title: Normalization of hindbrain morphology after decompression of Chiari malformation Type I.
abstract: OBJECTIVE
  Chiari malformation Type I (CM-I) is characterized by hindbrain deformity. We investigated the effects of craniocervical decompression surgery on the anatomical features of hindbrain deformity with a prospective MRI study of patients with CM-I.
  
  
  METHODS
  A prospective longitudinal study was conducted in 48 patients with CM-I (39 with syringomyelia) treated with craniocervical decompression. Clinical examinations and cervical MRI were performed before surgery and 1 week, 3-6 months, and annually after surgery. Hindbrain deformity was defined by tonsillar ectopia, pointed cerebellar tonsils, and/or cervicomedullary protuberance. The length of the clivus, basiocciput (sphenooccipital synchondrosis to basion), supraocciput (internal occipital protuberance to opisthion), and anteroposterior (AP) width of CSF pathways at the foramen magnum were measured and compared with those from 18 healthy volunteers (control group).
  
  
  RESULTS
  Before surgery, the patients' posterior fossa bones were short and their CSF pathways were narrow. All patients had tonsillar ectopia (mean [± SD] 12.3 ± 5.1 mm; normal 0.3 ± 1.0). The majority of patients had pointed tonsils and more than two-thirds exhibited a cervicomedullary protuberance. Clivus and basiocciput lengths were significantly shorter than the values obtained in the control group. However, the supraocciput length did not differ significantly from control measurements. The mean bulbopontine sulcus distance superior to the basion was 9.5 ± 2.6 mm (vs 13.6 ± 2.8 mm in controls; p < 0.0001). The AP widths of the CSF pathways at the level of the foramen magnum were significantly narrowed. After surgery, CSF pathways significantly expanded both ventrally and dorsally. By 3-6 months after surgery, pointed tonsils became round, cervicomedullary protuberance disappeared, and tonsillar ectopia diminished by 51% (to 6.0 ± 3.3 mm; p < 0.0001).
  
  
  CONCLUSIONS
  The cerebellar tonsils and brainstem assumed a normal appearance within 6 months after craniocervical decompression. These findings support the concept that the CM-I is not a congenital malformation of the neural elements but rather an acquired malformation that arises from pulsatile impaction of the cerebellar tonsils into the foramen magnum. Clinical trial registration no.: NCT00001327.
authors.affiliation: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA. heissj@ninds.nih.gov
authors.forename: John D
authors.initials: JD
authors.lastname: Heiss
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Suffredini
authors.forename: Kamran D
authors.initials: KD
authors.lastname: Bakhtian
authors.forename: Malisa
authors.initials: M
authors.lastname: Sarntinoranont
authors.forename: Edward H
authors.initials: EH
authors.lastname: Oldfield
mesh_terms: D000293:Adolescent; D000328:Adult; D001099:Arachnoid / Q000473:pathology; D001139:Arnold-Chiari Malformation / Q000473:pathology* / Q000601:surgery*; D001933:Brain Stem / Q000473:pathology; D002531:Cerebellum / Q000473:pathology; D003388:Cranial Fossa, Posterior / Q000473:pathology; D003627:Data Interpretation, Statistical; D019299:Decompression, Surgical; D005260:Female; D005539:Foramen Magnum / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019635:Neurosurgical Procedures; D012249:Rhombencephalon / Q000473:pathology*; D016896:Treatment Outcome; D055815:Young Adult