Eur J Pediatr Surg 1995; 5: 35-38
DOI: 10.1055/s-2008-1066261
Case report

© Georg Thieme Verlag KG Stuttgart · New York

Chiari Complex in Children - Neuroradiological Diagnosis, Neurosurgical Treatment and Proposal of a New Classification (312 Cases)

A.  Cama1 , P.  Tortori-Donati2 , G. L. Piatelli1 , M. P. Fondelli2 , L.  Andreussi1
  • 1Department of Pediatric Neurosurgery, Istituto Scientifico G. Gaslini, Genova, Italy
  • 2Department of Pediatric Neuroradiology, Istituto Scientifico G. Gaslini, Genova, Italy
Further Information

Publication History

Publication Date:
25 March 2008 (online)


Chiari malformations are a group of anomalies particulary involving the hindbrain and cervical spinal cord.

Since these malformations present many common features, we called them "Chiari Complex". After reviewing our 312 patients affected by different types of Chiari malformations we propose the following classification:

Chiari I (30 cases): 1) This malformation may be divided in two sub-types: a) classic and b) myelencephalic forms. 2) Only three children were admitted with specific clinical symptoms and they had an occipito-cervical surgical decompression.

Chiari II (276 cases): 1) Most of our patients (70%) presented with progressive hydrocephalus and they needed a CSF shunt to be inserted. 2) Seven sub-types of 4th ventricle morphology and size were identified. 3) Only 11 patients underwent a cervical decompression; in 182 children CSF shunting resulted in a good clinical outcome.

Chiari III (2 cases): Chiari II signs must be associated with an occipito-cervical cephalocele. In both cases there were other severe associated CNS malformations.

Chiari IV (4 cases): We propose this name for patients with myelomeningocele (MMC) and severe cerebellar hypoplasia.