gms | German Medical Science

59th Annual Meeting of the German Society of Neurosurgery (DGNC)
3rd Joint Meeting with the Italian Neurosurgical Society (SINch)

German Society of Neurosurgery (DGNC)

1 - 4 June 2008, Würzburg

Treatment of Chiari malformations in children

Behandlung von Chiari Malformationen im Kindesalter

Meeting Abstract

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  • corresponding author J. Klekamp - Zentrum Neurochirurgie, Christliches Krankenhaus Quakenbrück

Deutsche Gesellschaft für Neurochirurgie. Società Italiana di Neurochirurgia. 59. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3. Joint Meeting mit der Italienischen Gesellschaft für Neurochirurgie (SINch). Würzburg, 01.-04.06.2008. Düsseldorf: German Medical Science GMS Publishing House; 2008. DocMO.05.09

The electronic version of this article is the complete one and can be found online at:

Published: May 30, 2008

© 2008 Klekamp.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.



Objective: Whereas Chiari I malformation (CMI) is caused by a small posterior fossa, well treatable by decompression and does not represent a primary CNS abnormality, the pathophysiology of Chiari II malformation (CMII) and justification of its surgical treatment are discussed controversially. This paper demonstrates surgical results for children with CMI and CMII to provide treatment guidelines.

Methods: Hospital files, neuroradiological examinations, intraoperative documentations and follow-up examinations were analyzed. Rates of postoperative improvements and clinical recurrence rates according to Kaplan-Meier statistics were determined.

Results: 37 of 492 CMI-patients and 15 of 25 CMII-patients presented in the pediatric age group. Children with CMI were significantly older than those with CMII (12±4 years and 6±6 years, respectively). For both groups symptom patterns changed with age: in newborns, respiratory problems and caudal cranial nerve dysfunctions were observed, whereas in elder children scoliosis, gait ataxia and occipital pain predominated. Clinical history was significantly longer in CMI and more dramatic in CMII. All children underwent decompression either at the foramen magnum (CMI) or the cervical canal (CMII), provided no hydrocephalus or functional shunt was present. Postoperatively, 69% of children with CMI were improved compared to 33% with CMII, whereas 27% and 50%, respectively, remained unchanged. Clinical recurrence rates were 13% after 1 and 5 years for CMI and 16% and 44%, respectively, for CMII. Late deteriorations in CMII were related to postoperative cervical instability.

Conclusions: Children with CMI and CMII benefit from decompressive surgery. In CMII, indication for surgery should be considered early as further neurological progression can be prevented if measures are taken to avoid or treat postoperative instability. This study does not support views, that neurological deteriorations in sufficiently shunted children with CMII indicate an untreatable developmental defect of the CNS.