Unexplained neonatal cyanosis: don’t forget the dyshemoglobinemias

Abstract

The diagnosis of dyshemoglobinemias is a challenge. A high index of suspicion is required in neonates with central cyanosis out of proportion to the oxygen saturation, not responding to supplemental oxygen and an oxygen saturation gap exceeding 5%. Gold standard for the diagnosis is co-oximetry. We report two neonates with rare forms of dyshemoglobinemias. The first neonate has a, probably congenital, methemoglobinemia and was treated with methylene blue (rescue treatment) and vitamin B2 (maintenance therapy). The second neonate had a sulfhemoglobinemia due to Escherichia coli and was treated with blood transfusions. Early recognition of dyshemoglobinemias is vital because early therapy is life-saving.