Unilateral sternocostoclavicular hyperostosis, a case report
J. Heidotting1 J.J.A.M. van Raay1 1 Orthopaedic Surgery Department, Martini Hospital, Groningen, The Netherlands
Corresponding author: J. Heidotting, Jeffrey_heidotting@live.nl
The incidence of sternocostoclavicular hyperostosis is unknown, because there are no well-defined studies available.1 We report a 73-year-old patient with swelling of the sternoclavicular joint. The aim of this case report is to raise awareness for sternocostoclavicular hyperostosis, which is a rare disease. This case report also gives a recommendation about the differential diagnosis of a swelling of the sternoclavicular joint and treatment based on recent studies.
Patient A 73-year-old woman visited the orthopaedic outpatient clinic with pain in the right shoulder and a swollen right sternoclavicular joint. The shoulder pain was described as a burning sensation. It had started a year and a half earlier, without a direct cause. Abduction and retroflexion were painful, as was lying on the right shoulder. Physiotherapy did not improve the complaints. Rheumatologic diseases were ruled out by a rheumatologist. During the physical examination there was obvious swelling of the right sternoclavicular joint; the left sternoclavicular joint was slightly swollen. Pressure on the right sternoclavicular joint was painful. Movements of the right shoulder were not painful below shoulder level; abduction of the shoulder was painful. Rotations and scapulothoracic movements showed no deviations. An X-ray showed acromioclavicular arthrosis and a normal glenohumeral joint. A CT scan showed an ossification originating from the sternum to the soft tissue ventral of the right sternoclavicular joint with moderate arthrosis (figure 1). Bone scintigraphy was performed and showed an enlarged uptake around the right sternoclavicular joint, similar to half of the ‘bullhead sign’ (figure 2), confirming the diagnosis of unilateral sternocostoclavicular hyperostosis.
Figure 1. Computed tomography of the sternoclavicular joint showing an ossification originating from the sternum into the soft tissue ventral of the sternoclavicular joint (arrow).
Figure 2. Bone scintigraphy showing uptake around the right sternoclavicular joint. Half of the bullhead sign is visible.
Intervention Infiltration of the right sternoclavicular joint with 40 mg triamcinolonacetonide was performed. Comparison The disease of sternocostoclavicular hyperostosis can go unrecognized for years. Reports range from ages of 24 to 79, and there seems to be a predominance among women.3 There also tends to be a higher prevalence in Japanese and Scandinavian countries.4 Some studies show a higher prevalence of autoimmune disease, for example psoriasis vulgaris.5 In sternocostoclavicular hyperostosis inflammation of the sternoclavicular ligaments and surrounding soft tissues occurs.6,7,8 When the inflammatory process continues, it leads to hyperostosis and ossification of the soft tissue. Even fusion of the sternoclavicular joint may occur.6 The CT-scan of our patient showed ossification originating from the sternum into the surrounding soft tissues and bone scintigraphy was performed to confirm the diagnosis. Most cases of sternocostoclavicular hyperostosis present with pain and a palpable, warm swelling in the sternoclavicular region.7,8 Patients often present with a bilateral swelling, but unilateral swelling has also been described.9 Our patient presented with more pain on the right side, also shown on X-ray and bone scintigraphy. The disease shows a pattern of relapsing and remitting.9,10,11 Pain is usually described in the shoulder, neck, or anterior chest wall with an observable swelling.9 Sternocostoclavicular hyperostosis has been described in literature together with pustulotic skin changes and seronegative spondylarthropathy as part of the SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis).8 Some literature reports percentages of up to 53%.2 Our patient did not have the SAPHO syndrome, because there were no signs of pustulotic skin changes. No clear relationship between laboratory tests and disease activity was described.1 Our patient also had arthrosis of the acromioclavicular joint, which can be explained by inadequate freedom of movement of the sternoclavicular joint. This puts additional stress on the other joints in the movement chain of the SC-AC-glenohumeral-scapulothoracic joints, causing faster degeneration of the other joints in the kinematic chain. Radiographic diagnostics show ossification or hyperostosis of the clavicles, sternum and upper ribs.10 In the first stages of the disease joint spaces are preserved but erosive changes may be detected.6,8 Most cases involve the first to the fourth ribs, but cases involving the seventh rib are also described.6,10,11 With computed tomography small hyperostotic foci of at least 5 mm diameter at the sternal end of the first pair of ribs are described as early signs of sternocostoclavicular hyperostosis.8 These findings can be helpful in detecting the disease at an earlier stage. Another frequently used imaging modality is bone scintigraphy. Increased uptake is often found in the sternoclavicular and first and second ribs region.12,13 These configurations have been described multiple times as the bullhead sign,13 which is considered to be pathognomic for bilateral sternocostoclavicular hyperostosis.14 Our patient showed half of the bullhead sign on the right side, which confirmed the diagnosis of sternocostoclavicular hyperostosis. Other diseases in the differential diagnosis with an infectious or neoplastic aetiology must be ruled out.15,16 Sternoclavicular joint infection or subacute osteomyelitis are examples of an infectious aetiology. A Pancoast tumour and metastases of lymphomas can also be considered.8,9,10 Other possible causes are a rheumatoid arthritis, calciumpyrophosphate dihydrate crystal deposition disease, a healing fracture, avascular necrosis of the clavicle, osteitis condensans and posttraumatic arthrosis or subluxation.9,14 A few therapies have been tried to treat sternocostoclavicular hyperostosis. NSAIDs give pain improvement, in varying degrees and durations.9,10,14 Routine use of NSAIDs had no effect on serologic markers or showed no radiographic improvement.14 Injectable calcitonin seemed to improve pain and swelling but did not improve inflammatory markers.17 Antirheumatic drugs, cyclosporine A and TNF-α inhibitors have been tried without significant improvement.1 Bisphosphonates have shown relief of pain and improvement in serologic and radiographic values in case reports,18 although other studies show no lasting reduction in symptom severity and radiologic improvement.19 There is no place for operative treatment in sternocostoclavicular hyperostosis. Though there is no definite treatment for sternocostoclavicular hyperostosis, NSAIDs give pain reduction. Long-term follow-up suggests a good prognosis.9,12 Periods of painful exacerbations may occur in first decade of the disease.9,20 There may be spontaneous remission after the age of 60.21 Outcome Six weeks after the intra-articular injections in the acromioclavicular and sternoclavicular joints our patient was free of pain. Our advice was to start expanding mobility of the shoulder. Recommendations In conclusion, we report a patient with unilateral sternocostoclavicular hyperostosis. It is a rare and often underdiagnosed disease due to low awareness in the physician. Patients tend to present with pain and swelling in the sternoclavicular joint. Physical examination shows swelling of the joint without signs of inflammation. Pressure on the affected joint is painful. Laboratory findings do not usually show abnormalities. A CT scan shows ossification of the sternoclavicular ligaments and surrounding soft tissues. The bullhead sign on bone scintigraphy is pathognomic and confirms the diagnosis. At present there is no definite treatment for sternocostoclavicular hyperostosis. Studies show a positive effect of bisphosphonates and NSAIDs can give adequate pain reduction. The disease is known to have a good prognosis, and spontaneous remissions may occur after the sixth decade in life. Disclosure Statement The authors report no conflicts of interest.
References 1. Carroll MB. Sternocostoclavicular hyperostosis: a review. Ther Adv Musculoskel Dis 2010;0(0)1-10. 2. Chamot AM, Benhamou CL, Kahn MF, Beraneck L, Kaplan G, Prost A. Acne-pustulosis-hyperostosis-osteitis syndrome. Results of a national survey. 85 cases. Rev Rhum Mal Osteoartic 1987;54:187-196 [in French]. 3. Kloot WA van der, Chotkan SA, Kaptein AA, Hamdy NAT. Diagnostic delay in sternocostoclavicular hyperostosis: Impact on various aspects of quality of life. Arthritis Care Res 2010;62:251-257. 4. Johnston KA, Elston DM. Palmoplantar pustulosis associated with sternocostoclavicular hyperostosis. 1998;Cutis 62:75-76. 5. Valkema PA, Luymes CH, Witteveen JE, Cessie S le, Appelman-Dijkstra NM, Hogendoorn PCW, et al. High prevalence of autoimmune disease in the rare inflammatory bone disorder sternocostoclavicular hyperostosis: survey of a Dutch cohort. Orphanet Journal of Rare Diseases 2017;12:20 6. Fritz P, Baldauf G, Wilke HJ, Reitter I. Sternocostoclavicular hyperostosis: Its progression and radiologic features. A study of 12 cases. Ann Rheum Dis 1992;51:658-664. 7. Saghafi M, Henderson J, Buchanan WW. Sternocostoclavicular hyperostosis. Semin Arthritis Rheum 1993;22:215-223. 8. Dihlmann W, Dihlmann SW. Acquired hyperostosis syndrome: Spectrum of manifestations at the sternocostoclavicular region. Radiologic evaluation of 34 cases. Clin Rheumatol 1991;10:250-263. 9. Economou G, Jones PBB, Adams JE, Bernstein RM. Computed tomography in sternocostoclavicular hyperostosis. Br J Radiol 1993;66:1118-1124. 10. Prevo RL, Rasker JJ, Kruijsen MWM. Sternocostoclavicular hyperostosis or pustulotic arthroosteitis. J Rheumatol 1989;16:1602-1605. 11. Sartoris DJ, Schreiman JS, Kerr R, Resnik CS, Resnik D. Sternocostoclavicular hyperostosis: A review and report of 11 cases. Radiology 1986;158: 125-128. 12. Kalke S, Perera SD, Patel ND, Gordon TE, Dasgupta B. The sternoclavicular syndrome: Experience from a district general hospital and results of a national postal survey. Rheumatology 2001;40:170-177. 13. Dihlmann W, Dihlmann SW, Hering L. Acquired hyperostosis syndrome-AHYS (sternocostoclavicular hyperostosis, pustulotic arthro-osteitis, SAPHO-syndrome): Bone scintigraphy of the anterior chest wall. Clin Rheumatol 1997;16:13-24. 14. Freyschmidt J, Sternberg A. The bullhead sign: Scintigraphic pattern of sternocostoclavicular hyperostosis and pustulotic arthroosteitis. Eur Radiol 1998;8:807-812. 15. Dehdashti F, Siegel BA. Case 2: Sternocostoclavicular hyperostosis. Am J Roentgenol 1989;152:1319. 16. Katz ME, Shier CK, Ellis BI, Leisen JCC, Hardy DC, Jundt JW. A unified approach to symptomatic juxtasternal arthritis and enthesitis. Am J Roentgenol 1989;153:327-333. 17. Misaki T, Dokoh S, Mori E. Calcitonin treatment for intersternocostoclavicular ossification: Clinical experience in two cases. Ann Rheum Dis 1991;50:813-816. 18. Ringe JD, Faber H, Farahmand P. Rapid pain relief and remission of sternocostoclavicular hyperostosis after intravenous ibandronate therapy. J Bone Miner Metab 2006;24:87-93. 19. Yachoui R, Kreidy M, Parker BJ. Treatment-Refractory Sternocostoclavicular Hyperostosis Clin Med Res. 2017 Jun;15(1-2):37-40. 20. Köhler H, Uehlinger E, Kutzner J, West, T.B. Sternocostoclavicular hyperostosis: Painful swelling of the sternum, clavicles, and upper ribs. Ann Intern Med 1977;87:192-194. 21. Nungu S, Olerud C, Rehnburg L. Sternocostoclavicular hyperostosis. Upsala J Med Sci 1997;97:177-182.