Transmaxillary anterior decompressions in patients with severe basilar impression

Severe basilar impression leads to an upward translocation of the upper cervical spine and clivus into the foramen magnum and is a diagnosis best made with computed tomography or magnetic resonance imaging scans. Basilar impression may be a primary condition or secondary to bone softening disorders. Symptoms relating to direct neuraxial compression, obstruction to cerebral spinal fluid outflow, and vascular compromise all have been described. Management depends on the exact nature of the abnormality seen, but it is now firmly accepted that those with anterior neuraxial compression should have an anterior decompression. The severe basilar impression and craniofacial abnormalities seen in osteogenesis imperfecta together with the progressive nature of the condition have led to the development of a specific surgical response, the open door maxillotomy combined with a contoured loop fixation of the cervical spine. Little is known of the long term outcome of severe basilar impression, and long term studies undertaken by centers familiar with the condition and its management are required if definitive care is to be delivered to these patients.