Metabolic Bone Disease

For 20 months, a 23-year-old woman had bone pain that traveled to all of her extremities and was unrelated to physical activity. Physical findings were normal. An isolated increase in the serum calcium level was noted. Radiographs of the left (A) and right (B) femurs revealed diffuse diaphyseal thickening. A nuclear medicine bone scan demonstrated increased activity in the diaphyseal regions of the long bones, with a slight increase in cranial uptake (C).

Steve Anderson, NP, PhD, of Corpus Christi, Tex, writes that although genetic testing was not performed, the clinical and radiographic findings were consistent with Camurati-Engelmann disease-a congenital disorder that usually becomes symptomatic early in the third decade of life. Symptoms vary but generally involve bone pain, which is thought to be caused by dense sclerotic bone that lacks space for expansion of intraosseous vessels.

Believed to be related to ribbing disease (which affects the lower extremities only), Camurati-Engelmann disease often causes a marked thickening of the distal radius. Thickening of the diaphysis of long bones is frequently accompanied by cranial involvement.

Treatment ranges from oral over-the-counter NSAIDs and prescription analgesics to surgical reaming of the intramedullary canal. Obviously, reaming of all long bones is not appropriate; however, this surgery has been effective in relieving significant pain in a single bone.

References:

FOR MORE INFORMATION:

• Beals RK. Endosteal hyperostosis. J Bone Joint Surg Am. 1976;58:1172-1173.

• Beals RK, McLoughlin SW, Teed RL, McDonald C. Dominant endosteal hyperostosis. Skeletal characteristics and review of the literature. J Bone Joint Surg Am. 2001;83-A:1643-1649.

• Beals RK, Pearson JM, Mansoor A. Ribbing disease: a case report, a review of the literature, and a description of novel treatment. J Bone Joint Surg Am. 2002;84-A:2050-2055.

• Gelman MI. Autosomal dominant osteosclerosis. Radiology. 1977;125:289-296.