Abstract
Anatomico-pathological and radiological studies of osteopoikilosis were performed in two cases, one involving a femoral head, excised after a fracture of a femoral neck, in an elderly man and the other following biopsy of an iliac crest in a young woman. In both patients wide-spread radiological evidence of the disorder was present as an incidental finding. The radiological appearance of rounded and linear densities corresponded to old and inactive remodelling of spongy trabeculae in epiphyseal and metaphyseal locations. The distribution and appearance of these osteopoikilotic densities suggested them to have been related intimately to mechanical strain on spongy bone trabeculae. The diffuse nature of the lesions, their hereditary character, and their possible association with abnormalities of the skin suggest the existence of a particular terrain in which general metabolic conditions of connective tissue may interact with mechanical stresses in bone. Careful analysis of the findings in osteopoikilosis is desirable in order to provide data concerning the physiopathology of the skeleton and to permit more definitive interpretation of localised areas of bone condensation, including those observed not only in solitary bone islands and such conditions as osteopathia striata, but also those associated with infections and tumours.
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References
Archer MC, Fox KW (1946) Osteopoikilosis: Report of two cases. Radiology 47:279
Atherton DJ, Wells RS (1982) Juvenile elastoma and osteopoikilosis (the Buschke-Ollendorf syndrome). Clin Exp Dermatol 7:109
Baumann-Schenker R, Uehlinger E (1947) Über tubuläre Sklerose des Skeletts. Radiol Clin 16:221
Bethge JFJ, Ridderbusch KE (1967) Über Osteopoikilie und das neue Krankheitsbild Hyperostose bei Osteopoikilie. Ergeb Chir Orthop 49:138
Bonjean P, Tressaud A (1973) Application de la microsonde électronique à l'étude de la bio-mécanique de l'os. CR Acad Sci Paris 277:2243
Brücke H (1933) Über multiple Enostosen (Osteopoikilie). Dtsch Z Chir 239:554
Carlsen DH (1977) Osteopathia striata revisited. J Can Assoc Radiol 28:190
Claus HG (1964) Gibt es eine streifige Form der Osteopoikilie? RÖFO 101:522
Edström G (1942) Osteopoikílie und Sklerodermie bei einem Fall von Polyarthritis rheumatica chronica. Acta Chir Scand 87:117
Funstein L, Kotschiew K (1936) Über die Osteopoikilie. RÖFO 54:595
Grassberger A, Seyss R (1975) Knochenszintigramm bei Osteopoikilosis familiaris. Radiol Clin 44:372
Holly LE (1936) Osteopoikilosis. A five year study. AJR 36:512
Jaffe HL (1972) Metabolic, degenerative and inflammatory diseases of bones and joints. Urban and Schwarzenberg, München Berlin Wien
Jonasch E (1955) 12 Fälle von Osteopoikilie RÖFO 82:344
Lagier R (1983) L'ilot osseux bénin solitaire. Etude anatomo-radiologique d'un cas. Radiologie J CEPUR 3:125–127
Lagier R, de Montmollin B, Krucker V (1984) Parcellar algodystrophy of the femoral head (i.e., localized (focal) transient osteoporosis) associated with synovial chondromatosis of the hip. Case Report 256. Skeletal Radiol 11:69
Lagier R, Nusslé D (1978) Anatomy and radiology of a bone island. RÖFO 128:261–264
McKusick VA (1972) Heritable disorders of connective tissue. Mosby, Saint Louis
Mindell ER, Northup CS, Douglass HO (1978) Osteosarcoma associated with osteopoikilosis. Case report. J Bone Joint Surg [Am] 60:406
Resnick D, Niwayama G (1981) Enostosis, hyperostosis and periostitis. In: Resnick D, Niwayama G (eds) Diagnosis of bone and joint disorders. Sauders, Philadelphia London Toronto, p 2962
Schai E (1975) Drei Fälle von Osteopoikilie. Z. Rheumatol 34:27
Schmorl G (1931) Anatomische Befunde bei einem Falle von Osteopoikilie. RÖFO 44:1
Stubenrauch LV (1938) Zur Histogenese der Osteopoikilie. Mikroskopische Untersuchungen. Dtsch Z Chir 250:586
Szabo AD (1971) Osteopoikilosis in a twin. Clin Orthop 79:156
Uitto J, Santa Cruz DJ, Starcher BC, Whyte MP, Murphy WA (1981) Biochemical and ultrastructural demonstration of elastin accumulation in the skin lesions of the Buschke-Ollendorff syndrome. J Invest Dermatol 76:284
Verbov J (1977) Buschke-Ollendorff syndrome (disseminated dermatofibrosis with osteopoikilosis). Br J Dermatol 96:87
Whyte MP, Murphy WA, Siegel BA (1978) 99mTc-pyrophosphate bone imaging in osteopoikilosis, osteopathia striata and melorheostosis. Radiology 127:439
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Lagier, R., Mbakop, A. & Bigler, A. Osteopoikilosis: A radiological and pathological study. Skeletal Radiol 11, 161–168 (1984). https://doi.org/10.1007/BF00349489
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DOI: https://doi.org/10.1007/BF00349489