Congenital Pulmonary Airway Malformation in Preterm Infants: A Case Report and Review of the Literature

TL;DR

This paper reports a rare case of a preterm infant with a congenital lung malformation and discusses management strategies based on limited existing literature.

Contribution

The paper adds a new case to the limited literature on CPAM in preterm infants and suggests a conservative management approach.

Findings

The infant showed progressive reduction of the lesion with conservative management.

Only six cases of CPAM in preterm infants have been reported in the literature.

Conservative management appears appropriate due to the risk of complications with surgery.

Abstract

Congenital pulmonary airway malformations (CPAMs) represent a well-known cluster of rare lung malformations affecting 1 in 2500 live births. The natural history of many CPAMs is to increase their size in the second trimester, reach a plateau, and, in about 50% of cases, regress and to become barely detectable during the third trimester. Little is known about cases of affected neonates born prematurely: only six cases are described in the literature, recording different conduct and outcomes. Herein, we report the case of a very low birth weight infant born at GW 28 without antenatal findings and presenting at birth with severe respiratory distress, requiring ventilation. Chest X-rays and a CT scan showed the presence of a solid mass in the left lung. An initial conservative approach was adopted as the baby gained respiratory stability within the first days of life. Routine ultrasound (US) showed a progressive reduction of the lesion, mimicking the process of involution that CPAM can exhibit during late gestation. The rarity of the condition does not allow the formulation of any suggestions regarding one type of management over the other. An initial conservative approach seems to be appropriate with regards to the outcome and possible intra- and post-operative complications.

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