Development , validation , and incorporation of a small number of disease-specific patient-based instruments will improve the quality of research and care of CM-I patients
OBJECT Chiari malformation Type I ( CM-I ) is a common and often debilitating neurological disease .
Efforts to improve treatment of CM-I are impeded by inconsistent and limited methods of evaluating clinical outcomes .
Correlation of hindbrain CSF flow and outcome after surgical decompression for Chiari I malformation
Objective Many patients with symptomatic Chiari I malformation experience symptom recurrence after surgical decompression . Improved radiographic predictors of outcome are needed to better select patients most likely to benefit from surgical intervention . We examined whether ventral or dorsal cerebrospinal fluid ( CSF ) flow dynamics assessed by cine phase-contrast MRI scans could predict response to posterior fossa decompression for Chiari I malformation . Methods Forty-four consecutive pediatric patients undergoing pre-operative cine phase-contrast MRI followed by posterior fossa decompression for Chiari I malformation were retrospectively review ed . The association of pre-operative ventral or dorsal CSF flow abnormalities at the foramen magnum with symptom-free survival after surgical decompression was assessed via Kaplan – Meier plots and log-rank analysis . Results Mean ± SD age at time of surgery was 8 ± 6 years . Sixteen ( 36 % ) patients demonstrated decreased CSF flow dorsal to the cervico-medullary brainstem alone . Fourteen ( 32 % ) patients demonstrated abnormal CSF flow both ventral and dorsal to the cervico-medullary brainstem . Fourteen ( 32 % ) had normal hindbrain CSF flow . Overall , 13 ( 30 % ) patients experienced some degree of symptom recurrence by last follow-up ( mean of 27 ± 16 months post-operatively ) . Symptom recurrence did not differ as a function of degree of tonsilar ectopia ( p = 0.55 ) . Abnormal CSF flow dorsal to the cervico-medullary brainstem was not associated with symptom recurrence after surgical decompression ( p = 0.10 ) . However , combined pre-operative ventral and dorsal CSF flow abnormality was associated with a significant reduction ( 2.6-fold ) in the risk of post-operative symptom recurrence ( p < 0.05 ) . Only one patient ( 7 % ) with pre-operative ventral and dorsal CSF flow pathology experienced symptom recurrence 3.5 years after surgery versus 12 ( 40 % ) patients without ventral CSF flow pathology . There were otherwise no differences in baseline clinical , radiological , or operative variables between patients with abnormal versus normal ventral CSF flow . Conclusion The presence of decreased CSF flow both ventral and dorsal to the cervico-medullary brainstem was associated with improved response to hindbrain decompression for Chiari I malformation in children . Cine phase-contrast MRI may be a useful tool for surgical risk stratification and identifying patients that may be optimal surgical c and i date s. Combined ventral and dorsal hindbrain CSF flow pathology may better predict response to posterior fossa decompression compared to dorsal CSF flow pathology alone
Development , validation , and incorporation of a small number of disease-specific patient-based instruments will improve the quality of research and care of CM-I patients
OBJECT Chiari malformation Type I ( CM-I ) is a common and often debilitating neurological disease .
Efforts to improve treatment of CM-I are impeded by inconsistent and limited methods of evaluating clinical outcomes .