Abstract
Introduction: Brain stem disconnection (BD) is a rare posterior fossa abnormality defined by the nearly complete absence of a brain stem segment with the rostral and caudal brain stem portions connected only by a thin cord of tissue. BD has a poor outcome and the majority of children die within the first 2 months of life without achieving developmental milestones. Only 12 children with BD have been reported so far. We report the case of two new patients with BD and a prolonged spontaneous survival to expand the phenotype.
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