CHIARI COMPLEX IN CHILDREN - NEURORADIOLOGICAL DIAGNOSIS, NEUROSURGICAL TREATMENT AND PROPOSAL OF A NEW CLASSIFICATION (312 CASES)

Chiari malformations are a group of anomalies particulary involving th e hindbrain and cervical spinal cord. Since these malformations presen t many common features, we called them ''Chiari Complex''. After revie wing our 312 patients affected by different types of Chiari malformati ons we propose the following classification: Chiari I (30 cases): 1) T his malformation may be divided in two sub-types: a) classic and b) my elencephalic forms. 2) Only three children were admitted with specific clinical symptoms and they had an occipito-cervical surgical decompre ssion. Chiari II (276 cases): 1) Most of our patients (70%) presented with progressive hydrocephalus and they needed a CSF shunt to be inser ted. 2) Seven sub-types of 4th ventricle morphology and size were iden tified. 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-cervic al cephalocele. In both cases there were other severe associated CNS m alformations. Chiari IV (4 cases): We propose this name for patients w ith myelomeningocele (MMC) and severe cerebellar hypoplasia.