY - 2019/6/23/entrez KW - Chiari I malformation KW - Endoscopic third ventriculostomy KW - Hydrocephalus KW - Posterior cranial fossa SP - 1701 EP - 1709 JF - Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery JO - Childs Nerv Syst VL - 35 IS - 10 N2 - PURPOSE: The association between Chiari type I malformation (CIM) and hydrocephalus raises a great interest because of the still unclear pathogenesis and the management implications. The goal of this paper is to review the theories on the cause-effect mechanisms of such a relationship and to analyze the results of the management of this condition. METHODS: A review of the literature has been performed, focusing on the articles specifically addressing the problem of CIM and hydrocephalus and on the series reporting about its treatment. Also, the personal authors' experience is briefly discussed. RESULTS: As far as the pathogenesis is concerned, it seems clear that raised intracranial pressure due to hydrocephalus can cause a transient and reversible tonsillar caudal ectopia ("pressure from above" hypothesis), which is something different from CIM. A "complex" hypothesis, on the other hand, can explain the occurrence of hydrocephalus and CIM because of the venous engorgement resulting from the hypoplasia of the posterior cranial fossa (PCF) and the occlusion of the jugular foramina, leading to cerebellar edema (CIM) and CSF hypo-resorption (hydrocephalus). Nevertheless, such a mechanism can be advocated only in a minority of cases (syndromic craniosynostosis). In non-syndromic CIM subjects, the presence of hydrocephalus could be explained by an occlusion of the basal CSF pathways, which would occur completely in a minority of cases (only 7-10% of CIM patients show hydrocephalus) while it would be partial in the remaining cases (no hydrocephalus). This hypothesis still needs to be demonstrated. As far as the management is concerned, the strategy to treat the hydrocephalus first is commonly accepted. Because of the "obstructive" origin of CIM-related hydrocephalus, the use of endoscopic third ventriculostomy (ETV) is straightforward. Actually, the analysis of the literature, concerning 63 cases reported so far, reveals very high success rates of ETV in treating hydrocephalus (90.5%), CIM (78.5%), and syringomyelia symptoms (76%) as well as in giving a radiological improvement of both CIM (74%) and syringomyelia (89%). The failures of ETV were not attributable to CIM or syringomyelia. Only 11% of cases required PCF decompression after ETV. CONCLUSIONS: The association between CIM and hydrocephalus probably results from different, multifactorial, and not yet completely understood mechanisms, which place the affected patients in a peculiar subgroup among those constituting the heterogeneous CIM population. ETV is confirmed as the best first approach for this subset of patients. SN - 1433-0350 UR - https://www.unboundmedicine.com/medline/citation/31227858/Chiari_type_I_and_hydrocephalus_ L2 - https://doi.org/10.1007/s00381-019-04245-6 DB - PRIME DP - Unbound Medicine ER -