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  • Title: Congenital anomalies at the craniovertebral junction-posterior fossa region: report of two cases.
    Author: Madhugiri VS, Bhagavatula ID.
    Journal: J Neurol Surg A Cent Eur Neurosurg; 2013 Dec; 74 Suppl 1():e279-83. PubMed ID: 23959613.
    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.
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