Journal of the Formosan Medical Association
Volume 109, Issue 5 , Pages 397-400, May 2010

Pulmonary Manifestations and Management of Proteus Syndrome

  • Chia-Ying Li

      Affiliations

    • Division of Thoracic Surgery, Department of Surgery, National Taiwan University College of Medicine, Taipei, Taiwan
    • ,
  • Yih-Leong Chang

      Affiliations

    • Department of Pathology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
    • ,
  • Wei-Chou Chen

      Affiliations

    • Division of Thoracic Surgery, Department of Surgery, National Taiwan University College of Medicine, Taipei, Taiwan
    • ,
  • Yung-Chie Lee

      Affiliations

    • Division of Thoracic Surgery, Department of Surgery, National Taiwan University College of Medicine, Taipei, Taiwan
    • Corresponding Author InformationCorrespondence to: Dr Yung-Chie Lee, Department of Surgery, National Taiwan University Hospital and National Taiwan University College of Medicine, 7 Chung-Shan South Road, Taipei, 100 Taiwan

Received 2 February 2009; received in revised form 17 April 2009; accepted 26 June 2009.

Article Outline

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.

Key Words:  bullous lesion , mutation , pneumonectomy , pneumothorax , Proteus syndrome

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References 

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PII: S0929-6646(10)60069-1

doi:10.1016/S0929-6646(10)60069-1

Journal of the Formosan Medical Association
Volume 109, Issue 5 , Pages 397-400, May 2010

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