Bilateral lung transplantation for pulmonary emphysema associated with cutis laxa

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...

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Bibliographic Details
Published in:JHLT Open 2024-05, Vol.4, p.100086, Article 100086
Main Authors: Matsumoto, Akira, Ohsumi, Akihiro, Handa, Tomohiro, Sato, Susumu, Katsuragawa, Hiroyuki, Date, Hiroshi
Format: Article
Language:English
Subjects:
cutis laxa
elastic fiber disorder
lobar bronchial anastomosis
lung transplantation
pulmonary emphysema
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Summary: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.
ISSN:2950-1334
2950-1334
DOI:10.1016/j.jhlto.2024.100086