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Title: Chronic tonsillar herniation and Crouzon's syndrome. Author: Francis PM, Beals S, Rekate HL, Pittman HW, Manwaring K, Reiff J. Journal: Pediatr Neurosurg; 1992; 18(4):202-6. PubMed ID: 1472433. Abstract: Patients born with craniofacial syndromes such as Crouzon's syndrome will often develop hydrocephalus after their initial craniofacial reconstructive procedures. We have treated 10 patients with Crouzon's syndrome; 5 patients required a shunting procedure after cranial remodeling. Each of these 5 shunted patients later demonstrated chronic tonsillar herniation on magnetic resonance imaging studies. One of these patients exhibited signs of pseudotumor cerebri and 1 had a spastic quadriparesis. Of the 5 patients who did not require a shunt, none displayed chronic tonsillar herniation. Our evidence suggests that jugular foramen stenosis produces an increased cerebral venous turgor that leads to a cerebrospinal fluid absorption defect and hydrocephalus. After the hydrocephalus is treated the increased venous turgor remains and provides the driving force for the development of chronic tonsillar herniation.[Abstract] [Full Text] [Related] [New Search]