Chronic tonsillar herniation and Crouzon's syndrome

Pediatr Neurosurg. 1992;18(4):202-6. 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.

Publication types

  • Case Reports

MeSH terms

  • 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