Bone Dysplasias

Bone Dysplasias

 

a group of mostly congenital diseases of bone characterized by malformation of various parts of the skeleton. In bone dysplasias, one or more bones and sometimes the entire skeleton is affected, and the bones can be partially or completely underdeveloped. Gigantism is a type of bone dysplasia.

The commonest type is fibrous dysplasia, which usually arises in childhood or adolescence and can have a prolonged latent period. It is characterized by abnormal focal proliferation of osteogenic tissue in the bones, while the adjacent bony tissue’s structure remains normal. The affected bones are deformed; for example, tubular bones become twisted. Fractures occasionally occur and heal poorly. The course of fibrous dysplasia is chronic and generally benign.

References in periodicals archive ?
It is rarely associated with other sclerosing bone dysplasias such as melorheostosis and osteopoikilosis.
Sclerosing bone dysplasias: genetic and radioclinical features.
(1) It belongs to a group of conditions known as sclerosing bone dysplasias. (2) Albers-Schonberg first described osteopoikilosis in 1915.
The differential diagnosis of medullary sclerosis is broad, including malignancies (osteosarcoma, lymphoma, osteoblastic metastasis), benign tumors (osteoid osteoma), infections (chronic osteomyelitis), trauma (healing stress fractures), metabolic disorders, several sclerosing bone dysplasias, and acquired syndromes with increased bone density that include Erdheim-Chester disease, myelofibrosis, and sickle cell disease.
Sclerosis bone dysplasias are a heterogeneous group of genetic disorders resulting in errors of bone metabolism that often present with pain of the extremity involved.
Bone Dysplasias: An Atlas of Genetic Disorders of Skeletal Development.
Bone dysplasias. In Atlas of genetic disorders of skeletal development.
After birth, the differential diagnosis is made with syndromes that present bone dysplasia. Spondyloepiphyseal dysplasia congenita is characterized by a very short stature with shortening of all bones.
Osteopoikilosis (osteopathia condensans disseminate, spotted bone; OPK) is an asymptomatic, rare bone dysplasia. This disorder was first described by Alberg Schonberg in 1915.
Increased bone density without modifcation of bone shape Osteopetrosis precocious type Generalised AR delayed type Type 1 uniform AD intermediate type Type 2 endobones with renal tubular Generalised AR AR acidosis Similar other types Axial osteosclerosis osteomesopyknosis Focal sclerosis AD AR with bamboo hair in vertebrae/pelvis Pycnodysostosis Generalised AR Osteosclerosis Cortical AD Stanesu type thickening of long bones, defcient facial sinus development Osteopathia striata Radiodense SP XLD isolated with striations on all cranial sclerosis bones with cranial sclerosis Sponastrime Striated AR dysplasia metaphysis Melorheostosis Flowing SP hyperostosis Osteopoikilosis Radiodense spots AD Mixed sclerosing Combined pattern SP bone dysplasia 2.
Osteopoikilosis (osteopathia condensans disseminata) is a rare sclerosing bone dysplasia of unknown etiology.