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In neurology, the Chiari malformation (/ k i ˈ ɑː r i / kee-AR-ee; CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum (the opening at the base of the skull).
Obstruction to CSF flow and/or absorption can occur in hydrocephalus (blockage in ventricles or subarachnoid space at base of brain, e.g., by Arnold–Chiari malformation), extensive meningeal disease (e.g., infection, carcinoma, granuloma, or hemorrhage), or obstruction in cerebral convexities and superior sagittal sinus (decreased absorption).
The currently accepted radiographic definition for a Chiari malformation is that cerebellar tonsils lie at least 5mm below the level of the foramen magnum. Some clinicians have reported that some patients appear to experience symptoms consistent with a Chiari malformation without radiographic evidence of tonsillar herniation.
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With Austrian pathologist, Hans Chiari, his name is lent to a condition known as Arnold–Chiari malformation, a disorder that takes place when the cerebellar tonsils and the medulla oblongata protrude through the foramen magnum into the spinal canal, without displacing the lower brain stem. [2]
It is frequently co-morbid with atlanto-axial joint instability, Chiari malformation, [3] or tethered spinal cord syndrome. The condition can be brought on by physical trauma , including whiplash , laxity of the ligaments surrounding the joint, or other damage to the surrounding connective tissue.
Arnold–Chiari malformation, or simply "Chiari malformation", a malformation of the brain; Budd–Chiari syndrome, a disease with typical symptoms of abdominal pain, ascites and hepatomegaly caused by occlusion of the hepatic veins; Chiari–Frommel syndrome, an older term for hyperprolactinaemia with extended postpartum galactorrhea and ...
The Chiari malformation may be asymptomatic or present with ataxia, spasticity or abnormalities in breathing, swallowing or sleeping. [ 6 ] Due to the impaired venous outflow, which may be further complicated with an Arnold–Chiari malformation, there is often a clinical image of hydrocephalus present.
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