Posterior fossa arachnoid cysts and cerebellar tonsillar descent: short review

The objective of this study was to analyze the association of cerebellar tonsillar descent and syringomyelia in patients with posterior fossa arachnoid cysts. We reviewed the medical records of ten patients (mean, age 33; range, 24-49 years) diagnosed with posterior fossa arachnoid cyst and tonsillar descent. Symptoms evolved over a mean of 12 months (range, 6 months to 3 years). Syringomyelia was present in six cases. Six patients underwent a suboccipital craniectomy, three cases underwent an additional C1 laminectomy, and a further case had a limited craniectomy and tonsillar reduction. Three patients were also treated for hydrocephalus: one with a ventriculoperitoneal shunt and two with endoscopic third ventriculostomy. Two patients had conservative treatment. The posterior fossa arachnoid cysts were located at the vermis-cisterna magna (n = 4), the cerebellar hemispheres (n = 2), the cerebellopontine angle (n = 3), and the quadrigeminal cistern (n = 1). A patient with achondroplasia showed features of platybasia. Associated malformations included craniofacial dysmorphism in a patient diagnosed of trichorhinophalangeal syndrome and a case with a primary temporal arachnoid cyst. After a mean follow-up of 2 years (range, 3 months to 5 years), four patients showed resolution of their neurological symptoms, and two exhibited persisting ocular findings. Headaches and nuchalgia improved in four cases and persisted in four. Syringomyelia was resolved in four patients and improved in two. Patients harboring a posterior fossa arachnoid cyst may evolve with acquired Chiari malformation and syringomyelia. Initial management should be directed to decompressing the foramen magnum and should include the resection of the arachnoid cyst's walls. A wait-and-see attitude can be implemented in selected cases. In our experience, hydrocephalus should be properly addressed before treating the arachnoid cyst.