Chiari complex in children--neuroradiological diagnosis, neurosurgical treatment and proposal of a new classification (312 cases)

Abstract

Chiari malformations are a group of anomalies particularly 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.