Abstract
Background : Congenital bilateral vocal cord paralysis is a rare occurrence. Approximately half the cases areassociated with a major comorbidity, usually neurological, neuromuscular or malformative. Case presentation : In a male newborn, respiratory distress syndrome and stridor were observed immediatelyfollowing birth. The cause was bilateral vocal cord paralysis in the adducted position. Neuroradiologicalinvestigation revealed a unilateral discontinuity between the upper pons and the right medulla oblongata. Hypoplasia of the right posterior hemiarches of C1-C2and the right exo-occipital bone was observed, as wasa small clivus. MR angiography showed the absence of the distal right vertebral artery, with hypoplasia andparietal irregularities of the proximal segments. Respiratory autonomy was not obtained despite endoscopiclaser cordotomy, corticosteroid therapy and nasal continuous positive airway pressure. The infant died at theage of 4 weeks after treatment was limited to comfort care. Conclusions : A medullary lesion is an exceptional cause of congenital bilateral vocal cord paralysis. The strictlyunilateral neurological and vascular defect and the absence of associated intracranial or extracranial malformationmake this clinical case unique and suggest a disruptive mechanism. This case also highlights the help provided byadvanced neuroimaging techniques, i.e. fibre tracking using diffusion tensor imaging, in the decision-making process.