선천성 낭종양 선종양 기형과 외엽형 폐격리증은 각각드문 질환으로 이 두가지 질환이 퐁반되는 경우는 매우 드물다 본원에서는 출생 후 호흡시 흥벽 함몰과 빈호흡을 보인 생후 3주된 남아에서 컴퓨터 단층촬영을 통해 우폐하엽의 선천성 낭종성 선종양기형을의심하여 개흥술을시행하였다. 수술소견상 우폐하엽의 낭종성 병변 이외에 독립된 늑막에 둘러싸인채 비정상적인 혈류 공급을 받고 있는 종괴를 발견하여 하행 흉부 대동맥으로 부터 공급되는 2개의 동맥 가지와기정맥으로 유입되는 정맥분지를 결찰한 후 우하엽절제술과 더불어 격리폐를 절제하였다. 병리검사로 상기 두질환의 동반례로확진 하였고 환자는 건강한 모습으로 퇴원할 수 있었다.
Pulmonary sequestration and congenital cystic adenomatoid malformation are two infrequent congenital pulmonary diseases and the combination of these two entities is rare. We had experienced a 3 week old male patient with a pulmonary mass, who had been sufyerring from tachypnea and chest wall retraction after birth. The pulmonary mass was suspected as a congenital cystic adenomat id malformation by chest CT. And therefore, we performed urgent operation via standard thoracotomy incision. Upon a thoracotomy, there was an extrapleural mass with anomalous blood supply near the posterior diaphragm and multiple cystic lesions in right lower lobe. The two anomalous arteries arising from the thoracic aorta and one vein draining into the azygos vein were ligated. and then the extrapleural mass was removed and a right lower lobectomy was performed. Final histologic diagnosis was congenital cystic adenomatoid malformation associated with extralobar pulmonary sequestration. The patient was discharged without any problem.
