Congenital Anomalies at the Craniovertebral Junction–Posterior Fossa Region: Report of Two Cases

Abstract

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.

Keywords

craniovertebral junction - bony anomalies - Klippel-Feil anomaly - intracranial dermoid - dermal sinus - occipitalized C1 arch

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