Chronic tonsillar herniation and Crouzon's syndrome

• Published in: Pediatric neurosurgery Vol. 18; no. 4; p. 202
• Main Authors: Francis, P M, Beals, S, Rekate, H L, Pittman, H W, Manwaring, K, Reiff, J
• Format: Journal Article
• Language: English
• Published: Switzerland 1992
• Subjects: Cerebellar Diseases - diagnosis; Cerebellar Diseases - genetics; Cerebellar Diseases - surgery; Cerebral Angiography; Child; Child, Preschool; Craniofacial Dysostosis - diagnosis; Craniofacial Dysostosis - genetics; Craniofacial Dysostosis - surgery; Craniotomy; Encephalocele - diagnosis; Encephalocele - genetics; Encephalocele - surgery; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Neurologic Examination; Postoperative Complications - diagnosis; Postoperative Complications - surgery
• ISSN: 1016-2291
• DOI: 10.1159/000120663

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.