Rare manifestation of a craniovertebral junction anomaly: is blue breath holding always benign?

Breath-holding or reflex anoxic seizures predominantly occur in preschool children and usually resolve by late childhood, rarely persisting through adolescence and into adulthood. Investigations are generally unnecessary. Education and reassurance is the mainstay of management. In this report, the author describes a case in which an infant presented with breath-holding spells due to a significant craniovertebral anomaly, and underwent a C1-C2 posterior stabilization. Following the stabilization of his dislocation the breath-holding spells never reappeared. Authors have previously described a rare manifestation of craniovertebral junction anomaly. Additionally, it is important to recognize that in a subset of patients breath-holding spells may be hazardous and not benign.