폐포 단백증 치료를 위한 전폐세척술 후 발생한 뇌부종 1예

A Case of Brain Edema Complicated by Whole Lung Lavage to Treat Pulmonary Alveolar Proteinosis

호흡곤란 및 청색증을 주소로 입원한 44세의 남자환자에서 경기관지 폐생검상 폐포내 PAS염색 양성인 인지질 축적과, 전자현미경상 적층체가 관찰되어 폐포단백증으로 진단한 환자에서 치료목적으로 전폐세척술을 시행하였으나 부작용으로 뇌부종이 발생하여 사망한 1예를 경험하였기에 문헌고찰과 함께 보고하였다.

Pulmonary alveolar proteinosis is a disease of unknown etiology characterized by the accumulation of PAS positive lipoproteinaceous material in the alveolar spaces sparing septum. The therapy which has enjoyed the greatest success is whole lung lavage. The authors reported here, a case of 44 year old male patient with pulmonary alveolar proteinosis, and this is the 7th case in Korea. The patient underwent whole lung lavage but expired due to brain edema complicated by the procedure. He complained exertional dyspnea and cyanotic lips, and presented fine inspiratory crackle at both lower lung fields, decreased arterial oxygen pressure, and diffuse infiltration at whole lung field. Light microscopic finding of lung tissue obtained by transbronchial lung biopsy revealed PAS positive amorphous, granular material filled in the alveolar spaces, and electron microscopy of bronchoalveolar lavage fluid concentrate showed many electron-dense multi-lamellated structures. To treat the disease, the authors tried whole lung lavage of left lung with $37^{\circ}C$ isotonic saline under general anesthesia. However, he expired due to brain edema probably due to dilutional hyponatremia complicated by the procedure, 11 days after the procedure. Whole lung lavage is known relatively safe, but fatal complication may occur like this case.