@entity1516 of the tibia is a @entity395 with a variable history. The @entity1516 is usually not present at birth (and therefore is not truly congenital) but occurs during the first decade of life. Paget in 1891 was the first to describe a case. The etiology is unknown. @entity4786 plays a role in approximately 50% of @entity1 . In the others, hereditary and mechanical factors are debated. Various (morphologic) classification systems have been proposed (Andersen, Boyd, Crawford). Because the appearance changes during the course of the disease, all classification systems have limited value: the determining factor is the stage of the disease at which it was classified. Because of the rarity of the disease and the variability of its history, the European Pediatric Orthopaedic Society decided to carry out a multicenter study on this disease. This paper presents the epidemiologic data on the @entity1 involved. Data have been gathered on 340 @entity1 from 13 countries. Two hundred @entity1 were male (58.8%), 140 were female (41.2%). The right side was affected in 165 @entity1 (48.5%) and the left side in 172 (50.6%); 3 @entity1 had bilateral disease. Symptoms of @entity4786 were present in 54.7%. Histologic examinations in 192 @entity1 showed a nonspecific appearance in 45.3%; in 15.6% the ultrastructure resembled @entity82 , and in 39% there was histologic evidence of @entity4786 . Most of the lesions were initially localized in the middle or distal third of the tibia. In 29% the localization changed during the course of the disease.

XXXX of the tibia: history, etiology, classification, and epidemiologic data.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity82 :: ('MESH:D005355', 'Disease') :: ['fibrous dysplasia'] @entity4786 :: ('MESH:D017253', 'Disease') :: ['Neurofibromatosis', 'neurofibromatosis'] @entity395 :: ('MESH:D035583', 'Disease') :: ['rare disease'] @entity1516 :: ('MESH:D011542', 'Disease') :: ['Congenital pseudarthrosis', 'pseudarthrosis']
@entity1516 :: (MESH:D011542,Disease) :: ['Congenital pseudarthrosis']
