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Chiari I malformation associated with syringomyelia and scoliosis
I B Ghanem1, C Londono, O Delalande
1Département de Chirurgie Orthopédique, Hôpital Hotel-Dieu de France, Paris, France.
Insights
Suboccipital foraminotomy effectively treated syringomyelia in children with Chiari I malformation, though scoliosis outcomes varied. This surgery supports the hydrodynamic theory of cerebrospinal fluid abnormalities.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Spinal Deformity Correction
Background:
- Chiari I malformation is often associated with syringomyelia and scoliosis.
- Suboccipital foraminotomy is a surgical approach to decompress the hindbrain.
- Gardner's hydrodynamic theory suggests cerebrospinal fluid (CSF) flow abnormalities contribute to these conditions.
Purpose of the Study:
- To evaluate the surgical outcomes of suboccipital foraminotomy and duroplasty in pediatric patients with Chiari I malformation.
- To assess the impact of this surgery on associated syringomyelia and spinal deformities, specifically scoliosis.
Main Methods:
- A retrospective review of 12 pediatric patients undergoing suboccipital foraminotomy for Chiari I malformation.
- Assessment of neurological impairment, syringomyelia extent, and scoliosis severity pre- and post-operatively.
- Average follow-up of 4.5 years.
Main Results:
- Syringomyelia resolved or diminished in 11 out of 12 patients within a year post-surgery.
- Neurological deficits improved in most cases, with minimal persistent deficit in one.
- Scoliosis showed variable results: improvement in one, no change in one, and progression in five severe cases requiring further intervention.
Conclusions:
- Suboccipital foraminotomy is effective in improving syringomyelia and neurological deficits in pediatric Chiari I malformation.
- The findings support the role of abnormal CSF hydrodynamics in the pathogenesis of these conditions.
- Scoliosis management remains complex, with potential for progression despite surgical decompression.
Study Design:
A retrospective review of a series of 12 children who underwent suboccipital foraminotomy and duroplasty for Chiari I malformation.
Objective:
To assess the effects of this surgery on associated syringomyelia and scoliosis.
Summary Of Background Data:
Suboccipital foraminotomy for the treatment of syringomyelia associated with Chiari I malformation was greatly stimulated by Gardner's hydrodynamic theory, and its results proved to be encouraging. However, several authors reported improvement or stabilization of associated scoliosis after this surgery.
Methods:
A retrospective review was conducted on 12 patients who underwent suboccipital foraminotomy for Chiari I malformation associated with syringomyelia. Neurologic Impairment, extent of syringomyelia, and severity of associated spinal deformity were assessed preoperatively and at a 4.5-year average follow-up (range, 2.1-12 years). Anomaly of superficial abdominal reflexes was found in all cases, and para or tetraparesis in three cases. Syringomyelia was of variable localization and extent. Scoliosis was present in 7 cases (greater than 40 degrees in 5 cases).
Results:
Diminution or complete disappearance of syringomyelia was observed in 11 cases, 3 months to 1 year after surgery. Superficial abdominal reflexes anomaly improved in four cases. Minimal neurologic deficit persisted in one case. Scoliosis improved in one case, remained unchanged in one case, and progressed in the five cases with preoperative severe deformity, requiring instrumentation and fusion.
Conclusions:
Improvement of syringomyelia and neurologic deficit, observed with suboccipital foraminotomy, supports the theory that abnormal hydrodynamics of the cerebral spinal fluid is most likely to cause these deficits.
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