Analysis of the treatment of 576 patients with congenital craniovertebral junction malformations.

We aim to report our experience treating craniovertebral junction malformations (CVJM) and to investigate the management of this uncommon condition. Between 2000 and 2009, 629 patients with CVJM underwent surgery in our department. Fifty-three patients were lost to follow-up; therefore 576 patients completed follow-up, for an average period of 3 years and 2 months. All patients were diagnosed on the basis of clinical presentation and imaging features, and we found that anomalies of the occipitocervical junction manifested as four major types. Different microsurgical treatments were carried out in these patients according to disease type, and the effectiveness of individualised treatments was analysed. Categorizing patients with CVJM into these four types to simplify this somewhat unclear area could provide insight into the pathogenesis of the anomaly and a basis for rational surgical treatment.