caa a22 4500 475754387 CHVBK 20180406123534.0 cr unu---uuuuu 170329e20000101xx s 000 0 eng 10.1007/s002470050006 doi (NATIONALLICENCE)springer-10.1007/s002470050006 Spinal involvement in Pyle's disease [Elektronische Daten] [S. Turra, C. Gigante, G. Pavanini, C. Bardi] Background. Pyle's disease is a rare skeletal dysplasia involving the metaphyses of long bones. To date, spinal involvement has been only rarely described in the literature.¶Objective. To show that spinal changes, which are an expression of the same growth defect of the long bones, are an important and constant sign of the disease in the families studied.¶Materials and methods. The radiographic skeletal changes in five patients have been observed.¶Results. The pathognomonic metaphyseal widening of tubular bones (known as the 'Erlenmeyer flask sign') was associated with the spinal changes in all cases. Radiographic findings in the spine varied from moderate platyspondyly to the bodies having the appearance of a biconcave lens. This may be attributed to two main causes: (1) a defect in the modelling process of the vertebrae (comparable to that observed in the metaphyses) and (2) chronic pathological fractures secondary to osteoporosis (a typical feature of Pyle's disease).¶Conclusions. The finding of platyspondyly of varying severity widens the spectrum of radiographic findings in this disease and can assist in diagnosis. Springer-Verlag Berlin Heidelberg, 2000 Turra S. Istituto di Clinica Ortopedica, Università degli Studi di Padova, Via Giustiniani, I-35128 Padua, Italy, IT aut Gigante C. Istituto di Clinica Ortopedica, Università degli Studi di Padova, Via Giustiniani, I-35128 Padua, Italy, IT aut Pavanini G. Istituto di Clinica Ortopedica, Università degli Studi di Padova, Via Giustiniani, I-35128 Padua, Italy, IT aut Bardi C. Istituto di Clinica Ortopedica, Università degli Studi di Padova, Via Giustiniani, I-35128 Padua, Italy, IT aut https://doi.org/10.1007/s002470050006 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s002470050006 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Turra S. Istituto di Clinica Ortopedica, Università degli Studi di Padova, Via Giustiniani, I-35128 Padua, Italy, IT aut NATIONALLICENCE 700 1- Gigante C. Istituto di Clinica Ortopedica, Università degli Studi di Padova, Via Giustiniani, I-35128 Padua, Italy, IT aut NATIONALLICENCE 700 1- Pavanini G. Istituto di Clinica Ortopedica, Università degli Studi di Padova, Via Giustiniani, I-35128 Padua, Italy, IT aut NATIONALLICENCE 700 1- Bardi C. Istituto di Clinica Ortopedica, Università degli Studi di Padova, Via Giustiniani, I-35128 Padua, Italy, IT aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer