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Proteus Syndrome: High-resolution CT and CT Pulmonary Densitovolumetry Findings

Irion, Klaus L. MD, PhD, FBCR, FRCR*; Hocchegger, Bruno MD; Marchiori, Edson MD, PhD; Holemans, John A. BSc, MBBS, FRCP, FRCR, FBIR*; Smith, Rupert A. MBChB, DCH, MRCP; Raja, Ramesh C. MBChB, FRCR; Singanayagam, Selvadurai MBBS, MRCP, DMRD, FRCR

doi: 10.1097/RTI.0b013e31818b20cd
Case Reports—Pulmonary

Cystic transformation of the lungs in Proteus syndrome is considered an important manifestation of this disease. We describe a case of an 11-year-old girl with a diagnosis of Proteus syndrome with lung involvement. Low-dose multidetector computed tomography (CT) revealed extensive diffuse cystic lung disease with left lung predominance, affecting mostly the lower lung zones. The cystic lesions had various sizes and variable wall thickness. Postprocessing using CT histogram densitometric volumetry software (CT densitovolumetry), using the threshold −950 Houndsfield units (HU) for quantifying emphysema, revealed that 31% of her total lung volume was composed of areas with CT attenuation values below −950 HU.

*Liverpool Heart and Chest Hospital NHS Trust, and the Royal Liverpool University Hospital, Liverpool, United Kingdom and the Post-Graduation Program in Respiratory Sciences–UFRGS, Porto Alegre

The Post-Graduation Program in Radiology, UFRJ, Rio de Janeiro, and Fluminense Federal University, Niteroi

The Pennine Acute Hospitals NHS Trust, Great Manchester, Brazil

Reprints: Klaus L. Irion, MD, PhD, 912 Manchester Road, Bury, Postcode: BL9 8DW, UK (e-mail: Klaus.irion@LHCH.NHS.UK;

© 2009 Lippincott Williams & Wilkins, Inc.