Sternocostoclavicular Hyperostosis: Positive Clinical and Radiological Response on Pamidronate

BackgroundSternocostoclavicular 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 PresentationTwo 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.ConclusionsSCCH 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.

Sternocostoclavicular Hyperostosis: An Insufficiently Recognized Clinical Entity

A 79-year-old male chronic hemodialysis patient with no history of central venous catheterization was referred to our hospital with progressive swelling of the left upper limb ipsilateral to a forearm arteriovenous fistula. Radiological assessments revealed marked hyperostosis in the ribs, sternum, and clavicles with well-developed ossification of the sternocostoclavicular ligaments. Such characteristic structural abnormalities and our failure to identify the left subclavian vein with contrast material despite the abundant dilated collaterals in the left shoulder area encouraged us to diagnose our patient with sternocostoclavicular hyperostosis (SCCH) complicated by central vein obstruction. The structural impact of the sternocostoclavicular region as a potential risk for inducing central vein obstruction and the diagnostic concerns of SCCH in this patient are also discussed.