Objective: Developmental remnants around the foramen magnum, or proatlas segmentation abnormalities, have been recorded in postmortem studies but very rarely in a clinical setting. Because of their rarity, the pathological anatomy has been misunderstood, and treatment has been fraught with failures. The objectives of this prospective study were to understand the correlative anatomy, pathology, and embryology and to recognize the clinical presentation and gain insights on the treatment and management.
Methods: Our craniovertebral junction (CVJ) database started in 1977 and comprises 5200 cases. This prospective study has retrieval capabilities. Neurodiagnostic studies changed with the evolution of imaging. Seventy-two patients were recognized as having symptomatic proatlas segmentation abnormalities.
Results: Ventral bony masses from the clivus or medial occipital condyle occurred in 66% (44/72), lateral or anterolateral compressive masses in 37% (27 of 72 patients), and dorsal bony compression in 17% (12 of 72 patients). Hindbrain herniation was associated in 33%. The age at presentation was 3 to 23 years. Motor symptoms occurred in 72% (52 of 72 patients); palsies in Cranial Nerves IX, X, and XII in 33% (24 of 72 patients); and vertebrobasilar symptoms in 25% (18 of 72 patients). Trauma precipitated symptoms in 55% (40 of 72 patients). The best definition of the abnormality was demonstrated by 3-dimensional computed tomography combined with magnetic resonance imaging. Treatment was aimed at decompression of the pathology and stabilization.
Conclusion: Remnants of the occipital vertebrae around the foramen magnum were recognized in 72 of 5200 CVJ cases (7.2%). Magnetic resonance imaging with 3-dimensional computed tomography of the CVJ provides the best definition and understanding of the lesions. Brainstem myelopathy and lower cranial nerve deficits are common clinical presentations in the first and second decades of life. Treatment is aimed at decompression of the pathology and CVJ stabilization.