Genant, H. K.

Kottamasu, S. R.

Meema, H. E.

Rao, D. S.

English

Henry Ford Health System Scholarly Commons

Henry Ford Hospital Medical Journal Volume 31 Number 4 Article 20 12-1983 Radiology of Metabolic Bone Disease: Workshop Report S. R. Kottamasu D. S. Rao H. E. Meema H. K. Genant Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal  Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Kottamasu, S. R.; Rao, D. S.; Meema, H. E.; and Genant, H. K. (1983) "Radiology of Metabolic Bone Disease: Workshop Report," Henry Ford Hospital Medical Journal : Vol. 31 : No. 4 , 239-243. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol31/iss4/20 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Henry Ford Hosp Med J Vol 31, No 4,1983 Radiology of Metabolic Bone Disease: Workshop Report S.R. Kottamasu, MD,* D.S. Rao, MD, * * H.E. Meema, M D , * * * and H.K. Genant, M D * * * * fd. Note-This workshop was originally given at the International Symposium on Clinical Disorders of Bone and Mineral Metabolism, May 9-13, 1983. S.R . Kottamasu illustrated an overview of metabolic bone disease with several clinical and radiologic case studies. Case 1 A 55-year-old man with pain and limitation of motion of the left lower extremity. Radiographs demonstrated hyperostosis of the left hemipelvis, femur, tibia, and medial rays of the foot. The findings in the hemipelvis resembled Paget's disease, although a wavy and sclerotic bone contour extending along one side of tubular bones, like f lowing candle wax, is characteristic of MELORHEOSTOSIS (Figs. 1,2). Fig. 2 Case 1 . M e l o r h e o s t o s i s . F i g . 1 Case 1 . M e l o r h e o s t o s i s . •Depar tment of Diagnostic Radiology, Henry Ford Hospital • •Depar tment of Internal Med i c i ne , Bone and Mineral Metabol ism Div is ion, Henry Ford Hospital • • • D e p a r t m e n t of Diagnostic Radiology, Toronto General Hospital, To ron to , Ontar io • • • • D e p a r t m e n t of Diagnostic Radiology, University of Cal i fornia, San Francisco Address repr int requests to Dr. Kottamasu, Department of Diagnostic Radiology, Henry Ford Hospital, 2799 W Grand Blvd, Detro i t , M l 48202. 239 Kottamasu, Rao, Meema and Genant Case 2 A 15-year-old boy with blunt trauma to the foot. Radio-graphs demonstrated numerous, well-circumscribed foci of increased density in various bones, an incidental f ind-ing, characteristic of OSTEOPOIKILOSIS (Fig. 3). Pathologically, the focal sclerotic areas were foci of compact bone in spongiosa. Case 3 A 5-year-old boy with waddling gait and muscular weak-ness. Radiographs of lower extremities showed symmet-ric cortical thickening involving mid-diaphyses of fem-ora (Fig. 4). Radiographs of a 40-year-old man, father of Case 3, demonstrated sclerosis and marked cortical thickening with increase in the outer diameter of the bones. Meta-physes and epiphyses were conspicuously unaffected (Fig. 5). The findings were characteristic of ENGEL-MANN'S DISEASE (progressive diaphyseal dysplasia). Case 4 Radiographs of the spine in a 45-year-old man showed typical "bone-w i th in -bone" appearance. One of the classic conditions in the differential diagnosis of this radiologic f inding is OSTEOPETROSIS. Subsequent radi-ographs demonstrated generalized osteosclerosis, ab-sence of corticomedullary demarcation, and pathologic fractures of the proximal femur. Case 5 Rickets in osteopetrosis. Some children with the congen-ital form of osteopetrosis develop radiologic changes of Fig. 4, Case 3. Fig. 3 Case 2. Osteopoikilosis. Fig. 5 Engelmann's Disease. 240 Radiology of Metabolic Bone Disease rickets and abnormal biochemical changes (low serum calcium, phosphorus, and increased alkaline phospha-tase). The pathogenesis is not well understood, and treatment is unsatisfactory. Case 6 A 55-year-old man with pain in the left lower thigh. Radiographs of the femur demonstrated mult iple lytic lesions involving the distal femur. Some of the lesions were intracortical, and the lesions extended to the distal articular surface of the femur. The proximal end of the lesion had a "blade of grass" appearance, characteristic of the osteolytic phase of Paget's disease, OSTEOPORO-SIS CIRCUMSCRIPTA. Case 7 Radiographs demonstrated multiple lytic lesions in the fronto-parietal region of the skull. Follow-up radio-graphs showed sclerosis and thickening of bone at the sites of previous lytic lesions (Fig. 6). This case demonstrated spontaneous progression o f the lytic phase of FIBROUS DYSPLASIA to the more com-monly recognized sclerotic phase. This transition is less frequently documented in fibrous dysplasia than in Paget's disease. Case 8 A 45-year-old woman with skin rash and hepatomegaly. Skeletal radiographs demonstrated generalized mixed osteosclerotic and lytic pattern. This, associated with a brownish macular skin rash that urticated and hepato-splenomegaly, suggested the possibility of SYSTEMIC MASTOCYTOSIS (Fig. 7). Of patients with mastocytosis, 15-20% have detectable bone lesions. Skeletal lesions in mastocytosis may be Fig. 7 Case 8. Systemic Mastocytosis. lytic, sclerotic, or mixed, and they may demonstrate focal, multifocal, or diffuse distr ibution. Case 9 A 60-year-old man with generalized and progressive, severe skeletal pain and tenderness. Serum chemistries were normal except for a modest elevation of alkaline phosphatase (Fig. 8). Radiographs demonstrated indistinct cortices, coarse trabecular pattern, and spotty areas of increased bone density. Outer diameters of all the bones of the hands were increased symmetrically, a feature characteristic of FIBROGENESIS IMPERFECTA OSSIUM. There was loss of birefringence of collagen fibers on polarizing microscopy. Fig. 6 Case 7. Fibrous Dysplasia. 241 Kottamasu, Rao, Meema and Genant Fig. 9 Case 10. Osteosclerotic Mul t ip le Myeloma. Fig. 8 Case 9. Fibrogenesis Imperfecta Ossium. Case 10 A 60-year-old with generalized osteosclerosis and lytic lesions involving two ribs. A bone scan showed only minimal increased uptake in the lytic lesions in the ribs. Serum protein electrophoretic pattern was normal. Bone biopsy demonstrated an OSTEOSCLEROTIC FORM OF MULTIPLE MYELOMA (Figs. 9,10). Table I lists the major causes of generalized osteosclerosis. Case 11 A 35-year-old man presented with a pathologic fracture through a lytic lesion in the right tibia. The skeletal sur-vey demonstrated additional lytic lesions involving the left tibia and fibula. Follow-up films in six months showed SPONTANEOUS HEALING OF BROWN TUMORS WITH SCLEROSIS FOLLOWING PARATHYROIDECTOMY. Fig. 10 Case 10. Osteosclerotic Mul t ip le Myeloma. 242 Radiology of Metabolic Bone Disease Case 12 A 14-year-old girl with bilateral slipped capital femoral epiphysis. Subperiosteal resorption involved the pha-langes of the hand. There was a lytic lesion in the left distal clavicle, consistent with a Brown tumor. The laboratory findings revealed persistent hypocalcemia, elevation of serum phosphorus, alkaline phosphatase and iPTH, and resistance to the administration of exogenous PTH. This case represented RENAL RESISTANCE TO PARA-THYROID HORMONE WITH OSTEITIS FIBROSA (pseudo-hypo-hyper parathyroid ism). Discussion H.E. Meema discussed the periosteal and juxtaperiosteal components of hyperparathyroid bone resorption in chronic renal failure. Subperiosteal resorption in finger phalanges is a pathognomonic sign of hyperparathyroid-ism. It is generally considered to represent centripetal osteoclastic bone resorption f rom the periosteal sur-faces, i.e. surface erosion. This does not fully explain why in some cases there remains a thin remnant of periosteal bone covering large juxtaperiosteal resorption spaces. A longitudinal microradioscopic study of the evolution of subperiosteal resorption in patients with chronic renal failure showed increased juxtaperiosteal resorption (a variant of intracortical resorption) in many areas where subperiosteal resorption subsequently developed. It thus appears that intracortical resorption correlates sig-nificantly with the extent of elevation in iPTH, although the correlation is higher for subperiosteal than for intra-cortical resorption. H.K. Genant reviewed the clinical applications of com-puted tomography o f t h e spine. TABLE I Differential Diagnosis of Generalized Osteosclerosis Osteoblastic metastatic bone disease Hyperparathyroidism -primary or secondary Myelosclerosis Osteopetrosis Lymphoma Pyknodysostosis 7. Fluorosis 8. Systemic mastocytosis 9. Tuberous sclerosis 10. Van Buchem's disease 11. Osteosclerotic mult iple myeloma 12. Fibrogenesis imperfecta ossium The normal ranges of vertebral mineral and age-related bone losses were determined from cross-sectional studies of 120 normal men and 203 normal women, aged 20-80 years. The normal mean value for young men and women was approximately 175 mg/cm. M e n , by a linear regression, lost an average of 0.84%/year, and women, by a cubic regression, lost an average of 1.2%/year. Cor-recting for age-related bone marrow fat changes altered the observed rates of loss only by about 10%. Cross-sectional studies in 80 perimenopausal women showed an average of 5.6%/year vertebral cancellous-mineral loss by QCT compared with 3%/year peripheral cortical mineral loss by photon absorptiometry and radio-grammetry. Longitudinal studies in 31 women fol lowing oophorectomy showed an average 9%/year vertebral cancellous bone loss, and peripheral cortical bone loss was 2-3%. Cross-sectional studies in 25 young women with various forms of amenorrhea showed an average decrement from normal of 30% in vertebral mineral values and 10% in peripheral cortical values. 243 

Radiology of Metabolic Bone Disease: Workshop Report

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Radiology of Metabolic Bone Disease: Workshop Report

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