Stress fracture of the clavicle associated with sternocostoclavicular hyperostosis combined with bony ankylosis of the acromioclavicular joint and ossification of the coracoclavicular ligament.

Sternocostoclavicular hyperostosis (SCCH) has been reported in patients with pustulotic arthro-osteitis, but there are few reports of marked ossification of the lateral part of the clavicle. Here, we report a case of stress fracture in a patient with SCCH with marked ossification of the lateral part of the clavicle. In this case, the clavicular fracture was proximal and no dislocation. Conservative treatment with a clavicle band and the administration of corticosteroids resulted in rapid symptom improvement. Eight months later, the patient had no difficulty in daily life, but X-rays showed that bone fusion was not complete. Therefore, it is necessary to carefully follow-up such patients for any recurrence of symptoms and radiographic changes.

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.

[Coronary Artery Bypass Grafting complicated with sternocostoclavicular hyperostosis;Report of a Case].

The patient was 72-year-old man, who had old myocardial infarction, diabetes mellitus, dyslipidemia, hypertension and chronic obstructive pulmonary disease. He was complicated with congestive heart failure, and multi-vessel coronary artery disease and moderate aortic valve stenosis and regurgitation were diagnosed. We performed coronary artery bypass grafting(CABG) and aortic valve replacement. Intraoperative findings showed severe adhesions and tissue fibrosis around sternum. It was very difficult to dissect adhesions around left internal thoracic artery (LITA). LITA was injured, and great saphenous vein was anastomosed to left anterior descending coronary artery. Sternocostoclavicular hyperostosis was diagnosed on computed tomography( CT) findings such as remarkably thickened ster-num and adhesion of sternoclavicular joint. Postoperative course was complicated by osteomyelysis and necrosis of left side skin incision, for which omentopexy was needed. Sternocostoclavicular hyperostosis is rare disease, but we should recognize in preoperative evaluation.

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.

Radiologists need to be aware of secondary central venous stenosis in patients with SAPHO syndrome.

OBJECTIVES: We aimed to define central venous stenosis (CVS) caused by sternocostoclavicular hyperostosis as a feature of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome on routine contrast-enhanced computed tomography (CT) images. The relationship between SAPHO syndrome and CVS without venous thrombosis caused by anterior chest wall compression has not been investigated. Therefore, the present study evaluated CVS in patients with SAPHO syndrome at our hospital. METHODS: We retrospectively reviewed contrast-enhanced CT images of ten patients with suspected or diagnosed SAPHO syndrome between January 2007 and November 2015. The patients were assessed by contrast-enhanced CT using 16-, 64- or 128-detector row scanners. Two radiologists independently assessed the presence of CVS or obstruction and SAPHO syndrome in a retrospective review of CT images. RESULTS: Six of the ten patients had findings of CVS with SAPHO syndrome. The mean diameter and patency rate at the site of CVS were 1.88 mm and 27.2%, respectively. Stenosis was more significant in terms of the mean diameter of CVS sites than of stenotic sites that crossed the anteroposterior vein (p < 0.05). CONCLUSIONS: Radiologists who routinely assess contrast-enhanced CT images should be aware that sternocostoclavicular hyperostosis with SAPHO syndrome could cause secondary CVS. KEY POINTS: • SAPHO syndrome can cause central venous stenosis. • Radiologists should consider central venous stenosis in patients with SAPHO syndrome. • Early diagnosis of central venous stenosis due to SAPHO syndrome is challenging.

High prevalence of autoimmune disease in the rare inflammatory bone disorder sternocostoclavicular hyperostosis: survey of a Dutch cohort.

BACKGROUND: Sternocostoclavicular hyperostosis (SCCH; ORPHA178311) is a rare inflammatory disorder of the axial skeleton, the precise pathophysiology of which remains to be established. We addressed the potential association of SCCH with autoimmune processes by evaluating the lifetime prevalence of autoimmune disease in 70 patients with adult-onset SCCH and 518 SCCH-unaffected first-degree relatives (parents, siblings and children). Danish hospital registry data for autoimmune diseases were used as reference data. RESULTS: The mean age of interviewed patients was 56.3 years (range 26-80 years) and 86% were female. Interviewed patients belonged to 63 families, with four families having clusters of 2-3 patients. A diagnosis of at least one autoimmune disease was reported in 20 SCCH patients (29%) and in 47 relatives (9.1%), compared to an estimated 3.9% prevalence of autoimmune disease in the Danish reference population. A diversity of autoimmune diseases was reported in SCCH patients and relatives, most frequently psoriasis vulgaris (14%). Palmoplantar pustulosis was reported by 28 patients (40%). In SCCH patients, inclusion of palmoplantar pustulosis as putative autoimmune disease increased the overall prevalence to 54%. CONCLUSIONS: The high prevalence of autoimmune disease in patients with sternocostoclavicular hyperostosis and their first-degree relatives suggests that autoimmunity may play a role in the still elusive pathophysiology of the intriguing osteogenic response to inflammation observed in this rare bone disorder.

Prominencia esternoclavicular: hiperostosis u osificación interarticular ¿Un cajón de sastre? / Sterno-clavicular prominence: hyperostosis or interarticular ossifiction. ¿it is a ragbag?

Introducción. En la práctica clínica observamos con cierta frecuencia una prominencia esternoclavicular dolorosa, deformidad que constituye el principal signo de dos entidades con baja incidencia, aunque bien definidas: la hiperostosis esterno-costo-clavicular (HECC) y la osificación inter-esterno-costo-clavicular (OIECC). El diagnóstico diferencial de estas afecciones debe incluir la artrosis esterno-clavicular, la enfermedad de Paget, la osteitis condensante, la osteomielitis, la pustulosis, la enfermedad de Friederich, el síndrome de Tietze y el osteoma osteoide. Esto es especialmente importante en los casos en los que la afectación sea unilateral. Material y método. Presentamos una serie de nueve pacientes cuyo motivo de consulta fue una prominencia esterno-costo-clavicular, compatible con HECC u OIECC. El motivo de consulta en la mayoría de los pacientes fue el descartar la presencia de un tumor en esa localización. Resultados. Los estudios radiológicos mostraron un aumento variable tanto de la densidad como de la masa ósea, así como diferentes intensidades de afectación de la articulación esterno-costo-clavicular. Discusión. Descartado un tumor y con un diagnóstico concreto de la causa de la prominencia, generalmente, se considera suficiente con el tratamiento antiinflamatorio en la mayoría de los pacientes. En la práctica clínica parece irrelevante la diferenciación entre HECC y OIECC, ya que el tratamiento y el pronóstico de ambas afecciones son superponibles. La biopsia de la articulación y los procedimientos diagnósticos invasivos pueden ser innecesarios(AU)

Introduction. In clinical practice, we see quite often painful sterno-clavicular prominence, deformity, which is the hallmark of two entities with a low incidence, although well defined hyperostosis cost sterno-clavicular (HECC) and ossification cost inter-sterno-clavicular (OIECC). The differential diagnosis of these conditions, you must include sterno-clavicular arthritis, Paget's disease, condensing osteitis, osteomyelitis, pustulosis, Friederich's disease, Tietze's syndrome and osteoid osteoma. This is especially important in cases where the involvement is unilateral. Material and methods. We present a series of nine patients complaining of a painful sterno- clavicular prominence, compatible with HECC or OIECC. The reason for consultation in most patients was rule out the presence of a tumor in that location. Results. Image studies showed a variable increase both the density and bone mass as well as different intensities of joint involvement sterno-clavicular cost. Conclusion. Ruled out a tumor and a specific diagnosis of these, you do not need aggressive treatment, is generally considered sufficient anti-inflammatory treatment in most patients. In clinical practice, it is irrelevant and OIECC HECC differentiation, since treatment and prognosis of both conditions are similar. The biopsy of the joint and aggressive diagnostic procedures may be unnecessary (AU)

[Case of SAPHO syndrome with significant sternocostoclavicular hyperostosis].

A 59-year-old man with hypertension developed general fatigue, fever, and precordialgia in early March 2007. An antibacterial agent was intravenously administered; however, no improvement in his symptoms or laboratory findings was observed. He had acne and pustulosis, and radiographs and CT of the chest revealed sternocostoclavicular hyperostosis. As a result, the diagnosis was revised to SAPHO syndrome, and he improved with steroid administration. SAPHO syndrome is a condition that is rarely found at the initial examination in the Department of Internal Medicine. We report a case with a discussion of the literature.

The psychological burden of an initially unexplained illness: patients with sternocostoclavicular hyperostosis before and after delayed diagnosis.

BACKGROUND: Sternocostoclavicular hyperostosis (SCCH) is a rare, debilitating, chronic inflammatory disorder of the anterior chest wall due to a chronic sterile osteomyelitis of unknown origin. SCCH is largely underdiagnosed and often misdiagnosed. In individual cases it can remain unrecognized for years. The purpose of this study is twofold. Firstly, to evaluate the psychological condition of SCCH patients, both in the sometimes quite extended pre-diagnostic period between first manifestations and confirmed diagnosis of the disease, and in the current situation. Secondly, to investigate the relationships between the pre-diagnostic and the current psychological conditions of confirmed SCCH patients. METHODS: Structured interviews were held with 52 confirmed SCCH patients. Questionnaires were included to assess posttraumatic stress symptoms, social support, aspects of pain, illness perceptions, self-reported health status, and quality of life. RESULTS: SCCH patients reported stronger posttraumatic stress symptoms, more unfavorable illness perceptions, lower health status, and poorer quality of life than healthy individuals and patients with other diseases or traumatic experiences. Psychological distress in the pre-diagnostic period was associated with unfavorable conditions in the current situation. CONCLUSION: SCCH is an illness with serious psychological consequences. Psychological monitoring of patients with unexplained complaints is recommended as long as a diagnosis has not been reached.

Diagnostic delay in sternocostoclavicular hyperostosis: impact on various aspects of quality of life.

OBJECTIVE: Sternocostoclavicular hyperostosis (SCCH) is a rare, debilitating, chronic inflammatory disorder of the anterior chest wall due to a chronic sterile osteomyelitis of unknown origin, often associated with characteristic skin lesions of palms and soles: pustulosis palmoplantaris. SCCH goes often unrecognized for years before the diagnosis is established and treatment instituted. The objective of this study was to trace the diagnostic paths of patients with SCCH and to investigate the consequences associated with diagnostic delay. METHODS: Data were collected through structured interviews of 52 patients with a clinically, scintigraphically, and radiologically established diagnosis of SCCH. RESULTS: The majority of patients presented with swelling and/or pain in the sternocostoclavicular region and/or limited movement of the shoulder girdle. Pustulosis palmoplantaris was present in approximately 30% of patients. The disease went unrecognized for a median of 3.5 years. Patients were often seen by at least 3 members of the medical profession before the diagnosis was suspected and eventually established. Lack of recognition of the clinical manifestations of the disease and delay in diagnosis were associated with important physical, psychological, and socioeconomic consequences affecting quality of life. CONCLUSION: SCCH remains an ill-recognized disease despite its characteristic clinical features. A low level of awareness of the disorder leads to a delay in diagnosis, which has a significant impact on various aspects of quality of life. Awareness should be raised for this disorder, enabling timely diagnosis and initiation of treatment to prevent the irreversible physical and psychological sequelae associated with the protracted untreated state.

Asymptomatic enlargement of the clavicle: a review of underlying aetiologies.

An asymptomatic enlargement of the medial clavicle is a condition that is often both missed and misdiagnosed. We review the most common causes of an isolated and asymptomatic enlargement of the medial clavicle. Underlying aetiologies include osteoarthritis of the sternoclavicular joint, condensing osteitis, spontaneous dislocation of the sternoclavicular joint, and sternocostoclavicular hyperostosis. Key points in the history and physical examination as well as characteristic radiographic findings are sufficient for correct diagnoses. Treatment is conservative.

Clinical and radiologic evolution of synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: a single center study of a cohort of 71 subjects.

OBJECTIVE: To assess the basic features and outcomes of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. METHODS: We identified all patients seen in our unit between 1990 and 2008 diagnosed according to the proposed inclusion criteria with SAPHO syndrome, who had a followup of at least 2 years. RESULTS: Seventy-one patients (48 women, 23 men) with SAPHO syndrome were identified. The median disease duration at the end of followup was 10 years (interquartile range [IQR] 7-15 years), and the median followup duration was 11 years (IQR 6-11.5 years). Six patients were diagnosed with Crohn's disease. Fourteen patients had never had cutaneous involvement, but 8 patients presented >1 skin manifestation. Nine patients (13%) presented a limited (<6 months) monophasic disease course, 25 cases (35%) had a relapsing-remitting course, and 37 patients (52%) had an acute painful phase with a prolonged course lasting >6 months. A total of 4% of the patients were HLA-B27 positive. Female sex (odds ratio [OR] 7.2, 95% confidence interval [95% CI] 2.2-22.9) and the presence at onset of anterior chest wall (ACW) involvement (OR 5.7, 95% CI 1.8-18.1), peripheral synovitis (P = 0.0036), skin involvement (OR 10.3, 95% CI 3.4-31.1), and high values of acute-phase reactants (OR 7.7, 95% CI 2.7-22) were correlated with a chronic disease course and involvement of new osteoarticular sites. CONCLUSION: A chronic course is the more common evolution of SAPHO syndrome. Female sex, elevated erythrocyte sedimentation rate and C-reactive protein values, ACW involvement, peripheral synovitis, and skin involvement at the onset seem to be associated with a chronic course.

SAPHO syndrome: misdiagnosed and operated.

SAPHO is a rare disorder that results in synovitis, acne, pustulosis, hyperostosis and osteitis. Patients with this syndrome typically present with musculoskeletal complaints, frequently localized to the anterior chest wall. However, diagnosis can be difficult in case of involvement of only one symptomatic bone without skin lesions. Awareness of SAPHO syndrome is necessary for accurate diagnosis and to prevent inappropriate and unnecessary treatment.

Tonsillar focal infectious disease involving IgA nephropathy, pustulosis, and ossification.

We report a rare case of IgA nephropathy (IgAN), that was considered as showing tonsillar focal infection, involving pulmoplantar pustulosis (PPP), and sternocostoclavicular hyperosteosis (SCCH). A 53-year-old man with a 3-year history of PPP had hematuria and proteinuria, and he sometimes had anterior chest pain. He was also diagnosed with IgAN and SCCH. We performed tonsillectomy as a treatment. The tonsillectomy was done with the patient under general anesthesia, and this treatment was followed by steroid therapy. Interestingly, all the symptoms of IgAN, PPP, and SCCH were alleviated 6 months after the tonsillectomy. Thus, tonsillectomy and steroid therapy may be effective and could be considered as treatment for these diseases.

In SAPHO syndrome anti-TNF-alpha therapy may induce persistent amelioration of osteoarticular complaints, but may exacerbate cutaneous manifestations.

OBJECTIVES: SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a rare disease combining skin, bone and joint manifestations. In recent years new therapeutic strategies have been tried, among them TNF-alpha-blocking agents. We report our experience with infliximab in four cases of SAPHO syndrome refractory to conventional therapies. METHODS: Between 2002 and 2005, four cases of SAPHO syndrome (two females and two males; mean age 49.7 yr) responding poorly to conventional drugs were treated with infliximab. The dose was 5 mg/kg, according to the protocol used in spondyloarthropathies, with infusions at 0, 2 and 6 weeks followed by 6 weeks intervals. No active cutaneous manifestations were present at the time of starting therapy. RESULTS: Complete remission of osteoarticular involvement was achieved after the second or third infusion, and the positive response was maintained for up to 12 months. A patient relapsed after discontinuation of infliximab, because of infectious complication. Palmoplantaris pustulosis relapsed in two patients after three and six infusions, respectively; there was slight improvement after discontinuation of anti-TNF-alpha drugs. CONCLUSIONS: Infliximab seems to be a very effective therapy for osteoarticular complaints of SAPHO syndrome. Cutaneous involvement responded less favourably, palmoplantaris pustulosis relapse being a possible complication.

Rapid pain relief and remission of sternocostoclavicular hyperostosis after intravenous ibandronate therapy.

Sternocostoclavicular hyperostosis (SCCH) is an infrequent but painful, localized disturbance of bone metabolism of unknown etiology. The diagnosis of SCCH is generally one of exclusion, and it is therefore frequently missed or delayed, leaving patients with pain that frequently fails to respond to standard analgesic therapy. Consequently, SCCH leads to significantly impaired quality of life. Characteristic increased localized bone turnover and inflammatory osteitis provide a strong rationale for using intravenous bisphosphonates to treat the condition. We report on three patients with long-standing, treatment-refractory SCCH in whom intravenous ibandronate injections (a single administration of 4 mg followed by 2 mg every 3 months for up to a year) produced prompt, dramatic, persistent pain relief and resolution of the other symptoms of the disease. We also review recent evidence suggesting that SCCH is more common than generally believed and that technetium-99 bone scanning can aid in making an accurate diagnosis.