Skeletal cystic angiomatosis with severe hip joint deformation resembling massive osteolysis.
An aggressive destruction pattern resulting in joint deformation has not been described in skeletal cystic angiomatosis (SCA) so far. We present the case of a 6-year-old boy with such findings strongly resembling Gorham's disease (massive osteolysis). Since the prognosis of the latter entity tends to be less favorable than in SCA, particularly SCA without extraskeletal involvement, careful differentiation of both disorders appears to be important.
von Knoch
F
F
Bone and Biomaterials Research Laboratory, Institute for Histology and Embryology, University of Vienna, Austria. fabianvonknoch@yahoo.com
Grill
F
F
Herneth
A M
AM
Kainberger
F
F
Lang
S
S
Ploier
R
R
von Knoch
M
M
D000798:Angiomatosis / Q000175:diagnosis*; D001847:Bone Diseases / Q000175:diagnosis*; D003560:Cysts / Q000175:diagnosis*; D006621:Hip Joint / Q000002:abnormalities; D006801:Humans; D007223:Infant; D008159:Lumbar Vertebrae / Q000002:abnormalities; D008297:Male; D010015:Osteolysis, Essential / Q000175:diagnosis*; D013117:Spinal Cord Compression / Q000175:diagnosis*; D013904:Thoracic Vertebrae / Q000002:abnormalities