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[Paget's disease, ankylosing vertebral hyperostosis and hyperostosis frontalis interna].

Ankylosing vertebral hyperostosis and internal frontal hyperostosis have a frequency of 5% and 8% respectively. In Paget's disease, these two disorders are found with a frequency of 40% and 19.1% respectively. This frequency is not due to chance. There exists a link between the 3 diseases. Ankylosing vertebral hyperostosis is thusa new pathological association in Paget's disease. It is even the most frequent of these associations. It seems Paget's disease induces hyperostosis and not the contrary. The authors suggest the theory that cortical and periostal hypervascularisation in pagetoid bone favour the development of these hyperostoses.

Aged

[The acquired hyperostosis syndrome. Synthesis of 13 personal observations of sternocostoclavicular hyperostosis and 300 cases from the literature. 1].

Sterno-costo-clavicular hyperostosis (SCCH) is the most common manifestation of a syndrome, consisting of increased bone metabolism, mostly new bone formation and heterotopic ossification of fibrous tissue, which we have characterised as the acquired hyperostosis syndrome. In part I we discuss the terminology, radiological appearances, scintigraphy, clinical and laboratory findings, bacteriology, histology, nosology, complications, treatment and differential diagnosis of SCCH. Chronic recurrent multifocal osteomyelitis (CRMO) is regarded as a phenotype of SCCH, depending on the age. CRMO occurs in children, adolescents and young adults, SCCH predominantly in middle-aged and elderly adults.

Adult

[Pelvic and skeletal hyperostosis (diffuse idiopathic skeletal hyperostosis, Forestier disease)].

Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by, among other things, spinal and extraspinal manifestations. Two studies on the prevalence and clinical significance of hyperostotic spurs and ectopic ossifications in the pelvic area are presented. Spinal DISH seems to be a risk factor for the development of ectopic ossification, particularly in the postoperative course after total hip arthroplasty. Pain and reduced range of motion, however, are not obligatory consequences of these phenomena.

Aged

Diffuse idiopathic skeletal hyperostosis (DISH) of the shoulder: a cause of shoulder pain?

Shoulder pain is a common complaint and shoulder hyperostosis a frequent radiological condition. However, little is known about the association between the clinical and radiological findings. To evaluate the clinical relevance of shoulder hyperostosis we performed a controlled, blind study of 99 hospitalized probands with and without thoracospinal hyperostosis on lateral chest X-rays. The study included grading of the shoulder hyperostosis on the basis of three bilateral standard radiographs, assessing shoulder pain in a standardized way by an interviewer and recording extraskeletal causes of shoulder pain. The prevalence of shoulder hyperostosis was doubled in probands with thoracospinal hyperostosis compared to controls (chi 2 = 5.90, P less than 0.025, n = 99). Shoulder hyperostosis, irrespective of thoracospinal hyperostosis, predisposed to shoulder pain (40% versus 18%, chi 2 = 4.06, P less than 0.05, n = 74). Shoulder hyperostosis in combination with thoracospinal hyperostosis (shoulder DISH) predisposed to shoulder pain to an even greater extent (46% versus 12%, chi 2 = 6.64, P less than 0.01, n = 47). We conclude that shoulder hyperostosis is a radiological finding of potential clinical relevance.

Arthrography

Diffuse idiopathic skeletal hyperostosis (DISH) of the elbow: a cause of elbow pain? A controlled study.

Elbow pain is a common complaint and elbow hyperostosis a frequent radiological condition. However, little is known about the association between the clinical and radiological findings. To evaluate the relationship between spinal and extraspinal hyperostotic features and the clinical relevance of elbow hyperostosis we have performed the first controlled, double-blinded study of 85 hospitalized probands, 33 with and 52 without thoracospinal hyperostosis on lateral chest X-ray. Elbow and shoulder hyperostosis were graded on bilateral standard radiographs. Elbow pain was assessed by an interviewer using a standardized questionnaire and extraskeletal causes of elbow pain were recorded. The prevalence of elbow hyperostosis was increased in cases with thoracospinal hyperostosis compared to controls (82% versus 58%, chi 2 = 5.32, P less than 0.025, n = 85, olds ratio (OR) 3.30 (95% CI 1.16-9.35)). Similarly, the prevalence of elbow hyperostosis was increased in cases with shoulder hyperostosis compared to controls (83% versus 60%, chi 2 = 4.51, P less than 0.05, n = 84, OR = 3.20 (95% CI 1.06-9.66)), emphasizing the multifocal nature of hyperostotic features. Elbow pain was only slightly more prevalent in cases with elbow hyperostosis compared to controls (21% versus 13%, chi 2 = 0.75, NS, OR = 1.84 (95% CI 0.46-7.44)). We conclude that elbow hyperostosis is a radiological finding of doubtful clinical relevance.

Aged

[Association of lumbar canal stenosis and ankylosing vertebral hyperostosis. Results of a multicenter study].

The authors report data collected in a study of the association of narrow lumbar canal and vertebral hyperostosis. Five centres (Montpellier, Toulouse, Lille, Lyons and Paris) participated in this cooperative study which was both retrospective and prospective. Grid case forms were sent to homogenise the date provided. Two hundred and sixty nine cases of symptomatic lumbar canal stenosis were collected; 89 (33 per cent) had hyperostosis. Hyperostosis was definite in 74 cases and probable in 15 other cases. Certain radiological and/or CT scan morphological factors seen frequently in the hyperostosis patients group led us to undertake a second study in 2 of the 5 centres (Montpellier and Toulouse) in order to identify their specificity. Twenty eight items were adopted and studied by 3 different evaluators (2 rheumatologists and one radiologist) in the X-ray films and CT scan documents of 100 patients with acquired lumbar canal stenosis with or without hyperostosis (46 and 54 cases respectively). The most discriminative appearances, which we suggest as diagnostic criteria of narrow lumbar canal with hyperostosis concern anterior and/or posterolateral marginal somatic bone proliferations on the non-articular surfaces of the posterior apophyses and ossifications of the posterior joint capsule and of the ligaments (ligamentum flavum--posterior longitudinal ligament--supraspinous ligament). Four of these 6 criteria are necessary to make the diagnosis of lumbar stenosis with hyperostosis. The radiological and CT scan appearances of lumbar hyperostosis appear to differ from ordinary degenerative changes of osteoarthrosis and hyperostosis may be held responsible for compression of the dural cul-de-sac.

Humans

[Hyperostosis triangularis ilii -- a sacro-iliac stress phenomenon Part. 2 (Incidence, prognosis, pathogenesis, aetiology, tracer studies, differential diagnosis) (author's transl)].

The anterior iliac margin adjacent to the sacro-iliac joint contains a physiological zone of hyperostosis. Its shape is that of a triangular pyramid. Because of its small size, it is radiological invisible, but is nevertheless an indication of the presence of normal stresses passing from the mass of the trunk to the lower extremities. If for any reason, such as is described in this paper, the stresses on the hyperostosis zone are increased, the latter becomes enlarged, while still keeping its shape. It then appears on the radiograph as hyperostosis triangularis ilii. In approximately 50% of cases there is a simultaneous but variable sacral hyperostosis. Observations on 40 patients, together with two autopsies and radionucleide investigations suggest that hyperostosis triangularis ilii represents a potentially reversible bone reaction of the physiological hyperostosis of the anterior iliac margin. The clinical differential diagnosis raised by the radiological finding of a "triangular ilium hyperostosis" is discussed.

Adult

Porotic hyperostosis: a new perspective.

Porotic hyperostosis is a paleopathologic condition that has intrigued researchers for over a century and a half. It is now generally accepted that anemia, most probably an iron deficiency anemia, is the etiologic factor responsible for lesion production. Although there can be a number of factors involved in the development of iron deficiency anemia, a dietary explanation has often been invoked to explain the occurrence of porotic hyperostosis in past human skeletal populations. In fact, porotic hyperostosis has been referred to as a "nutritional" stress indicator. Traditionally those groups with a higher incidence of porotic hyperostosis have been considered to be less successful in adapting to their environment or more nutritionally disadvantaged than other groups. A new perspective is emerging that is challenging previous views of the role of iron in health and disease, thus having profound implications for the understanding of porotic hyperostosis. There is a new appreciation of the adaptability and flexibility of iron metabolism; as a result it has become apparent that diet plays a very minor role in the development of iron deficiency anemia. It is now understood that, rather than being detrimental, hypoferremia (deficiency of iron in the blood) is actually an adaptation to disease and microorganism invasion. When faced with chronic and/or heavy pathogen loads individuals become hypoferremic as part of their defense against these pathogens, thus increasing their susceptibility to iron deficiency anemia. Within the context of this new perspective porotic hyperostosis is seen not as a nutritional stress indicator, but as a indication that a population is attempting to adapt to the pathogen load in its environment.

Anemia, Hypochromic

[Familial infantile cortical hyperostosis (Caffey's disease) with osteolytic lesions of the skull].

BACKGROUND: The parietal and frontal bones are rarely affected in infantile cortical hyperostosis. CASE REPORTS: Case n. 1: A 14-day-old boy developed a swelling of the left eyelid that extended to the face in a few days. It was tender and associated with fever. Laboratory findings were increased (RBC sedimentation rate and other signs of inflammation). CT scan of the sinuses, soft tissues and bones was normal, but at the age of 23 days minimal patches of rarefaction were seen on X-rays of the frontal bones. These lacunar areas gradually extended, with the inflammation persisted. A new CT scan at 6 months of age confirmed the existence of lacunar areas in the skull, but failed to find any lesions of the mandibles or nasal bones. At that time, scintigraphy showed inflammatory lesions of the skull and ribs. All clinical, laboratory and radiological findings spontaneously disappeared within 3 years. Case n. 2: Similar swellings of the face developed at the age of 15 days in the brother of case n. 1. The laboratory findings were identical to those for his brother, and there were minimal lytic areas of the vault of skull without any other lesions. At 3 months of age, X-rays confirmed the existence of skull lesions and showed cortical hyperostosis of the left humerus. Similar cortical hyperostosis of nasal bones appeared at the age of 8 months, while the inflammatory syndrome persisted. All findings gradually and spontaneously disappeared within 18 months. CONCLUSIONS: Cortical hyperostosis are usually most prominent in the lower extremities in cases of familial infantile cortical hyperostosis. The lesions were largely confined to the skull in our cases, with no lesions of the mandible. The disease was only identified in the first case after a protracted course with unpredictable remissions and relapses and the discovery of the cortical hyperostosis in his brother.

Humans

The diagnostic value of hyperostosis in midline subfrontal meningioma.

Roentgenologic analysis of 66 patients with proved midline subfrontal meningioma indicates a very high incidence of hyperostosis. A total of 63 patients (95%) showed varying degrees of hyperostosis involving the cribiform plate, planum sphenoidale, or tuberculum sellae (including the chiasmatic sulcus). The planum sphenoidale is the most common site of hyperostosis (59%). In 19 cases (29%), the region of hyperostosis did not correspond to the site of tumor attachment. Mild or localized hyperostosis may be apparent only on tomograms. Small "blistering" and "saw-tooth-like" osteoma formation are reliable early signs of midline subfrontal meningioma; conversely, midline subfrontal meningioma can be virtually ruled out if hyperostosis is absent.

Adult

Juxtasellar hyperostosis of non-meningiomatous origin.

Seven patients are described who had juxtasellar hyperostosis with visual disturbance secondary to non-meningiomatous lesions. Two had chromophobe adenomas, one craniopharyngioma, one carcinoma of the sphenoid sinus, one a thrombosed aneurysm of the intracavernous portion of the internal carotid artery, one epidermoidoma of the orbit, and one chondroblastoma of the anterior clinoid process. The diagnosis of meningioma was entertained initially on the basis of hyperostosis plus visual impairment. Careful evaluation of hyperostosis is essential for correct diagnosis of meningioma, according to our experience. Suprasellar meningiomas almost invariably produce irregular hyperostosis of the planum sphenoidale, often associated with serration and blistering. Sphenoid meningioma, when it is sclerotic, always shows thickening or expansion of the sphenoid wings. Therefore, in the absence of typical meningiomatous hyperostosis, one can readily differentiate non-meningiomatous hyperostosis from true meningioma.

Adenocarcinoma

Stenosis of the lumbar spinal canal in vertebral ankylosing hyperostosis.

Certain morphologic features frequently observed in radiography or computed tomography (CT) scan in patients with hyperostosis led us to study the association between a narrowed spinal canal and vertebral hyperostosis. Twenty-eight items were selected and studied by three different investigators (two rheumatologists and one radiologist) in radiographs and CT scans of 100 patients with acquired stenosis of the lumbar canal, with or without hyperostosis (46 and 54 cases, respectively). The most distinctive points that we suggest can be used as diagnostic criteria of the hyperostotic narrowed lumbar canal are anterior or posterior lateral marginal somatic osseous proliferations, proliferations of the nonarticular aspects of the posterior apophyses, and ossifications of the posterior articular capsule and of the ligaments (yellow ligament, posterior longitudinal ligament, and the supraspinal ligament). Four of these six criteria should be present to establish the diagnosis of hyperostotic lumbar stenosis. The appearance of lumbar hyperostosis on X-ray or CT scans differs from that of simple degenerative changes due to arthrosis, and the hyperostosis can be held responsible for dural compression.

Aged

A radiographic study of porotic hyperostosis.

Skull lesions known as porotic hyperostosis have been of interest to researchers since the mid-19th century. The etiology of porotic hyperostosis has long been a matter for speculation yet there has never been complete acceptance or substantiation of any one of the many theories proposed. Today the most widely accepted theory suggests that anemias of either acquired or genetic origin are responsible for porotic hyperostosis. The present study tests this hypothesis using criteria which were chosen after the examination of clinical radiographs of patients with various types of anemia. These criteria are: the presence of "hair-on-end" trabeculation, outer table thinning, texture changes, diploic thickening, orbital roof thickening, orbital rim changes, and the underdevelopment of frontal sinuses. A comparison of these criteria from the clinical X-rays with X-rays of skulls with porotic hyperostosis provides a more rigorous, repeatable, and standardized method upon which to base a diagnosis. This approach enables radiography to provide the necessary link between the clinical and anthropological with which to investigate the origin of porotic hyperostosis.

Anemia

Bone imaging of sternocostoclavicular hyperostosis in palmoplantar pustulosis.

Sternocostoclavicular hyperostosis is a rare syndrome that is characterized by hyperostosis and soft tissue ossification between the clavicles and the anterior part of the upper ribs. This syndrome frequently is found in the case of palmoplantar pustulosis (PPP), especially in Japan. There have been few published reports, however, of Tc-99m MDP bone imaging findings in PPP. Eleven patients with PPP who were suspected to have sternocostoclavicular hyperostosis were studied with Tc-99m MDP whole body bone imaging. Bone images were abnormal in 11 patients. Abnormal radionuclide concentrations were observed in the sternoclavicular, sternocostal, and manubriosternal joints, in the ribs, and in the sternum. Whole body imaging revealed radionuclide accumulations unexpectedly in other bones such as the vertebrae, femur, tibia, or sacroiliac joints in five of 11 cases. Radiographs were available in nine patients. Three chest radiographs were negative, and six showed various degrees of hyperostosis or sclerotic changes in sternoclavicular, sternocostal, or manubriosternal joints, or in the sternum or anterior upper ribs. These bone lesions usually were more prominent and more easily recognized with bone scintigraphy. Bone scintigraphy should be used as a routine procedure in patients with PPP who are suspected to have sternocostoclavicular hyperostosis.

Adult