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Parasagittal meningioma en plaque with extracranial extension presenting diffuse massive hyperostosis of the skull.

BACKGROUND: We describe a case of convexity meningioma en plaque (MEP) invading the skull and scalp with diffuse massive hyperostosis, presenting striking radiological findings. CASE DESCRIPTION: A 48-year-old woman was admitted to our hospital with headache, general fatigue, diplopia, and blurred vision. A skull X-ray film, computed tomography (CT), and 3-dimensional CT demonstrated diffuse significant hyperostosis and sclerosis in the cranial vault. Magnetic resonance (MR) imaging revealed a frontoparietal, well-enhanced extra-axial mass lesion, which is compatible with findings of MEP. The tumor was partially resected. Histological examination revealed massive tumor invasion into the dura mater, hyperostotic skull, and scalp. CONCLUSION: Hyperostosis is frequently observed in MEP, although there has been no report of a case of MEP such as ours demonstrating diffuse tumor extension and diffuse hyperostosis. Analyzing the CT finding of hyperostotic bone is useful for differentiation of MEP from other diseases that include a hyperostotic condition.

Atrophy↗

Infantile cortical hyperostosis and facial nerve palsy.

Facial nerve palsy was diagnosed in a 13-month-old child with infantile cortical hyperostosis. Facial palsy preceded the roentgenographic evidence of mandibular hyperostosis but there was concurrent evidence of hyperostosis of multiple other bones. This case exemplifies the importance of maintaining a high index of suspicion for infantile cortical hyperostosis in infants and children presenting with facial swelling and facial nerve palsy.

Facial Paralysis↗

Diffuse idiopathic skeletal hyperostosis.

OBJECTIVE: To describe the axial and appendicular skeleton findings of diffuse idiopathic skeletal hyperostosis. To analyze the role of conventional radiography, computed tomography (CT) and magnetic resonance imaging (MRI) in the diagnosis of this condition. To discuss the differential diagnosis and diagnostic pitfalls of this disease. MATERIAL AND METHODS: The involvement of vertebral and extravertebral sites including the pelvis, calcaneum, ulnar olecranon, and patella is frequently found in the literature. The lesions described are the anterior and lateral ossification of the spine, hyperostosis at sites of tendon and ligament insertion, ligamentous ossification, and periarticular osteophytes. The criteria for the diagnosis of diffuse idiopathic skeletal hyperostosis involving the spine are: flowing ossification along the anterior and anterolateral aspects of at least four contiguous vertebrae, preserved intervertebral disc height, no bony ankylosis of the posterior spinal facet joints, and finally no erosion, sclerosis or bony ankylosis of the sacroiliac joints. RESULTS: The disease has about the same frequency in men (65%) and women (35%); it is most common in the thoracic spine and occurs less frequently in the lumbar and cervical spine. The disease most commonly presents in the sixth and seventh decades of life and its estimated frequency in the elderly is 5-15%. Signs and symptoms include stiffness and pain in the back, dysphagia due to direct esophageal compression/distorsion, pain related to associated tendinitis, myelopathy related to core compression associated to the ossification of the posterior longitudinal ligament, and pain related to vertebral complications--e.g. fracture/subluxation. CONCLUSION: While conventional radiography clearly confirms the diagnosis of diffuse idiopathic skeletal hyperostosis, CT and MRI better detect associated findings (e.g. ossification of the posterior longitudinal ligament) and complications (e.g. spinal cord compressive myelomalacia).

Aged↗

A special form of hyperostosis frontalis interna.

The article describes a very severe case of hyperostosis of the frontal bone and discusses its possible differential diagnosis. What makes this case special is the fact that the osseous changes include all 3 layers of the bone. Macroscopically the lesion resembles an osteoma as described by Burkhardt (1970) and v. Eiselsberg (1906). The histological examination lead to the final diagnosis of hyperostosis frontalis interna. One must note that there were certain effects caused by a metastasis of a lobular carcinoma of the breast which influenced the osseous changes from the outer surface. The rough, spicula-like structure in the centre of the frontal squama should be put down to tumour erosion. The peripheral areas of the hyperostosis, however, still exhibit the original smoother texture of the disease. The final diagnosis in this unusual and possibly unique case was an intense hyperostosis frontalis interna with secondary changes due to the metastases of a lobular carcinoma of the breast.

Aged↗

Sporadic congenital infantile cortical hyperostosis (Caffey's disease).

Prognosis of congenital infantile cortical hyperostosis (Caffey's disease) is poor particularly in premature babies. Two cases are presented of congenital Caffey's disease in premature babies. The first baby was hydropic at birth and had cortical hyperostosis involving the mandible and long bones of right upper limb and both lower limbs. The second baby had cortical hyperostosis of the nasal bones causing severe nasal nonchoanal stenosis that needed surgery, in addition to involvement of long bones of the four extremities. Both babies recovered from the disease and were discharged home well. These cases suggest that the improved outcome of congenital of infantile cortical hyperostosis may reflect improvement of neonatal mechanical ventilation and availability of neonatal total parenteral nutrition.

Fatal Outcome↗

[Anterior hyperostosis of the cervical spine: diagnostic studies and surgical therapy].

BACKGROUND: Dysphagia due external compression by anterior hyperostosis of the cervical spine is rare. The diagnosis may be established by conventional X-ray of the spine, esophagogram, and CT. PATIENTS: We operated on three patients with large anterior osteophytes from C3 to C7. In two cases morphologic changes of the cervical spine were the main cause of dysphagia. One patient with progressive hypopharynx cancer had hyperostosis of cervical spine as secondary findings. RESULTS: The patients were asymptomatic, post-operatively. CONCLUSIONS: Cervical osteophytes can be detected in 20-30% of the population in asymptomatic patients. The therapeutic approach depends on the extent of dysphagic complaints. Painful dysphagia is a indication for surgery. The anterolateral extrapharyngeal approach is commonly preferred with anterior hyperostosis between C4 and C7. The transoral intrapharyngeal approach has been used in patients with hyperostosis of cervical vertebra C2/C3. Interdisciplinary orthopedic and ENT surgical treatment is without complications and yields good functional results.

Cervical Vertebrae↗

[Exo- and endomarginal reactions in the hand in diffuse idiopathic skeletal hyperostosis, their quantification and age dependence].

Diffuse idiopathic skeletal hyperostosis (DISH) may lead to three types of reaction in the hand: increased exomarginal bone formation at numerous fibrous insertions, which increase with age, and endomarginal sclerosis which reaches its maximum during adult life, but which regresses partially with increasing age. The third reaction is increased subchondral sclerosis, which also becomes most prominent in middle age and then diminishes. DISH is more frequently seen in the hand than in the spine and in the hand leads to hyperostosis of varying intensity and in different localities. By quantifying the hyperostosis and comparing it with normal, it is possible to characterise the hyperostosis as it affects the hand.

Adult↗

Case reports: aseptic femoral osteitis and sternocostal hyperostosis from SAPHO syndrome.

Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome are characterized by peculiar bone lesions that commonly involve the anterior chest wall. Osteitis typically is the most prominent skeletal lesion seen in synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome. We present a patient with aseptic femoral osteitis and sternocostal hyperostosis. The classic clinical, radiographic, and histologic features of this syndrome are described. Diagnosis is difficult in patients with only one symptomatic bone. The lesion often is confused with suppurative osteomyelitis because of similar clinicopathologic findings. Although the optimal treatment is unclear, it is important to consider synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome in the differential diagnosis of any lytic, sclerotic, or hyperostotic bone lesion to avoid unnecessary long-term antibiotic therapy.

Acquired Hyperostosis Syndrome↗

Spontaneous symptomatic pseudoarthrosis at the T11-T12 intervertebral space with diffuse idiopathic skeletal hyperostosis: a case report.

STUDY DESIGN: We report on a 69-year-old male who had severe back pain due to spontaneous symptomatic pseudoarthrosis at the T11-T12 intervertebral space with diffuse idiopathic skeletal hyperostosis. OBJECTIVE: To describe a rare clinical entity and successful treatment by spinal fusion with a 4-year follow-up. SUMMARY OF BACKGROUND DATA: There have been a few reports of spontaneous symptomatic pseudoarthrosis of an intervertebral space associated with diffuse idiopathic skeletal hyperostosis, but there have been no reports of surgical treatment for this clinical condition. METHODS: Plain radiographs of the patient, who was admitted to our hospital with severe back pain but no history of trauma, revealed manifestations of diffuse idiopathic skeletal hyperostosis and a pseudoarthrosis at the T11-T12 intervertebral space. Posterior instrumentation from T9 to L2 and anterior bone grafting at the T11-T12 intervertebral space were performed. RESULTS: The patient has been followed for 4 years and is currently asymptomatic. CONCLUSIONS: A rare case of spontaneous symptomatic pseudoarthrosis at the T11-T12 intervertebral space with diffuse idiopathic skeletal hyperostosis was treated successfully by spinal fusion.

Aged↗

[Diffuse idiopathic skeletal hyperostosis (D.I.S.H.)].

Diffuse idiopathic skeletal hyperostosis (D.I.S.H.) is a common disorder of unknown aetiology characterized by exuberant hyperostosis of the antero-lateral aspect of the spinal column, that sometimes leads to bone ankilosis, and by ossification of extra-spinal entheses. This condition is often associated with the metabolic derangement of type 2 diabetes. Primary hypertension, its cardiovascular aftereffects and lithiasis are also often present in these patients. D.I.S.H. has to be distinguished from osteoarthritis, although they often coexist in the same patient. The mean difference lies in the anatomical target of the pathological process, that is represented by articular cartilage in osteoarthritis and by entheses in diffuse idiopathic skeletal hyperostosis. The enthesopathy leads to the ossification of the anterior longitudinal ligament of the spine and causes the formation of flowing osteophytes, while intervertebral disc space is quite preserved in early phases of the disease. Symptoms of spine involvement are not typical of the disease and consist of pain and stiffness, usually worsened by inaction and damp. It has also been described the ossification of posterior longitudinal ligament which can lead to medullary canal stenosis. Appendicular skeleton is symmetrically involved in early phases of the disease, the most distinctive affected sites being feet, olecranus and patella. Hip involvement is also frequent and may lead to severe disability and represents an important cause of invalidity. The purpose of the present review is to remark on aetiopathogenetic and clinical aspects of diffuse idiopathic skeletal hyperostosis.

Humans↗

[Experimental study of hyperostosis induced by hypervitaminosis A].

Radiological and histological studies were made on retinoid-induced hyperostosis in rats. Vitamin A (VA) was administered intraperitoneally in rats for 6 months. Hyperostosis was observed in 94 percent of rats administered VA and in 38 percent of the control. Chondrocytes and vascular proliferation were observed in the attachment of the tendons and in the anterior corner of the vertebral body after 3 months. Hyperostosis was observed as osteophytes in the attachment of ligaments or tendons and as heterotopic ossification in the tendons or the joint capsules of the whole body after 6 months. Immature cells were observed around the osteophytes. These areas were stained with pH 4.1 toluidine blue and disappeared following streptomyces hyaluronidase treatment. This would indicate that hyaluronic acid increased around these areas. These results appear to demonstrate that hypervitaminosis A is capable of producing hyperostosis.

Animals↗

[Plasma cell dyscrasias and diffuse idiopathic skeletal hyperostosis. Is it a merely accidental association?].

The radiologic staging of a series of 144 patients (88 males and 56 females) affected with plasma-cell dyscrasias and observed over a 26-month period, revealed both the well-known bone myeloma-related abnormalities and hyperostotic lesions similar to those described in diffuse idiopathic skeletal hyperostosis. The incidence of skeletal hyperostosis was 31.94%, much higher than that reported in literature for the general population (5%). Typically, the axial skeleton is the most common location for abnormalities in multiple myeloma (MM) as well as in DISH: involvement of the dorsal spine was observed in 65% of cases, the cervical spine was involved in 34.8% of patients, and the lumbar spine in 28.3%. Peripheral ossifying enthesopathy, considered as "whiskering" in the pelvis, was found in 12 cases (8.2%), 7 males and 5 females. DISH was indifferently present in both MM (23 cases), with severe osteolysis (stage III) or simple osteoporosis (stage I), and monoclonal gammopathy of undetermined significance (MGUS) (17 cases), usually without any myeloma-related bone lesions, and in Waldenström disease (4 cases). Many hypotheses are discussed as to the possible pathogenesis (e.g.: accidental, dysmetabolic, or degenerative) of hyperostosis in dysgammaglobulinemias, but, to date, they are no more than mere guesses. DISH is a disorder the etiology of which is still unknown: it is likely to be an ossifying diathesis, but its incidence in both illnesses--which are both plasma-cell dyscarsias--is too high for the association to be accidental. Thus, a pathogenetic factor produced by multiple myeloma can be hypothesized, capable of increasing the so-called idiopathic hyperostosis.

Adult↗

[Ankylosing hyperostosis of the spine (Forestier's disease). A study of its clinical picture based on the data from a mail questionnaire].

Postal questionnaires were used to study the incidence and intensity of complaints of the spine and peripheral skeleton in patients with ankylosing hyperostosis of the spine. Selection of persons to fill in questionnaires was carried out by means of a preliminary non-randomized analysis of the records of the spinal fluorograms in patients of one of the outpatient clinics of Moscow. Criteria for ankylosing hyperostosis of the spine were the presence of continuous ossification of the anterior longitudinal ligament over a length of not less than 4 successive segments of the spine and the lack of other x-ray alterations in the given part of the spine. It has been established that as compared to the control group (n = 41) without any x-ray alteration in the thoracic part of the spine, the patients with ankylosing hyperostosis of the spine (n = 146) complained of the lumber part and shoulder joints more frequently. The incidence of complaints of other 6 out of 8 parts of the skeleton under analysis was not high in ankylosing hyperostosis of the spine.

Arthrography↗

Gerontological aspects of hyperostosis frontalis interna.

Authors discuss hyperostosis frontalis interna observed in a large number of aged persons, on the basis of age and sex distribution as well as its clinical and roentgenomorphological analysis. In various forms of the ossification of the frontal bone no significant difference was found between the localisation of hyperostosis and the clinical symptoms. On other hand, there is a direct correlation between the extension and severity of hyperostosis and the frequency of occurence of the associated symptoms (obesity, hypertension). They found the aetiological classification more adequate than the morphological categorization of Moore. Their cases are discussed 1. as partial phenomenon of the Morgagni's syndrome; 2. as independent alteration, showing no other symptoms; 3. as transitionary forms inserted between the two groups mentioned above. They discuss also the question of senile, compensatory hyperostosis frontalis interna. On the basis of the study of a large autopsy material they support the opinion that there is a direct connection of this form with old age.

Aged↗

[Sternocostoclavicular hyperostosis. Nosological concepts].

The term sternocostoclavicular hyperostosis groups affections of different pathogenicity. These result from an ossifying enthesiopathy, either inflammatory, isolated to the thoracic wall anteriorly or combined with a spondylarthropathy, particularly ankylosing spondylitis, or degenerative, the anterior thoracic hyperostosis usually being part of an ensheathing vertebral hyperostosis. A differential diagnosis is anterior thoracic hyperostosis due to an inflammatory osteopathy occurring in young patients and often associated with other bone lesions of pelvis and spine or long bones.

Adult↗

Infantile cortical hyperostosis with unusual clinical manifestations.

Infantile cortical hyperostosis may mimic a variety of serious conditions in early infancy, such as trauma, vitamin deficiencies, infection, or tumors, In a patient in good general condition there are typical roentgenological features often with involvement of the mandible that are diagnostic of the condition. In 3 cases of infantile cortical hyperostosis (Caffey's disease), one had the clinical picture of Erb's palsy and subsequently developed hyperostosis of the scapula. The second, had a "pulled elbow" and radial nerve palsy and later developed hyperostotic changes in the humerus and radius on the same side. The third was referred for treatment of congenital torticollis and found to develop hyperostosis of the clavicle on the same side. The recognition of this condition obviates unnecessary prolonged investigation and treatment.

Clavicle↗

[A case of a patient with sternocostoclavicular hyperostosis complaining of severe chest pain, which must be distinguished from cardiovascular disease].

We report a case of a patient complaining of severe chest pain which required a differential diagnosis between cardiovascular disease and sternocostoclavicular hyperostosis. The patient was a 61-year old male who began experiencing pain across both scapulas at the end of September, 1990. He was admitted to our hospital when the pain extended to the back and anterior chest areas. Examination on admission revealed inflammation with a white blood cell count at 11,800/mm3, an erythrocyte sedimentation rate of 136 mm/hr, and CRP at 14.2 mg/dl. Angina pectoris was suspected based on findings from coronary arteriography which showed 60% stenosis at Seg 6. A Ga-scintigram conducted to determine the cause of the chest pain revealed accumulations in the upper mediastinum. CT and MRI both showed hyperostosis of the sternum, and bone scintigram confirmed marked accumulations in the same area. Palmoplantar pustulosis (PPP) was also clearly noted on the palms and soles of the feet. Sternocostoclavicular hyperostosis was diagnosed based on these findings. Sternocostoclavicular hyperostosis was suspected in this case based on the clinical findings, inflammatory state, and accumulations revealed by bone scintigraphy. However, the diagnosis could have been more conclusive if non-suppurative hyperosteostic osteomyelitis were observed by bone biopsy. A differential diagnosis for unknown sources of chest pain should be considered in cases such as this.

Bone and Bones↗

[Endocrinologic and pathologic findings in ornamental birds with polyostotic hyperostosis].

Polyostotic hyperostosis is a finding that is often seen on x-rays in companion birds. Hyperestrogenism might be a causative for polyostotic hyperostosis. The purpose of this study was to compare endocrinologic and pathologic findings in birds with polyostotic hyperostosis. Thirty-five affected and three control birds were examined. Blood samples were taken for endocrinologic statement (estrone, estradiol, testosterone and estrone sulfate) and the values were compared with the pathologic findings. In the present study the comparison of endocrinologic and pathologic findings did not reveal any significant results supporting the hypothesis that polyostotic hyperostosis was caused by hyperestrogenism.

Animals↗