Congenital Chylothorax and Congenital Pulmonary Airway Malformation: Case Report and Literature Review

The association of two rare but important congenital conditions-congenital chylothorax (CCT) and congenital pulmonary airway malformation (CPAM)-can be challenging to manage, especially in the absence of well-established protocols. We report an association between CPAM and CCT in a newborn. After birth, CCT did not respond to conservative treatment, and at the time of CPAM resection, thoracic duct ligation and abrasive pleurodesis were also performed. Despite these interventions, the CCT persisted even with the subsequent administration of octreotide and propranolol. Finally, after 56 days, chemical pleurodesis with povidone-iodine was performed. Chest tube drainage ceased, and the thoracic drain was removed 4 days later. Conservative treatment remains the first-line approach for neonatal CCT. However, when CCT is associated with CPAM and fails to respond to conservative measures, thoracic duct ligation should be considered at the time of lung malformation resection. If these interventions remain ineffective, chemical pleurodesis is a viable therapeutic option.