• Paolo Perrini, Nicola Benedetto, Enrico Guidi, and Nicola Di Lorenzo.
• Neurosurgical Department, University of Pisa, Pisa, Italy. p.perrini@ao-pisa.toscana.it
• Neurosurgery. 2009 May 1;64(5 Suppl 2):331-42; discussion 342.

ObjectiveTo review our experience with the surgical management of craniovertebral junction malformations, focusing on the selection of surgical approach, management of the associated Chiari malformation, and postoperative instability.MethodsDuring a 7-year period (May 2000-May 2007), 34 patients with a mean age of 55 years (age range, 32-75 years) underwent transoral surgery for fixed or nearly fixed ventral compression at the craniovertebral junction caused by basilar invagination and/or atlantoaxial dislocation. Chiari malformation was detected in 13 patients. The most common presenting signs were motor deficits (88%), followed by sensory loss (35%). All patients but one who had posterior stabilization performed elsewhere underwent single-stage anterior decompression and posterior occipitocervical fixation. Adjuncts to the transoral approach were tailored to the local anatomy (severity of basilar invagination, extent of mandibular excursion) found in each patient. Posterior fossa decompression was performed in 3 patients with Chiari malformation.ResultsThirty-one patients were alive at the time of the last follow-up evaluation (average, 3.7 years; range, 0.5-7.5 years). Of the 28 surviving patients admitted with preoperative motor impairment, 24 patients (86%) improved at least 1 Nurick grade, whereas the grade did not change in 4 (14%) patients. There were 2 (6%) perioperative deaths, and 1 other patient died subsequently of causes unrelated to surgery. Surgical morbidity was 18% and included dural laceration, cerebrospinal fluid leak with meningitis, malocclusion, oral wound dehiscence, and occipital wound infection. Delayed instability occurred in 1 patient because of cranial settling of the C2 vertebral body.ConclusionSuccessful decompression of the abnormal craniovertebral junction requires extensive preoperative evaluation, appropriate tailoring of the operative approach, and an adequate learning curve. Transmaxillary approaches are useful adjuncts to the transoral approach in patients with severe basilar invagination or in cases of limited jaw mobility. Anterior decompression has been proven effective in relieving obstruction of the subarachnoid space at the foramen magnum in most patients with associated Chiari malformation.

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