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At least 19 recordsLinked to original sources
PubMed1990

Radiographic findings in hereditary multiple exostoses and a new theory of the pathogenesis of exostoses.

Analysis of 330 exostoses in 18 patients affected by hereditary multiple exostoses disease suggested a new classification of exostoses as eccentric or full-thickness. Radiographically arrest of metaphyseal remodeling with failure of coning and persistence of the primary metaphyseal trabeculae was evident in full-thickness exostoses. Similar bone lesions can be obtained experimentally with inhibitors of bone turn-over. A localized, peripheral defect in remodeling over a limited time can give a satisfactory explanation also for the origin of eccentric exostoses. The thesis that this is the basic mechanism of exostosis formation is presented.

Adolescent↗
PubMed1991

[Costal exostoses, complicated in the neonatal period, by brachial plexus paralysis. A distinct entity of exostoses?].

Two highly unusual cases of brachial plexus palsy due to compression by exostosis of the first rib in the neonatal period are reported. Etiologic diagnosis in these patients required elimination of other tumors of the first rib, including multiple exostoses. The contradictions found lead the authors to suggest individualization of a form of multiple exostoses different from classical multiple exostoses by a number of features including growth, complications, and inheritance. At present, it is not known whether this new entity carries the same risk of malignant transformation as classical multiple exostoses.

Bone Neoplasms↗
PubMedDate not supplied

Exostoses of the external auditory canal.

Exostosis of the external ear canal is a disease unique to man. It has been identified in prehistoric man, affecting the aborigines of the North American continent. Aural exostoses are typically firm, sessile, multinodular bony masses which arise from the tympanic ring of the bony portion of the external auditory canal. These growths develop subsequent to prolonged irritation of the canal. The large, primitive jaw of prehistoric man placed great mechanical stress on the tympanic ring. Chronic aural suppuration seen in the preantibiotic era was soon followed by exostoses. Today, prolonged contact of the external ear canal with cold sea water is the most prevalent cause (aquatic theory). As a result the disease is now essentially limited to coastal regions. In this way we have seen exostoses appear in different stages of the evolution of man as a result of mechanical, chemical and now thermal irritation. The author is an otolaryngologist in a coastal region. In examining 11,000 patients during a ten-year period, 70 cases of symptomatic exostoses of the external auditory canal were identified. The incidence of exostoses was found to be 6.36 per 1,000 patients examined for otolaryngologic disease. It is a predominantly male disease. The development of these "irritation nodules" is painless until the tenth year of aquatic exposure to irritation, when symptoms of obstruction occur. The hearing loss associated with exostoses is usually a conductive type, secondary to occlusion of the canal by impacted cerumen or acute external otitis. The results of studying the thermal characteristics of the body of water used for such aquatic activities is presented.

Adolescent↗
PubMed1991

Behavior-induced auditory exostoses in imperial Roman society: evidence from coeval urban and rural communities near Rome.

Presence and features of auditory exostoses were investigated in two cranial samples of Roman imperial age (1st-3rd century A.D.). The skeletal material comes from the necropolises of Portus (Isola Sacra) and Lucus Feroniae (Via Capenate), two towns along the Tevere River, in close relation with the social and economic life of Rome. Deep-rooted differences between the human communities represented by the skeletal samples (83 and 71 individuals, respectively, in this study) are documented both historically and archaeologically. The results show lack of exostoses in the female sex, a negligible incidence among the males of Lucus Feroniae, but a high frequency in the male sample from Isola Sacra (31.3%). Auditory exostoses are commonly recognised as localized hyperplastic growths of predominantly acquired origin. Features of the exostoses found in the male crania from Isola Sacra (particularly in relation to the age at death of the affected individuals) support this view. Furthermore, several clinical and anthropological studies have pointed out close links between the occurrence of auditory exostoses and prolonged cold water exposure, generally due to the practice of aquatic sports, or to working activities involving water contact or diving. In this perspective, the differences observed between the two Roman populations and between the sexes (in Isola Sacra) appear to result from different social habits: the middle class population of Portus habitually used thermal baths, whereas it is probable that thermae were seldom frequented (if at all) by the Lucus Feroniae population represented in the necropolis (mostly composed by slaves or freedmen farm laborers).(ABSTRACT TRUNCATED AT 250 WORDS)

Baths↗
PubMed1989

[Multiple cartilaginous exostoses].

Multiple cartilaginous exostosis (MKE) is an autosomally dominant hereditary disease with great individual expression. The disease becomes apparent most frequently at the age of 2-5 years and approximately 80% of the cases have been diagnosed before the age of ten years. The exostoses continue to grow until shortly after puberty. The exostoses may give rise to severe deformation of the skeleton particularly around the knee, ankle and wrist joints. Reduction of joint mobility is frequently observed either on account of the size of the exostoses and their localization or on account of the joint deformity. Treatment of MKE is surgical and corrective surgery with removal of symptom-producing exostoses is the commonest intervention. Osteotomies or introduction of joint-prostheses may prove necessary in cases with the severest joint deformities. The exostoses may undergo malignant transformation, most frequently to chondrosarcomata which grow relatively slowly and metastasize late. Treatment of these must be undertaken in a tumour centre where limb-saving tumour surgery is available in cooperation between the orthopaedic surgeon and the oncologist. Families with MKE should be given patho-genetic advice.

Adult↗
PubMed1986

The relationship between auditory exostoses and cold water: a latitudinal analysis.

The frequency of auditory exostoses was examined by latitude. It was found that discrete bony lesions of the external auditory canal were, with very few exceptions, either absent or in very low frequency (less than 3.0%) in 0-30 degrees N and S latitudes and above 45 degrees N. The highest frequencies of auditory exostoses were found in the middle latitudes (30-45 degrees N and S) among populations who exploit either marine or fresh water resources. Clinical and experimental data are discussed, and these data are found to support strongly the hypothesis that there is a causative relationship between the formation of auditory exostoses and exploitation of resources in cold water, particularly through diving. It is therefore suggested that since auditory exostoses are behavioral rather than genetic in etiology, they should not be included in estimates of population distance based on nonmetric variables.

California↗
PubMed1979

Exostoses induced by 224Ra (ThX) in children.

Exostoses are benign cartilaginous tumors of bone. They can occur naturally or be induced by radiation therapy during the time of skeletal growth. We have observed exostoses in 28 of 218 children given repeated injections of radioactive bone-seeking 224Ra. The younger the age at irradiation, the higher the incidence of exostoses. Boys are more susceptible than girls. To our knowledge, none of these radiation-induced exostoses have become malignant, although 36 of these children have developed bone sarcomas elsewhere in the skeleton.

Adolescent↗
PubMed1989

[A combination of multiple cartilaginous exostoses and enchondromatosis of bone in a family].

Four cases of multiple cartilaginous exostoses in a family (mother and three children) are described. In two children the exostoses are combined with enchondromatosis of the large tubular bones. At some phalanges of these children the changes show the roentgenologic signs of solitary enchondromas and ecchondromas and in one of the cases with cartilaginous exostoses osteochondroma-like changes were found. The combination of the described symptoms seems to indicate a close relationship between enchondromatosis and the cartilaginous exostoses, as well as between these chondrodysplasias and the cartilaginous tumours.

Adult↗
PubMed1987

Experimental lathyrism: exostoses and aneurysmal-like bone cysts of the mandible in the rat.

The effect of chronic lathyrism on the mandible of the rat was studied. Exostoses, thinning and discoloration of the cortical plates, intraosseous bleeding and hemorrhagic cyst were observed after six weeks of administration of beta-aminoproprionitrile fumarate. The exostoses consisted of homogeneous cellular fibrous tissue containing osteoid, hyalin-like material and abundant ground substance. The cysts were filled with erythrocytes and lined by spindle-shaped fibroblasts, collagen fibers or osteoblast-like cells. Between the cysts, bands and sheets of dense and cellular connective tissue presenting foci of hemorrhage, osteoid and metaplastic cartilage were found. Numerous mast cells were demonstrated with alcian and toluidine blue in the hematopoietic bone marrow, cancellous bone and around the capillary network surrounding the exostoses and cysts. Exostoses have already been studied and explained by the action of BAPN (lysyl oxidase). Hemorrhagic cysts could be explained by histamine release from the mast cells (hypervascularity, hyperemia, increased permeability, rupture and progressive confluence of these small hemorrhagic cysts). Histopathology of the experimental cysts had some points in common with the human aneurysmal bone cyst. Could the pathogenic mechanisms be similar for both lesions?

Aminopropionitrile↗
PubMed1986

Subacromial impingement syndrome in hereditary multiple exostoses.

An unusual cause of subacromial pain was observed in a 32-year-old woman with multiple exostoses. The pain was the direct result of the mechanical impingement syndrome from these exostoses. Hereditary multiple exostoses should be investigated systematically, because many other, more common pain-producing conditions are possible. Secondary malignant degeneration of one of the cartilaginous coverings of the exostoses is very rare.

Acromion↗
PubMed1989

Acute spinal cord compression in hereditary multiple exostoses: case report.

A case of hereditary multiple exostoses with spinal cord compression by a costal exostosis is reported in a 12-year-old boy. Paraplegia is an unusual complication of hereditary multiple exostoses. In the patient with spinal cord signs, the offending exostoses should be defined with appropriate roentgenograms and myelographic and CT scanning investigations.

Adolescent↗
PubMed1992

The gene for hereditary multiple exostoses does not map to the Langer-Giedion region (8q23-q24).

Hereditary multiple exostoses is a dominantly inherited skeletal disorder which alters enchondral bone during growth and is characterised by exostoses of the juxta-epiphyseal regions. Using polymorphic DNA probes, we have been able to exclude the disease gene from close proximity to the 8q24.1 region where a dominant syndrome with multiple exostoses, the trichorhinophalangeal syndrome type II (TRP II, Langer-Giedion syndrome, MIM 15025), has been previously localised (pairwise linkage Z = -8.96 at theta = 0 with probe L48 at locus D8S51). Multipoint linkage analysis using probes L48, L24, and L1 consistently excluded the HME gene from a large area of the distal long arm of chromosome 8, spanning the smallest region of overlap assigned to the TRP II gene. These studies support the clinical view that HME and TRP II are distinct entities.

Chromosomes, Human, Pair 8↗
PubMed1975

Multiple cartilaginous exostoses in the dog.

The clinicopathologic aspects of multiple cartilaginous exostoses (MCE) in 2 dogs were compared with those in 9 previously reported cases of MCE in dogs. Although a familial tendency is probable, there is apparently no sex or breed predilection. Only bones that developed by endochondral ossification were affected. The vertebrae, ribs, and long bones were the most frequent location of exostoses. Bones of the carpal and tarsal joints and the skull were not affected in the cases evaluated. Physical examination and radiography may provide strong supportive evidence for a diagnosis of MCE, but a definitive diagnosis must be based on microscopic evaluation of osseous lesions. Treatment is unnecessary unless growth of exostoses results in clinical sequelae. Surgical extirpation of lesions should be considered if dysfunction of the skeletal, muscular, or neurologic systems develops. The prognosis is variable, being dependent on the location and number of lesions, the age of the patient at the time of diagnosis, and the presence or absence of clinical complications.

Animals↗
PubMed1979

Hereditary multiple exostoses: clinicopathologic features of a comparative study in horses and man.

Investigation of hereditary multiple exostoses in horses under controlled research conditions for 10 years and epidemiologic studies that have spanned up to five generations of human families contain notable similarities. The present study demonstrated that a single dominant autosomal gene is responsible for hereditary multiple exostoses in horses and man. Affected individuals transmit this trait to approximately 50% of their progeny, whereas nonaffected individuals do not transmit the condition to their offspring. The tumors in affected horses are most often present at birth. They tend to be bilaterally symmetrical and vary in size, shape, and texture. Those on the legs generally do not appear to enlarge as the animal matures, but others, notably those on the ribs and scapulae, enlarge until skeletal maturity, Histologically, the tumors appear as typical ostosteochondromas in both horse and man. Sarcomatous transformations have not yet been detected after 10 years in horses, although such changes are occasionally reported in the similar disease condition in man. The remarkable similarities of hereditary multiple exostoses in the horse to that in man provide an opportunity for comparative biomedical study.

Animals↗
PubMed1979

Hereditary multiple exostoses with myelopathy.

A 58-year-old woman with hereditary multiple exostoses had slowly progressive myelopathy due to a vertebral exostosis that compressed the spinal cord at T1-2. She did not show skeletal deformities, but had numerous palpable long-bone exostoses. While CNS complications are rare in hereditary multiple exostosis, 17 other cases have been reported.

Exostoses, Multiple Hereditary↗
PubMed1990

Case report 622. Multiple cartilaginous exostoses.

The case is presented of multiple cartilaginous exostoses involving the right and left metatarsals and phalanges, left scapula, ends of several distal ribs, and the spinous processes of several thoracic and lumbar vertebrae in a 3-month-old female Scottish terrier dog. Radiographical studies showed circumscribed expansile lesions in the affected bones. The dog developed neurological deficits 3 weeks later. Myelography displayed extradural compression of the opaque column at several thoracolumbar vertebrae. The biopsy specimen from an affected phalanx consisted of trabecular bone and hemopoietic tissue covered by a thin cap of hyaline cartilage. The dog was euthanized due to the poor prognosis. At autopsy, the surface of the affected bones was covered by a bluish-white, smooth, undulating cartilage cap. Histopathologically, the cartilaginous cap consisted of hyaline cartilage without a reserve zone, and abnormal endochondral ossification. The hereditary nature and the malignant transformation of multiple cartilaginous exostoses in the dog have been considered.

Animals↗
PubMed1991

Forearm deformities in multiple cartilaginous exostoses.

Sixteen patients with 20 forearm deformities were reviewed. The deformities were classified into three types. The degree of ulnar tilt of the radius, ulnar displacement of the carpus, and relative ulnar shortening were determined. The severity of the deformity correlated with these measurements. Metacarpal lengths were also measured. Significant metacarpal shortening without exostoses was seen in 10 of 11 patients and with exostoses remote from the metaphysis in 10 of 13 patients. Metacarpal shortening correlated with the type and severity of deformity.

Adolescent↗