Congenital Pulmonary Airway Malformation With Pectus Carinatum: A Novel Case Report
Klein Dantis, Shubham Kabra, Deepinder KaurIntroduction
Congenital pulmonary airway malformation (CPAM) is a spectrum of cystic lung lesions caused by abnormal fetal airway development. It presents diagnostic and therapeutic challenges due to variable manifestations and potential complications. Surgical management aims to balance complete lesion removal with lung function preservation. This report highlights a rare case of CPAM coexisting with pectus carinatum, emphasizing individualized surgical planning.
Case Presentation
An 11-year-old boy, prenatally diagnosed with right lung CPAM, was asymptomatic until imaging revealed a large (10 × 8.7 × 9 cm), multiseptated cystic lesion in the right lower lobe consistent with Stocker-type I CPAM. Physical examination showed symmetric pectus carinatum (PC) without cardiopulmonary compromise. Pulmonary function tests indicated mild restrictive lung disease. The patient underwent right posterolateral thoracotomy with wedge resection of basal lung segments, preserving lung parenchyma. Histopathology confirmed CPAM. Postoperative recovery was uneventful, with no recurrence or complications during 44 weeks of follow-up. The PC showed favorable response to the orthotic treatment.
Conclusion
Wedge resection is a safe, effective alternative to lobectomy in selected older children with localized CPAM. Long-term follow-up with pulmonary function tests and imaging is essential. The rare association of CPAM with PC expands related anomalies and emphasizes the need for individualized, multidisciplinary management.