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Proteus syndrome.

The term Proteus syndrome was coined in 1983 to describe a disorder of skeletal, hamartomatous, and other mesodermal malformations. The syndrome was named after the Greek god Proteus, whose name means "the Polymorphous." Clinical features of this new syndrome are currently being defined. Including the case reported herein, we have found 34 patients with Proteus syndrome described in the English literature. Major clinical findings, defined as those findings seen in more than half of the cases, include hemihypertrophy, macrodactyly, exostoses, epidermal nevi, characteristic cerebriform masses involving the plantar or palmar surfaces, a variety of subcutaneous masses, and scoliosis. Histologic examination of subcutaneous masses has identified a variety of lipomatous, hamartomatous, and angiomatous tumors.

Abnormalities, Multiple↗

[Operations for lengthening the upper limbs in children and adolescents].

We report 16 cases of lengthening of the upper limb: 4 humerus, 12 ulna from 1976 to 1987. Humerus lengthening are progressive with Wagner apparatus. They gave lengthening from 7.5 to 12 cm. There were no important complication. Ulna lengthening were done in patients with multiple hereditary osteochondromes and hereditary multiple exostoses, to correct the deformities of the forearm, and prevent dislocation of the proximal radial head. Actually progressive lengthenings replace immediate ones with radial osteotomy.

Adolescent↗

Microradiographic study of bone and tooth alterations in bovine fluorosis.

Exostoses were found in the 5th rib, the cannon bone and the mandible of a 6-year-old fluorotic cow. Microradiographic alterations of lamellar bone in these skeletal items included both matrix modifications and mineralization troubles. In the incisors, the enamel was covered by a layer of cementum, and the dentine presented numerous giant tubules and accentuation of incremental lines. In the cementum, hypomineralized areas were located along the periodontal fibres.

Animals↗

[The treatment of old dislocations of the radial head in children by osteotomy of the upper end of the ulna. Apropos of 15 cases].

Fifteen old dislocations of the radial head in children were treated by open reduction combined with a high osteotomy of the ulna and a reconstruction of the annular ligament. This method, described by the authors in 1978, was used in nine post-traumatic, three congenital, two paralytic and one dislocation associated with multiple exostoses. Twelve children were reviewed with a follow-up of two to 11 years. Nine radial heads remained stable and well reduced. The three congenital dislocations recurred. Eight elbows showed normal alignment and the other four were only improved. Ten elbows had normal movement or better movement than before the operation. Eleven children used their elbow normally or better than before. In all, 14 children were improved, and only one elbow had unaltered function. The results obtained in congenital dislocations should be able to be improved by a number of technical precautions.

Child↗

Ankylosis of hock joints in group caged male B6C3F1 mice.

Enlarged hock joints were observed during 1983 in B6C3F1 mice of chronic toxicity and carcinogenicity studies sponsored by the National Toxicology Program (NTP). Subsequently, approximately 9,500 B6C3F1 mice on 32 NTP chemical toxicity and carcinogenicity studies were evaluated for this condition by clinical examination. Group caged male B6C3F1 mice had thickening and reduced mobility of the hock joints at prevalences of 1.2% up to 6 months of age; 23% at 6 to 12 months of age; and 62% at 13 to 26 months of age. Group caged female B6C3F1 mice had a prevalence of 2% or less. Histologically, affected mice had periarticular exostoses on the bones of the hock joints, with formation of bony bridges around joints and deposition of new bone in joint spaces, resulting in partial or complete ankylosis. Individually caged male and female B6C3F1 mice were not affected. The cause of the ankylosis was not determined, but its occurrence in the NTP studies has been reduced by individual caging.

Animals↗

[Osteoma of the temporal bone as a rare differential diagnosis of a retroauricular tumor].

Extracanalicular osteomas of the temporal bone represent a rare differential diagnosis of osseous tumours in the ear region in contrast to the common finding of exostoses of the external auditory canal. Basing on a report on a retroauricular osteoma of the squama of the temporal bone treated in the authors' ENT clinic, the morphological and clinical characteristics of this tumour are discussed.

Adult↗

Costal bone changes similar to hypertrophic osteopathy associated with pulmonary and abdominal mesothelioma in a dog.

An 8-year-old male Irish Setter was admitted because of nonweight bearing lameness of the left forelimb. Radiography failed to reveal any bony lesions of the forelimb; however, extensive periosteal new bone formation over most rib surfaces and an atypical cardiac silhouette suggestive of a mediastinal mass with pleural effusion were observed. New bone formation was seen on the ilium and a filling defect in the urethra, distal to the prostate, was apparent with contrast urethrocystography. Necropsy revealed scirrhous mesothelioma of the thorax and abdomen, multifocal periosteal exostoses, paraprostatic cyst, and benign prostatic cystic hyperplasia. Tumor cells were associated with the periosteal exostotic costal bone. The specific cause of the lameness was not determined.

Abdominal Neoplasms↗

[Monomelic melorheostosis of the hand].

Melorheostosis is a rare sclerosing bone dysplasia affecting children and adults. Concerning the etiology it seems to be a metameric abnormality of the tissue of mesodermal origin, which is initiated early in embryonic life prior to the formation of the extremity buds. The flowing hyperostosis is usually associated with limb deformity, soft tissue contractures, severe pain and stiffness of joints. Bone scanning complements standard radiologic studies and permits evaluation of the extent and activity of the disease. Conservative treatment is disappointing. Thus we recommend the excision of the exostoses at an early state to prevent further deformity as is shown in a clinical case report.

Female↗

Industrial fluoride pollution. Chronic fluoride poisoning in Cornwall Island cattle.

An aluminum plant on the south bank of the St. Lawrence river, southwest of Cornwall Island, Ontario, Canada, has emitted 0.816 metric tons of fluoride daily since 1973; considerably higher amounts were emitted from 1959 to 1973. The plant has been designated as the "major source of fluoride emissions impacting on Cornwall Island." Chronic fluoride poisoning in Cornwall island cattle was manifested clinically by stunted growth and dental fluorosis to a degree of severe interference with drinking and mastication. Cows died at or were slaughtered after the third pregnancy. The deterioration of cows did not allow further pregnancies. Fluoride concentrations in ash of biopsied coccygeal vertebrae increased significantly with age and were dependent on distance from and direction to the aluminum plant. Fluoride in bone ash of a 7-month old-fetus exceeded 500 ppm; fluoride thus was passed transplacentally. Analyses of fluoride in ash of bones obtained at necropsy of cattle from 4 months of age to 4 to 5 years of age showed increased amounts with age. Cancellous bone retained far higher amounts than cortical bone, a reflection of the normally higher metabolic rate of cancellous bone. Concentrations exceeding 10,000 ppm fluoride were recorded in cancellous bone of a 4-to 5-year-old cow. The target cells for fluoride in chronic fluorosis were shown to be the ameloblasts, the dental pulp cells and the odontoblasts and, in bone, primarily the resorbing osteocytes and also the osteoblasts. Atrophy and necrosis of the ameloblasts were responsible for enamel defects. The existing enamel showed brown discoloration from fluoride deposits. The pulp cells underwent fibrous and osseous metaplasia and necrosis of the ectopic bone occurred. The odontoblasts were atrophic and the dentin showed brown discoloration. The resorbing osteocytes were inactive and osteosclerosis resulted. This was especially pronounced in areas of normally great apposition, i.e. in the metaphyses. The epiphyseal plate became squeezed between petrotic bone and growth was stunted. Resorption of alveolar bone surrounding the deciduous teeth was severely retarded or arrested. A delay in eruption of permanent teeth occurred; it was up to 3.5 years in incisor teeth. Interference with the resorbing osteocytes in fluorotic bone was also demonstrated by loss of collagen birefringency in such bone. Failure of bone resorption also caused retention of trabecular bone in the cortices; this was observed even in a 4-t0-5-year-old cow. In areas where modeling into osteonic bone had begun, fluoride deposits were extremely heavy but this bone showed numerous soft osteons in microradiographs. The toxic effect of fluoride on osteocytes also resulted in the death of the cells. Such osteonecrosis occurred mainly in gnathic bone. There was atrophy of the osteoblasts. Osteopenia thus resulted from osteonecrosis and osteoporosis. Subperiosteal exostoses were not observed in long bones. The degree of fluorosis in Cornwall Island cattle was severe...

Air Pollutants↗

[Radiographic aspects of the hand in diffuse idiopathic skeletal hyperostosis].

Radiographs of the hand of 53 patients with diffuse idiopathic skeletal hyperostosis (DISH) were compared with 20 acromegalic subjects and 100 matched controls. Bone qualitative changes, characteristic of DISH, include: (1) arrow-heading of the distal phalangeal tufts; (2) enthesopathy of the proximal phalanges; (3) enlarged sesamoid bones; (4) hook-like exostoses at the metacarpophalangeal heads; (5) cortical thickness of diaphyseal tubular bones. Therefore, the hand in this disease appears as a "robust" form, particularly in males, resembling acromegalic pattern, but in this disease, the hand is much more coarse (so called squared hand), especially because of the soft tissue involvement and articular cartilaginous hypertrophy. Only enthesopathy seems to be the most characteristic and peculiar finding of the disease. Quantitative bone change measurements have demonstrated in DISH that metacarpal and Exton Smith indices differ statistically toward the controls. The hand is wider, but in males bone mass may result the same and in female is shortly reduced. Soft tissue alterations, compared with the controls, are not significant. On the contrary, in acromegaly there is a significant statistical difference in all the quantitative measurements of both the skeletal and soft tissue lesions, compared with the controls and patients affected by DISH.

Acromegaly↗

Iatrogenic exostosis in a patient treated for osteomyelitis.

A 10-month-old child treated surgically for osteomyelitis of the distal femur subsequently developed an exostosis at that site. No previous similar case has been reported. The etiology of exostoses is reviewed here, with evidence that this patient's exostosis could be iatrogenic.

Child, Preschool↗

Effects of castration, with and without testosterone replacement, on leg bone integrity in the domestic fowl.

Studies were conducted to assess the effects of castration on leg bone integrity of male White Leghorn chickens. Eighteen 5-week-old males were randomly allocated to 3 groups: group 1--intact controls, group 2--castrated, and group 3--castrated and subsequently treated with testosterone (T)-filled Silastic capsule implants. Body weight and plasma concentrations of luteinizing hormone, androgens, and total calcium were measured in each bird at intervals throughout the experiment. Subsequently, radiographs were performed upon the tibiotarsus-tarsometatarsus region of the left limb of birds from the 3 treatment groups at 7, 35, and 47 weeks of age and assigned a score of 1 to 3 (1 = normal; 3 = greatest pathologic change). Analysis of radiologic scores indicated that at 35 and 47 weeks, castrated birds (group 2) had a mean score significantly greater than that of control birds (group 1). The abnormality was typified by hyperossification with exostoses in the ossified tibial cartilage and lateral calcaneal ridge. In contrast, radiologic scores from individual T-implanted castrated birds (group 3) at 35 and 47 weeks were more variable, but the mean score was not statistically different from that of the control group. We conclude that the depression of circulating concentrations of sex steroids, by castration, in young male chickens results in hypercalcification of the tibiotarsus-tarsometatarsus region of the limb. This effect appears to be mediated directly or indirectly by T or a metabolite of T.

Animals↗

The pathology of the feline model of mucopolysaccharidosis VI.

Three cats with feline arylsulfatase-B--deficient mucopolysaccharidosis were studied by light and transmission electron microscopy. Membrane-bound cytoplasmic inclusions were present in hepatocytes, bone marrow granulocytes, vascular smooth muscle cells, and fibroblasts in skin, cornea, and cardiac valves. Central nervous system lesions were restricted to mild ventricular dilatation, perithelial cell vacuolation, and, in one animal, cord compression by vertebral exostoses. The lesions in these cats closely resembled those described in human patients with mucopolysaccharidosis VI (Maroteaux-Lamy syndrome).

Animals↗

Nutritional problems in cats: taurine deficiency and vitamin A excess.

Two nutritional problems of the cat are reviewed. One represents a deficiency of taurine, the other vitamin A toxicity. Taurine deficiency in cats is insidious because the progressive retinal degeneration induced may go unnoticed until the damage is advanced and irreversible. Both rods and cones undergo degeneration along with the underlying tapetum lucidum. The hyperreflective focal lesion is easily observed in the area centralis with an ophthalmoscope and has been previously identified as feline central retinal degeneration. This lesion is not reversed by taurine supplementation, even though the remaining retina may be saved from further degeneration. The cat requires dietary taurine, found in meat and fish, because it cannot synthesize enough to meet demands for bile acid conjugation and tissue metabolism, especially those of muscle and central nervous system. Vitamin A toxicity is not commonly observed in cats but may occur if cats are fed beef liver in which appreciable vitamin A is stored. Cats exhibit muscle soreness and hyperesthesia, especially along the neck and forelimbs where bony exostoses of cervical verterbrae and longbones are common. The diagnosis is readily made from radiographs. The response to removal of vitamin A from the diet is generally rapid and, unless the toxicity has been chronic in young kittens, recovery is generally satisfactory.

Animal Nutritional Physiological Phenomena↗

Periosteal and metaplastic bone formation in mouse minced muscle regeneration.

Structures resembling exostoses derived from periosteum and metaplastic nodules of cartilage and bone were induced in mouse legs by isotopic implants of minced skeletal muscle or heterotopic implants of nonlimb skeletal and cardiac muscle that does not regenerate. Liver mince implants produced no periosteal response. Control experiments demonstrated that, after excision of the muscle without the placing of any implant, no periosteal response or metaplastic bone occurred unless the periosteum was deliberately injured. Metaplastic nodules of cartilage and bone arose from the fibrous stroma that constituted the greater part of the implant when muscle mince regeneration was unsuccessful. The development of both the periosteal response and the metaplastic bony nodules is inversely related to the efficiency of phagocytosis by macrophages that remove the implanted debris and the concomitant degree of regeneration of new muscle. These phenomena, and, therefore, bone and cartilage formation, are strongly age related. The rapidity and extent of development of both abnormal bone and cartilage--less than 7 days--and its reproducibility may make this phenomenon a useful model for studies on the pathophysiology of bone and cartilage formation, particularly with regard to age.

Animals↗

[Cartilaginous tumors of the spine : a report on three cases with neurological symptoms (author's transl)].

Three cases of cartilaginous tumors of the spine are reported. The first patient had a benign lumbar vertebral enchondroma which had recurred locally on several occasions, causing severe functional sequelae. The second case concerned a patient with a chondrosarcoma of the base of the skull, in whom radiological signs were observed only secondarily on a standard film. The third patient had exostosing disease with an intra-canal osteochondroma provoking neurological disorders. The relative value of computed tomography and opaque myelography is discussed in relation to the last case.

Adolescent↗