Sternocostoclavicular Hyperostosis: Positive Clinical and Radiological Response on Pamidronate.

Background: Sternocostoclavicular hyperostosis (SCCH) is a rare disease, constituting a chronic sterile osteomyelitis with elevated bone turnover in the axial skeleton, causing pain and shoulder dysfunction. SCCH severely interferes with daily activities, work, and quality of life. SCCH has a relapse-remitting disease course, but inflammatory-induced sclerotic transformation in the affected area is slowly progressive. Here we present two patients with clinical and radiological diagnosis of SCCH treated with intravenous pamidronate, leading to clinical remission in both, but complete resolution of sclerosis in one of them, which is a novel finding in our experience. Case Presentation: Two adult female SCCH-patients presented with longstanding pain, swelling of the anterior chest wall, and compromised shoulder function. Subsequent single photon emission computed tomography-computed tomography (SPECT/CT) illustrated elevated bone activity and sclerosis in the SC region, with hyperostosis, confirming the diagnosis of SCCH. As symptoms in both patients were eventually refractory to standard painkillers such as non-steroidal anti-inflammatory drugs (NSAIDs), intravenous pamidronate treatment in 3-month cycles was started. Pamidronate was effective in reducing pain and improving shoulder function and also led to decreased bone turnover on skeletal scintigraphy. Sclerosis in the first patient persisted. In the second patient, however, a complete resolution of sclerosis was observed. Conclusions: SCCH remains a rare bone disorder for which no evidence-based therapies are yet available. While disease burden is high, SCCH lacks recognition and is often diagnosed long after symptomatic presentation. As for the cases in this report, pamidronate was successful in reducing symptoms, and in the second case even led to regression of sclerotic changes on CT-imaging.

Treatment-Refractory Sternocostoclavicular Hyperostosis.

Sternocostoclavicular hyperostosis (SCCH) is an infrequent chronic inflammatory disorder of the axial skeleton of unknown origin. SCCH goes often unrecognized due to a low level of awareness for the disorder. It typically presents with relapsing and remitting pain in the shoulder, neck, and anterior chest wall area with occasional swelling and tenderness of the sternoclavicular area. The diagnosis is confirmed radiologically by sclerosis and hyperostosis of the sternoclavicular joints. There have been several reports in which intravenous bisphosphonates and tumor necrosis factor-inhibitors have shown reasonable efficacy in the treatment of this disorder. We report a patient with a long history of SCCH in whom pamidronate 60 mg intravenously every 3 months for 3 years failed to reduce symptom severity and improve radiologic findings.

[Isolated bilateral Sternocostoclavicular Hyperostosis (SCCH) with a pathological clavicular fracture--a 10-year follow-up]. / Isolierte, bilaterale sternokostoklavikuläre Hyperostose (SCCH) mit pathologischer Klavikulafraktur--Ein Verlauf über 10 Jahre.

The presented case demonstrates the clinical and radiological course of a woman suffering from bilateral sternocostoclavicular hyperostosis (SCCH) from early changes to a spontaneous clavicula fracture after 10 years. SCCH is characterised by a chronic recurrent painful swelling of the sternoclavicular region due to an aseptic inflammation and hyperostosis of the clavicula, sternum, upper ribs and the adjacent soft tissues. Frequently the picture is accompanied by cutaneous and other skeletal symptoms. The disease can be part of the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. The chronic process is represented by the typical "bull horn sign" in a bone scan. NSAIDS should represent the first line treatment.

Stress fracture of the clavicle associated with sternocostoclavicular hyperostosis.

We report a case of stress fracture of the clavicle associated with sternocostoclavicular hyperostosis. A 60-year-old man sustained a stress fracture of the right clavicle with no history of trauma. On radiography, hyperostosis of the anterior chest wall and ankylosis of the sternoclavicular joint were evident in addition to the fracture. Fracture healing was uneventful after 2.5 months. Ankylosis of the sternoclavicular joint may have caused increased stress at the midshaft of the clavicle by daily activity or minor trauma. Such a fracture is a rare complication of sternocostoclavicular hyperostosis.

Panclavicular ankylosis in pustulotic arthroosteitis. A case report.

A 54-year-old man who had palmoplantar pustulosis showed bilateral, complete osseous fusion of the sternoclavicular and acromioclavicular joints. No significant abnormality was seen in the glenohumeral joint. The left clavicle had a nonunion develop, whereas the right did not. The elevation of the right shoulder was limited to 100 degrees in active and passive measurements. The total external rotation and internal rotation at the side was 135 degrees. This decreased to 90 degrees at 60 degrees elevation and to 10 degrees at maximum (100 degrees) elevation. Based on the kinematic data on normal shoulders, it was thought that the ankylosis of both ends of the clavicle held the scapula unrotated during the arm movement so that, at the arm to trunk angle of 100 degrees, the position of the humerus relative to the scapula was equivalent to that of a normal shoulder in complete elevation. The current case provided an extremely rare clinical setting where shoulder mobility depended only on glenohumeral motion as a result of the complete loss of scapulothoracic motion.

Palmoplantar pustulosis associated with sternocostoclavicular hyperostosis.

Palmoplantar pustulosis associated with sternocostoclavicular hyperostosis is characterized by asymmetrical and nonerosive involvement of the sternocostoclavicular joint, spine, and peripheral joints in association with palmoplantar pustulosis. This seronegative arthrosteitis has been most frequently described in the rheumatologic literature. Male and female patients are affect equally, but there tends to be a higher prevalence in Japan and Scandinavian countries. We present a case of 50-year-old man who presented with palmoplantar pustulosis that preceded his sternocostoclavicular hyperostotic symptoms by 1 to 2 years.

The bullhead sign: scintigraphic pattern of sternocostoclavicular hyperostosis and pustulotic arthroosteitis.

OBJECTIVE: The purpose of this retrospective study was to examine the value of whole-body nuclear medicine imaging and to evaluate the typical scintigraphic pattern of sternocostoclavicular hyperostosis (SCCH) and/or pustulotic arthroosteitis (PAO). In this entity the correct diagnosis is frequently missed because of uncharacteristic changes in other imaging modalities. MATERIALS AND METHODS: Forty-nine patients (age range 15-65 years old, mean age 36 years) with sternocostoclavicular hyperostosis (SCCH) and/or pustulotic arthroosteitis (PAO) were examined with whole-body scintigraphy and conventional radiography. RESULTS: Forty-three of 49 patients with SCCH/PAO showed a characteristic "bullhead"-like high tracer uptake of the sternocostoclavicular region with the manubrium sterni representing the upper skull and the inflamed sternoclavicular joints corresponding to the horns (= bullhead sign). Scintigraphy revealed additional skeletal manifestations (spondylitis, sacroiliitis, osteitis) in 33 of 49 patients with SCCH and/or PAO. CONCLUSIONS: Bone scintigraphy is the imaging modality of choice for the diagnosis of skeletal involvement in PAO. Nuclear medicine reveals unexpected locations and shows the typical pattern of focal hot spots of the spine, sacroiliac joints and/or appendicular skeleton in the large majority of cases in combination with a bullhead-like tracer uptake of the sternocostoclavicular region. The bullhead sign is the typical and highly specific scintigraphic manifestation of SCCH and PAO in radionuclide bone scans and helps to avoid unnecessary biopsies.

[Spondarthritis hyperostotica pustulo-psoriatica: nosologic study with clinical and radiologic presentation in relation to the SAPHO syndrome]. / Die Spondarthritis hyperostotica pustulo-psoriatica: Nosologische Studie mit klinischer und radiologischer Darstellung innerhalb des SAPHO-Syndroms.

Spondarthritis hyperostotica pustulo-psoriatica (Spond.hyp.pp-Schilling), corresponding nosologically to pustulotic arthroosteitis, is a dermato-skeletal "double system" disease of adults. It consists of the triad (a) palmo-plantar pustulosis (Ppp) or, alternatively, Königsbeck-Barber-type psoriasis, (b) sternocostoclavicular hyperostosis (SCCH), and (c) truncal-skeletal changes with syndesmophite-like, hyperostotic and/or parasyndesmophite-like ossifications of layers of the anterior vertebral ligament taken together in the sense of a desmophytal hyperostosis. There is also a potential for sclerosing inflammatory arthritis of the sacro-iliac joints and "dry" inflammatory arthritis of peripheral joints. Thus, the pustulo-psoriatic terrain seems to have a decisive influence on osseous pathology. A total of 38 cases from a study during the years 1982 to 1992 is analysed with regard to morphological characteristics. Rare cases with diaphyseal and pelvic hyperostotic lesions subsequent to bland sclerosing osteomyelitis constitute an overlapping region to chronic recurrent multifocal osteomyelitis (CRMO) and illustrate the relationship between hyperostotic spondarthritis and CRMO. The syndromes of "acquired hyperostosis" and "SAPHO", the former more radiologically oriented and the latter more clinically oriented, together with mainly CRMO and hyperostotic spondarthritis and its forms, constitute the "Spond.hyp.pp.". Although hyperostosis is a guidepost for the radiologist and SAPHO symptoms are one for the clinician, the syndrome does not represent a diagnosis by itself and requires further differentiation. In this report the entity "Spond.hyp. pp." is considered and required contributions from rheumatologically and osteologically oriented radiologist.

[The "bull horn sign"--scintigraphic pattern in sternocostoclavicular hyperostosis and pustular arthro-osteitis]. / Das "Stierkopf-Zeichen"-szintigraphisches Muster bei sternocostoclaviculärer Hyperostose und Pustulöser Arthroosteitis.

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.

Acquired hyperostosis syndrome--AHYS--(sternocostoclavicular hyperostosis, pustulotic arthro-osteitis, SAPHO-syndrome): bone scintigraphy of the anterior chest wall.

The objective of this report is to provide a description of diagnostically significant scintigraphically recognizable sites and patterns of acquired hyperostosis syndrome (AHYS) on the anterior chest wall (ACW), which is involved in 82% of AHYS patients. In 49/90 of our own AHYS patients, planar bone scans of the ACW were performed with the gamma camera, applying an average of 650 MBq of 99mTc-phosphate complexes. In addition, 53 atraumatic patients with extrathoracic cancer were available for routine whole-body scintigraphy. None of these patients had increased uptake identifiable as metastasis clinically or by imaging modalities in either the ACW or the rest of the skeleton. The scintigraphic involvement of the various morphological ACW structures is described in AHYS. Moreover, attention is called to the diagnostic significance of focal hyperactivities at the anterior end of the 2nd-8th rib of adults, which are in the 5th place with respect to their frequency in AHYS. The diagnostic significance of sternocostal-joint involvement in AHYS can likewise be recognized by bone-scan scintigraphy and will be discussed. Bone scintigraphy is more sensitive than radiomorphological imaging in AHYS. This, however, only applies under three conditions. 1. The increased radiotracer uptake in the upper sternocostoclavicular region must be assessed on both the anterior and the posterior view of the ACW scan. 2. In addition to the anterior view of the routine scintiscan, further anterior scans with reduced scan time of the gamma camera are usually necessary. This ensures better visibility of the involvement of certain morphological structures that are important for AHYS diagnosis. Moreover, a statement can be made about the inflammatory ossifying activity/inactivity of the AHYS on ACW.3. Increased radionuclide uptake in the manubrium sterni and corpus sterni on the anterior scan should be verified by additional lateral or oblique scans of the thorax (sternum).

Osseous manifestations of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome.

The SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a complex group of disorders characterized by peculiar bone lesions, most commonly involving the anterior chest wall, and sometimes accompanied by dermatologic manifestations. The clinical and radiographic features of this syndrome are well described, but few studies have examined the histologic features of the bone lesions. We describe the clinical, radiographic, and histologic features of the osseous lesions encountered in eight patients with a clinical diagnosis of SAPHO syndrome. The patients included five female and three male patients ranging in age from 5 to 63 years (mean, 35.3 years). The most common clinical presentation was pain related to the sites of osseous involvement. Two patients also had some form of pustular dermatosis. The radiographic features of the osseous lesions varied but often suggested the possibility of a neoplasm. Nine pathologic specimens were available for review, five from the clavicle, two from the first rib, one from the calcaneus/cuboid, and one from the tibia. The histologic features varied but seemed related to the duration of the patients' musculoskeletal symptoms. Early lesions contained acute inflammation, edema, and prominent periosteal bone formation, histologically indistinguishable from ordinary bacterial osteomyelitis, whereas late lesions demonstrated markedly sclerotic bone trabeculae with prominent marrow fibrosis and only mild chronic inflammation; one of these biopsies appeared virtually identical to Paget's disease. One biopsy was performed after an intermediate duration of symptoms and contained prominent chronic inflammation only. The histologic findings in SAPHO are variable and nonspecific and may depend on the duration of disease, but it is important to recognize the spectrum of histologic changes possible in the syndrome and to realize that clinicopathologic correlation is necessary to avoid misdiagnosis and unnecessary long-term antibiotic therapy.

[Sternocostoclavicular hyperostosis]. / Die sternokostoklavikuläre Hyperostose.

HISTORY AND CLINICAL FINDINGS: A 35-year-old previously healthy man developed a painful swelling in the area of the left sternoclavicular joint (SCJ), restricting movement. There had been no history of trauma. INVESTIGATIONS: A moderately increased erythrocyte sedimentation rate was the only abnormal finding among the usual laboratory tests. Radiologically there were marked hyperostotic changes in the area of the left upper thoracic cage with synostosis of the SCJ. Biopsy of the left clavicle showed non-specific chronic sclerosing osteomyelitis. TREATMENT AND COURSE: Over the next 6 years the SCJ became completely ankylosed and there were now extensive fibroses, some presternal and some in the upper mediastinum with thrombosis of the left subclavian, axillary and jugular veins causing inflow occlusion (Paget-von-Schroetter-syndrome). Lymph nodes in the region of the mandible, jugular veins bilaterally and mediastinum were noted for the first time, remaining unchanged in size over six months. CONCLUSION: Sternoclavicular hyperostosis is an important condition in the differential diagnosis of inflammatory or malignant processes of this joint.