Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale.
OBJECTIVE
The pathogenesis of CM-I is incompletely understood. We describe an association of CM-I and TCS that occurs in a subset of patients with normal size of the PCF.


METHODS
The prevalence of TCS was determined in a consecutively accrued cohort of 2987 patients with CM-I and 289 patients with low-lying cerebellar tonsils (LLCT). Findings in 74 children and 244 adults undergoing SFT were reviewed retrospectively. Posterior cranial fossa size and volume were measured using reconstructed 2D computed tomographic scans and MR images. Results were compared to those in 155 age- and sex-matched healthy control individuals and 280 patients with generic CM-I. The relationships of neural and osseus structures at the CCJ and TLJ were investigated morphometrically on MR images. Intraoperative CDU was used to measure anatomical structures and CSF flow in the lumbar theca.


RESULTS
Tethered cord syndrome was present in 408 patients with CM-I (14%) and 182 patients with LLCT (63%). In 318 patients undergoing SFT, there were no significant differences in the size or volume of the PCF as compared to healthy control individuals. Morphometric measurements demonstrated elongation of the brain stem (mean, 8.3 mm; P < .001), downward displacement of the medulla (mean, 4.6 mm; P < .001), and normal position of the CMD except in very young patients. Compared to patients with generic CM-I, the FM was significantly enlarged (P < .001). The FT was typically thin and taut (mean transverse diameter, 0.8 mm). After SFT, the cut ends of the FT distracted widely (mean, 41.7 mm) and CSF flow in the lumbar theca increased from a mean of 0.7 cm/s to a mean of 3.7 cm/s (P < .001). Symptoms were improved or resolved in 69 children (93%) and 203 adults (83%) and unchanged in 5 children (7%) and 39 adults (16%) and, worse, in 2 adults (1%) over a follow-up period of 6 to 27 months (mean, 16.1 months +/- 4.6 SD). Magnetic resonance imaging 1 to 18 months after surgery (mean, 5.7 months +/- 3.8 SD) revealed upward migration of the CMD (mean, 5.1 mm, P < .001), ascent of the cerebellar tonsils (mean, 3.8 mm, P < .001), reduction of brain stem length (mean, 3.9 mm, P < .001), and improvement of scoliosis or syringomyelia in some cases.


CONCLUSIONS
Chiari malformation type I/TCS appears to be a unique clinical entity that occurs as a continuum with LLCT/TCS and is distinguished from generic CM-I by enlargement of the FM and the absence of a small PCF. Distinctive features include elongation and downward displacement of the hindbrain, normal position of the CMD, tight FT, and reduced CSF flow in the lumbar theca. There is preliminary evidence that SFT can reverse moderate degrees of tonsillar ectopia and is appropriate treatment for cerebellar ptosis after Chiari surgery in this cohort.
Milhorat
Thomas H
TH
Department of Neurosurgery, The Chiari Institute, Harvey Cushing Institutes of Neuroscience, North Shore-Long Island Jewish Health System, Manhasset, NY 11030, USA. milhorat@nshs.edu
Bolognese
Paolo A
PA
Nishikawa
Misao
M
Francomano
Clair A
CA
McDonnell
Nazli B
NB
Roonprapunt
Chan
C
Kula
Roger W
RW
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001139:Arnold-Chiari Malformation / Q000453:epidemiology* / Q000503:physiopathology / Q000601:surgery*; D002420:Cauda Equina / Q000002:abnormalities / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D003388:Cranial Fossa, Posterior / Q000002:abnormalities; D004677:Encephalocele / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery; D005260:Female; D020546:Fourth Ventricle / Q000002:abnormalities / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009436:Neural Tube Defects / Q000453:epidemiology* / Q000503:physiopathology / Q000601:surgery*; D019635:Neurosurgical Procedures / Q000379:methods* / Q000706:statistics & numerical data; D015995:Prevalence; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult