Pulmonary manifestations and management of proteus syndrome.
Proteus syndrome is a very rare, sporadic and congenital condition that is characterized by postnatal mosaic overgrowth. This disorder is thought to be caused by a somatic gene mutation, but the exact etiology is unknown. Commonly involved tissues include connective tissue, bone, skin and the central nervous system. Another less common symptom involves pulmonary emphysematous changes. This report documents a 25-year-old man with Proteus syndrome who presented with progressive exertional dyspnea and asymmetric overgrowth of his extremities. He underwent left pneumonectomy and his postoperative course was uneventful. Lung tissue showed emphysematous changes with multiple bulla formation and scattered calcification. We also review recent literature related to pulmonary manifestations and management of Proteus syndrome.
Li
Chia-Ying
CY
Division of Thoracic Surgery, Department of Surgery, National Taiwan University College of Medicine, Taipei, Taiwan.
Chang
Yih-Leong
YL
Chen
Wei-Chou
WC
Lee
Yung-Chie
YC
D000328:Adult; D006801:Humans; D008168:Lung / Q000473:pathology / Q000601:surgery*; D008297:Male; D016715:Proteus Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D011656:Pulmonary Emphysema / Q000150:complications / Q000000981:diagnostic imaging / Q000209:etiology*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome