title: Chronic tonsillar herniation and Crouzon's syndrome.
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.
authors: Division of Neurological Surgery, Barrow Neurological Institute, Phoenix, Ariz 85013.
authors: P M
authors: PM
authors: Francis
authors: S
authors: S
authors: Beals
authors: H L
authors: HL
authors: Rekate
authors: H W
authors: HW
authors: Pittman
authors: K
authors: K
authors: Manwaring
authors: J
authors: J
authors: Reiff
mesh_terms: D002526:Cerebellar Diseases / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D002533:Cerebral Angiography; D002648:Child; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D003399:Craniotomy; D004677:Encephalocele / Q000175:diagnosis / Q000235:genetics / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D011183:Postoperative Complications / Q000175:diagnosis / Q000601:surgery