Craniovertebral Junction Anomalies: World Federation of Neurosurgical Societies Spine Committee Recommendations Overview.

Abstract

Objective: The published literature on craniovertebral junction (CVJ) anomalies lacks a comprehensive appraisal that integrates common diagnostic, management, and treatment concepts for different conditions, such as Chiari malformation (CM), basilar invagination (BI), os odontoideum, and syndromic malformations. The authors aimed to fill this knowledge gap offering guidelines and recommendations with a global outreach and applicability.

Materials and methods: A group of expert spine surgeons and World Federation of Neurosurgical Societies Spine Committee members, most of which were extensively published on CVJ anomalies in the past, reviewed the literature issued from 2011 to 2022. Following a common methodology, based on the Delphi method, the authors analyzed the strength of the literature and voted on statements concerning the diagnosis and management of these conditions.

Results: A total of 8 papers were produced: (1) CM diagnosis, classifications, natural history, and conservative management, (2) CM: indications for surgery and surgical options, (3) pediatric CM, (4) syndromic atlantoaxial instability, (5) os odontoideum, (6) BI: diagnosis, radiology, and classification, (7) surgical treatment of BI, and (8) BI with associated CM.

Conclusions: Despite grey zones on the natural history of CVJ anomalies and controversies on timing and type of surgical treatments, whenever atlantoaxial instability is present, C1 to C2 stabilization through instrumentation and fusion is necessary. If only recurrent pain and neurological dysfunction occur, surgical decompression is appropriate. If no atlantoaxial instability is present, patients with Down syndrome can participate in competitive sports. In general, contact sports are not recommended.