Proteus syndrome: a case report.

[proteus syndrome]

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 .