We report on a term neonate with unexplained respiratory distress, dilatation of the descending aorta, and low serum retinol concentration. The respiratory distress did not respond to conventional medical management and persisted for 22 days requiring an inspired oxygen fraction of 0.4 to 0.5 to maintain adequate arterial oxygen saturation. One week after intramuscular vitamin A therapy, the respiratory distress and requirement for supplementary oxygen resolved. Dilatation of the distal aorta resolved at 7 weeks of age. An association between vitamin A deficiency and aortic dilatation has previously been described in rats, but the association in humans has rarely been reported. We suggest that unexplained neonatal respiratory distress and a dilated aorta should prompt suspicion of vitamin A deficiency. An underlying infective or inflammatory process may give rise to a falsely low serum retinol concentration. Serum retinol and retinol binding protein concentrations in both the mother and infant should be used to guide vitamin A status, treatment, and subsequent response.

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