Radiology, Vol 199, 233-240, Copyright © 1996 by Radiological Society of North America
Uremic leontiasis ossea: "bighead" disease in humans? Radiologic, clinical, and pathologic features
VS Lee, MS Webb Jr, S Martinez, CP McKay and GS Leight Jr
Department of Radiology, Duke University Medical Center, Durham, NC 27710, USA.
PURPOSE: To describe the clinical, radiologic, and pathologic findings in
patients with uremic leontiasis ossea (ULO). MATERIALS AND METHODS: Five
patients with renal osteodystrophy developed marked hyperostosis of the
facial and cranial bones. Radiologic studies included plain radiography of
the skull (n = 5), computed tomography with three- dimensional
reconstruction (n = 4), magnetic resonance imaging (n = 3), and fluorine-18
sodium fluoride positron emission tomography (PET) (n = 1). Specimens from
bone biopsies (three patients) were examined. RESULTS: Skull and facial
alterations were remarkably similar. Numerous nodules of varying
attenuation and signal intensity in the widened diploic space suggested
brown tumors in different stages of evolution. Biochemical data and PET
findings enabled confirmation of markedly increased bone turnover. Bone
specimens demonstrated severe osteitis fibrosa. After parathyroidectomy,
facial changes in all patients stabilized or improved mildly. CONCLUSION: A
similar entity in animals, "bighead" disease, which results from
nutritional and uremic secondary hyperparathyroidism, may provide a useful
animal model for ULO in humans. Mild forms of this entity may be more
common than the scarcity of previous reports suggests.
