Bilateral lung transplantation for pulmonary emphysema associated with cutis laxa

Abstract

Cutis laxa is a rare elastic tissue disorder that mainly affects the skin and results in loss of elasticity. Occasionally, pulmonary emphysema complicates this condition. Herein, we report the first case of successful lung transplantation for severe juvenile emphysema associated with cutis laxa. The patient underwent bilateral deceased-donor lung transplantation. The patient’s lungs had very thin visceral pleura due to a deficiency of elastic fibers along with bronchomalacia and fragile bronchial cartilage. The right upper bronchus and truncus intermedius were individually anastomosed to improve bronchial healing. At the 6-month follow-up, bronchoscopy revealed successful healing of all bronchial anastomoses, and the patient’s quality of life improved. Lung transplantation is effective for treating pulmonary emphysema caused by cutis laxa.

Keywords

• cutis laxa
• lung transplantation
• elastic fiber disorder
• pulmonary emphysema
• lobar bronchial anastomosis