Open Cyst Drainage for Reliable Decompression in a Critically Ill Neonate with Congenital Pulmonary Airway Malformation.

INTRODUCTION: Congenital pulmonary airway malformation (CPAM) is a rare congenital lung lesion that is often diagnosed prenatally. Large macrocystic lesions can cause severe respiratory compromise after birth, requiring urgent decompression. Although staged management with cyst drainage followed by delayed resection has been reported, the optimal method for achieving reliable decompression in critically unstable neonates remains unclear. We report a case in which emergency open cyst drainage under direct visualization enabled secure catheter placement and reliable decompression before definitive surgery.

CASE PRESENTATION: A female neonate with a large macrocystic CPAM underwent thoracoamniotic shunting during the fetal period. After birth, she developed severe respiratory failure caused by rapid enlargement of the cystic lesion and mediastinal shift. Because the precise extent of the lesion remained unclear and immediate definitive resection was considered unsafe, emergency open cyst drainage was performed. Under direct visualization, the previously placed shunt tubes were removed, and drainage catheters were securely inserted into the cystic lesions and fixed to the cyst walls. Continuous drainage achieved reliable decompression, relieved compression of the normal lung, and improved respiratory status. After sufficient lung expansion and a more accurate assessment of the lesion extent, delayed lower lobectomy was safely performed. The postoperative course was favorable.

CONCLUSIONS: Emergency open cyst drainage under direct visualization may provide reliable decompression in critically ill neonates with severe CPAM. This technique may be a useful alternative to percutaneous drainage when secure catheter placement and predictable decompression are required. Effective decompression may also facilitate staged management and allow definitive resection under safer conditions.