| Popis: |
Syringomyelia is commonly associated with Chiari I malformation and varies widely in presenting symptoms. In the context of syringomyelia-Chiari complex, posterior fossa decompression via a suboccipital approach is the most common mode of operative management, and typically produces excellent reduction in syrinx size. However, no comprehensive review specific to the operative management of holocord syringomyelia, particularly in the context of Chiari I malformation, has yet been completed. Here we present a case of a 26-year-old female with holocord syringomyelia and Chiari I malformation in the context of a congenital occiput-C1 fusion. The patient underwent decompressive surgery via suboccipital craniectomy and C1 laminectomy. Decompression of the cerebellar tonsils was achieved via resection of arachnoid over the tonsils and tonsillar coagulation, followed by expansile duraplasty. Significant reduction in holocord syrinx size was demonstrated radiographically at one month post-operatively. Notably, a large suboccipital pseudomeningocele developed, possibly due to a small defect in the duraplasty, and was accompanied by concordant ventricular interval enlargement which caused progressive nausea and headache. This was successfully managed via ventriculoperitoneal shunt placement. In the case presented here, surgical decompression via craniectomy and C1 laminectomy achieved dramatic radiographic improvement in the patient’s holocord syringomyelia that corresponded with substantial reduction of the severity of the presenting symptoms. A systematic review of the literature demonstrated that among patients with isolated holocord syringomyelia-Chiari I complex, posterior fossa decompression via suboccipital craniectomy, typically with C1 +/− C2 laminectomy and with an open-dura approach, was universally employed, with excellent outcomes and minimal complications. |
