BACKGROUND: Various combinations of bony and soft tissue anomalies have been described at the craniovertebral junction (CVJ). These include posterior fossa dermoids (with or without dermal sinuses) associated with Klippel-Feil anomalies and dermoids (no previous reports of overlying dermal sinuses) associated with bony anomalies.PATIENTS: Two patients with a combination of CVJ anomalies are described.RESULTS: Both patients had a posterior fossa dermoid, dermal sinus, and occipitalized C1 arch. Furthermore, one patient had a complex bony CVJ anomaly, and the other had a Klippel-Feil anomaly in addition. The clinical presentation and management are elucidated.CONCLUSIONS: Patients who present with any one of these lesions need to be evaluated for the existence of other congenital anomalies. These are complex malformations to treat.
Adult Atlanto-Occipital Joint