Proteus syndrome: high-resolution CT and CT pulmonary densitovolumetry findings.
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.
Irion
Klaus L
KL
Liverpool Heart and Chest Hospital NHS Trust, Royal Liverpool University Hospital, Liverpool, United Kingdom. Klaus.irion@LHCH.NHS.UK
Hocchegger
Bruno
B
Marchiori
Edson
E
Holemans
John A
JA
Smith
Rupert A
RA
Raja
Ramesh C
RC
Singanayagam
Selvadurai
S
D002648:Child; D005260:Female; D006801:Humans; D008171:Lung Diseases / Q000000981:diagnostic imaging*; D016715:Proteus Syndrome / Q000000981:diagnostic imaging*; D011857:Radiographic Image Interpretation, Computer-Assisted; D013902:Radiography, Thoracic; D014057:Tomography, X-Ray Computed / Q000379:methods*