NEONATAL HYPOXEMIA DUE TO MISALIGNED LUNG VESSELS WITH ALVEOLAR-CAPILLARY DYSPLASIA

Background. - Refractory hypoxemia in the newborn requires a precise d iagnostic investigation for optimal and fast management. Case report. - A full term newborn presented with refractory hypoxemia associated w ith radiologically clear lung fields and extrapulmonary shunt. Echocar diography ruled out a cardiac malformation. The persistence of hypoxem ia despite treatment of the extrapulmonary shunt and the absence of pa renchymatous pulmonary disease led to suspect misaligned lung vessels with alveolar capillary dysplasia. This diagnosis was confirmed by pos t mortem microscopic examination of the lung. Conclusion. - The diagno sis of misaligned lung vessels with alveolar capillary dysplasia can b e suspected on clinical features. The disposition of pulmonary veins m ust be checked to recognize this disease in case of neonatal death wit h pulmonary hypertension. (C) 1998, Elsevier, Paris.