Sternocostoclavicular hyperostosis in children: a report of eight cases.

OBJECTIVE: The aim of this paper is to clarify the clinical and radiologic features of sternocostoclavicular hyperostosis by reviewing eight previously unpublished cases in children, identifying its similarities to chronic recurrent multifocal osteomyelitis and the differences between the pediatric and adult population affected with sternocostoclavicular hyperostosis. Appropriate imaging workup will obviate unnecessary diagnostic and therapeutic procedures. MATERIALS AND METHODS: We investigated the clinical and imaging features of sternocostoclavicular hyperostosis in eight children (seven girls and one boy) and compared those features with the characteristic features of chronic recurrent multifocal osteomyelitis and sclerosing Garré's osteomyelitis to determine if sternocostoclavicular hyperostosis can justifiably be classified as a separate entity. All patients underwent one or more bone biopsies to determine the cause of the bone lesion(s). RESULTS: Seven of the eight patients had involvement of the clavicle. Five of the eight patients had associated distant involvement in the pelvis, femur, tibia, fibula, talus, or sacroiliac joints. Except for predominant localization in the anterior chest wall, the symptoms, the clinical and imaging features, and the results of biopsy and histopathologic examination resemble those of chronic recurrent nonspecific sclerosing osteomyelitis. No skin lesion and no causative organism was found in any of the cases. CONCLUSION: Sternocostoclavicular hyperostosis is a descriptive term used to designate a form of chronic sclerosing osteomyelitis. Its only distinctive feature is localization on one or more sites of the anterior chest wall.

[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.

[The acquired hyperostosis syndrome. 2]. / Das akquirierte Hyperostose-Syndrom. Teil 2.

In the second part of this publication, we describe some additional findings in cases of sternocostoclavicular hyperostosis. These include focal hyperostosis of the spine, in the pelvis and in the extremities and psoriatic skin lesions and severe forms of acne (acne conglobata, acne fulminans). Other features, which are not diagnostic, include erosive or non-erosive peripheral arthritis and unilateral or bilateral sacro-iliitis. An analysis of our 13 patients and of the relevant literature indicates that the hyperostosis is due to increased bone metabolism and heterotopic ossification of fibrous tissue and that these are the pathogenic bases of the changes in the axial skeleton, the pelvis and the bones of the extremities. We have suggested a scheme which would categorize the syndrome into complete, incomplete and possibly acquired forms.