Objective: First report of a term neonate with coexistent congenital cystic adenomatoid malformation (CCAM) of the lung and alveolar capillary dysplasia (ACD).
Methods And Design: Case report and literature review. Our institutional review board waived the need for consent.
Setting: We describe a term neonate with antenatally diagnosed CCAM and persistent pulmonary hypertension of the newborn (PPHN) who underwent right upper lobe resection on day 9 of life. Histology confirmed CCAM but closer examination also showed ACD. Postoperatively pulmonary hypertension persisted despite high-frequency oscillation and inhaled nitric oxide, and she was placed on extracorporeal membrane oxygenation. Due to the lack of any improvement, intensive care treatment was withdrawn 4 days later.
Conclusions: This is the first description of an association between these two rare malformations. Although a causative link between CCAM and ACD is possible, it is unlikely. ACD should always be considered as a cause of severe PPHN when persistent beyond 10 days, even if another etiology of PPHN is present.
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