Abstract
Congenital cystic adenomatoid malformation of the lung is a rare pulmonary malformation, Although it is one of the most common congenital anomalies which cause acute respiratory distress in the newborn infants, characterized by marked proliferation of terminal respiratory structures. We have experienced an unusual case of congenital cystic adenomatoid malformation associated with pectus excavatum. The patient was 3-year-old female who suffered from cough and high fever for 20 days, and antibiotic therapy was given in other hospital before transfer to our hospital. The findings on chest X-ray, chest CT, aortogram, and selective bronchial arteriogram showed cystic lesions in the right upper and middle lobe accompanied but severe pectus excavatum. Right bilobectomy for pulmonary lesion and costosternal elevation for pectus excavatum was performed simultaneously with successful result. The postoperative course was uneventful and the patient was discharged on the twentieth postoperative day.
선천성 낭종성 선종양기 형은 말초 기관지가 심하게 분화하여 선종(adenomatoid)의 형태를 취하는 질환으로 발생 빈도는 매우 낮으나 신생아에서 급성 호흡부전을 일으키는 선천성 폐기 형중 가장 빈도가 높다. 대부분적 선천성 낭종성 선종양기 형은 단일 폐염을 침범하며 다른 선천성 질환을 동반하는 경우는 드물다. 저자들은 빈번한 호흡기 감염을 주소로 내원하여 치료를 받던중 누두흉을 동반한 선천성 낭종성 선종양기 형으로 진단된 3세의 여아를 양폐엽절제술과 변형 흉골거상술을 동시에 시행하여 양호한 성적을 얻었기에 문헌고찰과 더불어 보고하고저 한다.