These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
Pubmed for Handhelds
PUBMED FOR HANDHELDS
Search MEDLINE/PubMed
Title: Spinal involvement in Pyle's disease. Author: Turra S, Gigante C, Pavanini G, Bardi C. Journal: Pediatr Radiol; 2000 Jan; 30(1):25-7. PubMed ID: 10663503. Abstract: 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 METHOD: 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.[Abstract] [Full Text] [Related] [New Search]