Chiari I malformation and acute acquired comitant esotropia

Case report and review of the literature

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✓The authors describe a rare case of a Chiari I malformation presenting with acute acquired comitant esotropia (AACE) in a 5-year-old boy. A posterior fossa decompression with duraplasty and a C1–2 laminectomy were performed. There was an immediate postoperative improvement in the esotropia, which completely resolved by 7 months following surgery. The pertinent literature is discussed and reasons are presented for recommending posterior fossa decompression in certain patients, rather than strabismus surgery, as the initial treatment for esotropia. The authors suggest that in patients with AACE, even subtle symptoms and signs of Chiari I malformation should prompt imaging of the posterior fossa. Strong consideration should be given to performing posterior fossa decompression in patients with Chiari I malformation and AACE before strabismus surgery because the esotropia may completely resolve with decompression.

Article Information

Address reprint requests to: Frederick F. Lang, M.D., Department of Neurosurgery, M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 442, Houston, Texas 77030-4009. email: flang@mdanderson.org.

© AANS, except where prohibited by US copyright law.

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Figures

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    Preoperative unenhanced sagittal T1-weighted (A) and axial T2-weighted (B) MR images demonstrating tonsillar herniation to the C2–3 level, along with early hydromyelia or edema of the lower brainstem and upper cervical spinal cord (arrowhead).

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    Postoperative MR images. A suboccipital decompressive craniectomy, C1–2 laminectomies, and duraplasty have been performed with significant reduction of the tonsillar herniation previously seen on the sagittal T1-weighted image (A) and resolution of the brainstem edema previously seen on the axial T2-weighted image (B).

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