Proteus syndrome: a case report.
Proteus syndrome is a rare, sporadically occurring hamartomatous disorder with complex multisystem involvement and wide clinical variability. Clinical characteristics include craniofacial abnormalities; asymmetrical overgrowth of the trunk, limbs, and digits; lipomas; and vascular malformations. Cystic lung disease is noted in approximately 10 per cent of patients. These cystic malformations may lead to cystic pulmonary emphysema, which may cause significant morbidity for the patient. We describe the case of a 27-year-old woman with known Proteus syndrome who developed acute on chronic respiratory failure. Subsequent CT scan of the chest was significant for extensive bullous disease with nearly complete replacement of the right lung tissue. Our operative management and the patient's clinical course are described.
Zusan
Erin
E
Department of Surgery, Good Samaritan Hospital, Cincinnati, Ohio 45220, USA.
Smith
J Michael
JM
Parker
Thomas
T
D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D011013:Pneumonectomy / Q000379:methods*; D016715:Proteus Syndrome / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D013902:Radiography, Thoracic; D012131:Respiratory Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D014057:Tomography, X-Ray Computed