Chiari Malformation

Chiari malformations are neurological congenital anomalies that are affect the content of posterior cranial fossa. The anomalies are classified anatomically into four types mainly, the severity of each type is determined according to the part involved. The classification mainly relies on the displacement and herniation of the posterior fossa content such as cerebellum and the brainstem. Chiari malformation is ranging from simple or asymptomatic herniation of the cerebellar tonsils to very severe displacement involving the brainstem or complete agenesis of the cerebellum. The clinical presentation is commonly the occipital and cervical pai. Other presentations are related to the part that is affected, such as brainstem syndromes or dysfunction, spinal symptoms such as weakness or sensory loss of the upper and lower limbs, the cerebellar symptoms such as ataxia. Chiari malformation tends to be associated with other anomalies such as hydrocephalus and syringomyelia. The diagnostic imaging of choice in Chiari malformation is magnetic resonance imaging, the scan can detect the anatomical herniation and defects and also useful in preoperative planning. Other imaging techniques are also beneficial options to detect specific findings or establishing the diagnosis prenatally. Chiari malformation is mainly a surgical disease, the medical treatment is not available option. The decision making depends on multiple factors mainly the clinical presentation, the severity of symptoms and the associated anomalies. Severe symptoms or progressive presentation indicate the surgical approach. The surgical management include decompressing the posterior fossa contents, reduce herniation, maintaining the cerebrospinal fluid flow in addition to the dural graft. The treatment also indicates the repair of the associated anomalies before the decompression such as hydrocephalus.