What is the best strategy for craniovertebral junction (CVJ) anomalies associated with posterior fossa arachnoid cyst and hydrocephalus: An extremely rare case report

Occurrence of craniovertebral junction (CVJ) anomalies in association with posterior fossa arachnoid cyst and hydrocephalus is extremely rare; no such prior cases have been documented in the literature. There are no definitive guidelines for the management of such lesions. Management strategy should account for high intracranial pressure, compression of the brain stem and cervical cord, as well as for the craniospinal instability. We report the first such case and describe the management procedure. Staged surgical intervention was carried out. The patient developed serious complications after the first staged operation, although the final outcome was good. The authors emphasize the importance of fixation and decompression for patients with such complex lesions. Our experience may provide a treatment strategy for similar cases and even for all craniovertebral junction (CVJ) anomalies associated with posterior fossa lesions