Skeletal manifestations of primary oxalosis.
We describe the clinical, radiographic and histological features of skeletal involvement in four patients with end-stage renal failure due to primary oxalosis. The clinical features were unrelenting bone pain, and in two patients multiple fractures. Radiographic features were, in chronological order: (1) radiodense metaphyses and other red marrow bone; (2) cortical defects in metaphyses; (3) spontaneous fracture-separations of epiphyses of long limb bones which healed poorly. The fractures occurred through crystal deposits, and fracture displacement was associated with extrusion of crystalline material from bone. On histological examination crystals were found to replace metaphyseal bone. Pericrystalline giant cell granulomata replaced bone marrow. Erosion surfaces near granulomas were increased. Subperiosteal and intra-osseous tophi of calcium oxalate were seen. Calcium oxalate appears to precipitate with greater facility than does physiological mineral. Bone showed the features of mixed uraemic osteodystrophy in all four patients. We conclude that: (1) the fractures occurred through heavy crystal deposits; (2) ununited fractures and intra-osseous and subperiosteal tophi contributed to the pain; (3) spontaneous fractures are of poor prognostic significance. We recommend that unstable fractures be internally fixed.
Schnitzler
C M
CM
Department of Orthopaedic Surgery, School of Pathology, University of the Witwatersrand, Johannesburg, Republic of South Africa.
Kok
J A
JA
Jacobs
D W
DW
Thomson
P D
PD
Milne
F J
FJ
Mesquita
J M
JM
King
P C
PC
Fabian
V A
VA
D000293:Adolescent; D001706:Biopsy; D001842:Bone and Bones / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D002129:Calcium Oxalate / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D005598:Fractures, Spontaneous / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000378:metabolism; D008297:Male; D010146:Pain / Q000209:etiology; D011859:Radiography
D002129:Calcium Oxalate