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[The "bull horn sign"--scintigraphic pattern in sternocostoclavicular hyperostosis and pustular arthro-osteitis].

27 patients with sternocostoclavicular hyperostosis (SCC) and/or pustulotic arthroosteitis (PAO) were examined with whole body scintigraphy, conventional radiography, and other imaging modalities, such as CT, MRI. 25 of 27 patients with SCCH showed a characteristic high bullhorn-like uptake of the sternocostoclavicular region with the manubrium sterni representing the skull and the inflamed sternocostoclavicular joints corresponding to the horns (= bullhorn-sign). Scintigraphy revealed additional skeletal manifestations (spondylitis, sacroilitis, osteitis, periostitis) in 19 of the 27 patients with SCCH and/ or PAO. In combination with PPP or psoriasis pustulosa, the typical scintigraphic bullhorn pattern enables the diagnosis of PAO (19 patients) with high confidence. Patients with SCCH but without skin disease at the time of presentation (8 of 27 patients) may develop PPP later and, therefore, it is justified to classify them as incomplete PAO with high risk to develop other skeletal manifestations later in the course of the disease.

Adult↗

[SAPHO syndrome: clinico-rheumatologic and radiologic differentiation and classification of a patient sample of 86 cases].

Synovitis (inflammatory arthritis), acne (pustulosa), pustulosis (psoriasis, palmoplantar pustulosis), hyperostosis (acquired), and ostitis (bland osteomyelitis) are symptoms forming the acronym SAPHO, which is a syndrome of nosologic heterogeneity. All entities forming the SAPHO syndrome are connected by a non-obligate dermatoskeletal association with an aseptic pustulous character. 86 cases were analyzed clinically, radiologically and by histology/histopathology. 31 adult patients showed the typical triad of pustulosis palmo-plantaris (psoriatica, PPP), sterno-costo-clavicular hyperostosis (SCCH), and "productive" spondylopathy, which we define as entity I. spondarthritis hyperostotica pustulopsoriatica (Spond.hyp.pp). Twelve adolescent and 13 adult patients showed entity no. II: chronic recurrent multifocal osteomyelitis (CRMO), being characterized by non-purulent osteomyelitis of plasma-cell sclerotic type, potentially being a reactive inflammatory process. 50% of the adult patients with CRMO showed PPP. Differentiation between these two entities is possible by detection of ossifying enthesiopathy in cases of Spond. hyp.pp and primarily chronic osteomyelitis in cases of CRMO. Two more entities or abortive forms of group I and II are III: the inflammatory syndrome of the anterior chest-wall (ACW syndrome) and IV: the more productive form of isolated sterno-costoclavicular hyperostosis (SCCH). Both are connected quite frequently to HLA-B-27-independent forms of spondarthritis and to pustulous dermatosis. More rarely we find osteo-articular symptoms in cases of acne pustulosa, which form group V: acne-associated spondarthritis and CRMO in the case of acne. Adult forms of CRMO with different forms of appearance (lumosacro-iliac hyperostosis with retroperitobeal fibrosis, pelvic type with affection of the hip-joint) are described. The immunologic theory of a "reactive osteomyelitis" potentially triggered by saprophytes is described. The inverse acne triad is brought in a context of skin symptoms. A case of intercurrent postpartum symptoms together with ulcerative colitis is described. Three cases of patients with Crohn's disease are described. Clinical features, radiological findings, and histopathological elements are brought together to determine the connections between the different entities and the possibilities of differentiation. With these elements together with bone-scan, it is often not necessary to obtain a bone specimen. Therapeutical possibilities, especially concerning CRMO, are discussed. "SAPHO syndrome" is more a sign-post on the way to a more subtle diagnosis when it comes to hyperostotic, skin-associated diseases, and it needs interdisciplinary work to clear the situation.

Acquired Hyperostosis Syndrome↗

Hemophilic pseudotumor. Diagnosis, treatment, and complications.

Patients with hemophilia are living longer as a result of improved therapeutic measures. Associated with this longevity is the increased occurrence of complications affecting the osseous system. One of the more serious bone complications is the hemophilic pseudotumor or hemophilic cyst. We studied its diagnosis, treatment, and complications, and report a case of an early hemophilic cyst occurring in a new location, the proximal part of the left clavicle. The increased incidence of this complication should alert the clinician to the possibility of these lesions appearing in previously unreported sites and demonstrating atypical roentgenographic features. Early recognition and treatment is essential if optimal therapeutic results are to be obtained.

Adult↗

Formation of new myotubes occurs exclusively at the multiple innervation zones of an embryonic large muscle.

This work examines the general principle of whether production of embryonic muscle fibres is invariably linked to sites of innervation, as we have previously reported in small rodent muscles (Duxson et al. [1989] Development 107:743-750). The experimental strategy has been to make a detailed electron microscopic analysis of the formation of new myotubes in a large muscle having multiple, discrete innervation zones. The particular model system is the guinea pig sternomastoid muscle, a strap-like, parallel-fibred muscle with four distinct endplate bands, both in the embryo and the adult. Primary myotubes in the developing muscle extended from tendon to tendon of the muscle and were innervated at each of the multiple endplate zones. Each point of innervation of the primary myotubes was a focus around which many new secondary myotubes formed, and each secondary myotube was approximately centred on one of the innervation sites of its supporting primary myotube. This confirms our previous report, in rat IVth lumbrical muscle, of an invariable association between sites of formation of new secondary myotubes and sites of innervation. We suggest that, in vivo, nerve terminals either directly induce the initial myoblast fusions which give rise to new secondary myotubes, or induce some precondition for fusion. An alternative hypothesis is that a common patterning influence in the muscle localizes both innervation and secondary myotube formation to the same zone. The pattern of secondary myotube production in the embryo has important implications for the size and final architecture of muscles in larger animals, and some of these are discussed.

Acetylcholinesterase↗

The microscopic structure of fibrous articular surfaces: a review.

The present knowledge of the microscopic structure of fibrous articulating surface is reviewed. The influence of dissection, postmortem change, fixation, and dehydration on the microscopic appearances of these surfaces is considered. The results of those studies of surface morphology that investigate intact, supported specimens and that are controlled by surveys of fresh, unfixed, or frozen material are advantageous. New information on the effects of alterations in intermaxillary relationship on the morphology of fibrous articulating surfaces is presented. There is need for further work in this field of research.

Animals↗

Osteomyelitis of the clavicle: clinical, radiologic, and bacteriologic findings in ten patients.

Most lesions of the clavicle are traumatic and pose few diagnostic difficulties. Nontraumatic clavicular lesions, on the other hand, are rare and frequently present problems in diagnosis. This report reviews the clinical, radiologic, and bacteriologic findings in ten patients, six of whom were diagnosed as having acute osteomyelitis and four chronic osteomyelitis. The differential diagnosis of clavicular osteomyelitis is also discussed. The clinical duration of the infectious process in these patients ranged from 2 weeks to 1.5 years. All patients presented with pain; six had fever, three had localized swelling or a mass, and three had soft tissue abscesses. The radiographic findings also varied: the lesion was predominantly sclerotic in four patients, lytic in three, and mixed in two patients; in the one patient in whom magnetic resonance imaging was the only imaging study performed, these features could not be properly evaluated. Periosteal reaction was detected in three patients. Staphylococcus aureus was the causal organism in four patients, while in the remaining six patients different microorganisms were cultured, including Coccidiodes immitis and Mycobacterium tuberculosis. Six patients required biopsy for final diagnosis. Although clavicular osteomyelitis is rare, particularly in adults, it should be considered in the differential diagnosis of a clavicular lesion. The final diagnosis often depends on the results of biopsy and cultures.

Acute Disease↗

The acquired hyperostosis syndrome: a little known skeletal disorder with distinctive radiological and clinical features.

The acquired hyperostosis syndrome (AHS) is a chronic inflammatory disorder of bone of unknown etiology. It is accompanied by circumscribed hyperostosis which can be associated with ossifying lesions at sites of tendinous and ligamentary insertions and erosive or non-erosive arthritis. The predominant location of lesions is the sternocostoclavicular region (approximately 80% of patients), less frequent are involvement of the spine, pelvis, and appendicular skeleton. In 20%-60% of cases AHS is associated with palmoplantar pustulosis, psoriasis, or severe acne (acne fulminans or conglobata). The X-ray appearance of AHS is a more or less homogeneous increase in density with blurred margins, which on scintiscan with labeled phosphate compounds is associated with intense accretion of tracer. These features are associated with a variable increase in the acute phase reactants and a conspicuously low increase, if any, in serum alkaline phosphatase. The therapeutic modalities which have been used so far are entirely symptomatic. Long-lasting improvement has been reported following percutaneous anti-inflammatory radiation therapy.

Acne Vulgaris↗

Computed tomography of the sternum and its articulations.

The chest wall present diagnostic difficulties for both the clinician and the radiologist. Because of normal variations in anatomy and ossification, analysis of the sternal region can be particularly confusing. We reviewed the normal computed tomographic (CT) appearance of the sternum in 354 patients. Important normal sternal variants included cortical unsharpness along the posterior aspect of the manubrium , lateral surfaces of the body, and at the sternal fibrocartilaginous articulations; soft tissue prominence at the junction of the sternum and costochondral cartilage; and bony sclerosis at the transitions from manubrium to body and from body to xiphoid. In seven patients with clinically significant sternal abnormality, key CT features were abnormal soft tissue mass (7/7), destruction or irregularity of the cortical contour (7/7), and abnormal increased attenuation of bone (1/7). CT should be the radiologic study of choice in patients with suspected abnormality of the sternum and its articulations.

Adolescent↗

Ankylosing spondylitis presenting as juxta-articular masses in females.

Juxta-articular inflammatory masses, sternomanubrial or sternoclavicular, were noted in two women who were subsequently found to have ankylosing spondylitis. The differential diagnosis of juxta-articular masses should include systemic rheumatic disorders as well as tumor or infection.

Adult↗

Acquired hyperostosis syndrome: spectrum of manifestations at the sternocostoclavicular region. Radiologic evaluation of 34 cases.

Thirty-four patients with chest wall hyperostosis, a condition which has been designated by various terms in the literature were evaluated radiologically. We prefer the name acquired hyperostosis syndrome (AHS), which we categorize into the complete, incomplete and possible form. In complete AHS, sternocostoclavicular hyperostosis is associated with axial and/or peripheral (endosteal, periosteal, enthesopathic, metaplastic) hyperostosis and with psoriasiform or acneform dermatosis. In addition, these three manifestations are accompanied by erosive or non-erosive peripheral and/or axial arthritis to a variable degree. Sometimes, concomitant findings which are consistent with ankylosing spondylitis are also to be found in the axial skeleton. AHS is manifested at 11 different sites on the anterior chest wall. Ossification forms of the costal cartilage, inflammatory enthesopathies (three different insertions) and focal hyperostoses as well as processes of remodelling of the ribs, clavicles and sternum which are described in detail have particular diagnostic significance. AHS can start simultaneously at one, two or several sites on the anterior chest wall. Conventional tomography (possibly supplemented by CT) is necessary for early diagnosis and for analysis of the various findings on the anterior chest wall.

Adult↗

SAPHO syndrome: 20-year follow-up.

Considerable attention has been paid in the past 10 years to the radiological spectrum of disease entities belonging to the SAPHO syndrome. We report an unusual case presenting with an extra-axial (femoral) lesion, prior to description of this syndrome, which was radiologically and histologically mistaken for a parosteal osteosarcoma. Nineteen years later, a further lesion developed in the scapula together with the typical sternoclavicular manifestations, at which stage the correct diagnosis of SAPHO syndrome was established.

Acquired Hyperostosis Syndrome↗