肺移植逆转胸膜实质纤维弹性组织增生症的扁平胸

Flat Chest of Pleuroparenchymal Fibroelastosis Reversed by Lung Transplantation.

作者信息

Yanagiya Masahiro, Sato Masaaki, Kawashima Shun, Kuwano Hideki, Nagayama Kazuhiro, Nitadori Jun-Ichi, Anraku Masaki, Nakajima Jun

机构信息

Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.

出版信息

Ann Thorac Surg. 2016 Oct;102(4):e347-9. doi: 10.1016/j.athoracsur.2016.02.092.

DOI:10.1016/j.athoracsur.2016.02.092

PMID:27645979

Abstract

A patient with pleuroparenchymal fibroelastosis (PPFE) was successfully treated with living-donor lobar lung transplantation. A 27-year-old woman with a 3-month history of dyspnea received a diagnosis of PPFE. Her chest wall was extremely flattened over time, and her respiratory condition progressively deteriorated. She underwent semielective bilateral living-donor lobar lung transplantation. Her chest wall rigidity, which was secondary to PPFE, required intensive pulmonary rehabilitation postoperatively. By 6 months after transplantation, the flattening of her chest wall was reversed. Living-donor lobar lung transplantation was a life-saving procedure for this patient and improved the chest wall deformity of PPFE.

摘要

一名胸膜实质纤维弹性组织增生症（PPFE）患者通过活体供体肺叶移植获得成功治疗。一名有3个月呼吸困难病史的27岁女性被诊断为PPFE。随着时间推移，她的胸壁极度扁平，呼吸状况逐渐恶化。她接受了半择期双侧活体供体肺叶移植。她因PPFE继发的胸壁僵硬在术后需要强化肺部康复治疗。移植后6个月，她胸壁的扁平状态得到了逆转。活体供体肺叶移植对该患者来说是一种挽救生命的手术，并改善了PPFE的胸壁畸形。