Desimpel, Julie

Vanhoenacker, Filip

English

Ghent University Academic Bibliography

Case 14212 Chronic Recurrent Multifocal Osteomyelitis (CRMO) of theclavicleJulie Desimpel , Filip M. Vanhoenacker1, 2 1, 2, 31. Department of radiology, Antwerp University Hospital, Edegem, Belgium2. Department of radiology, General Hospital Sint-Maarten, Duffel-Mechelen, Belgium3. Department of radiology, Gent University Hospital, BelgiumUniversity Hospital Antwerp, Department of Radiology, University Hospital Antwerp;Wilrijkstraat, 10 2650 Antwerp, Belgium; Email:filip.vanhoenacker@telenet.be University Hospital Antwerp  Musculoskeletal System Section: 2017, Jan. 7 Published: 11 year(s), femalePatient:Clinical HistoryAn 11-year-old girl presented with a progressive, painless swelling of the medial side of the rightclavicle (Fig. 1). There are no systemic complaints and her previous medical history isunremarkable.Imaging FindingsStandard radiography shows bone expansion at the medial side of the right clavicle. There isextensive bone sclerosis with a solid periosteal reaction (Fig. 2).Subsequent Computed Tomography (CT) confirms the sclerotic expansile lesion in the right medialclavicle (Fig. 3). Magnetic Resonance Imaging (MRI) shows hypo-intensity of the lesion comparedto normal bone marrow on T1-weighted image (Fig. 4) and hypo- intense signal compared to fluidon fat suppressed (FS) T2-weighted image (Fig. 5). Slightly heterogeneous enhancement is seen onT1-weighted image after gadolinium contrast administration (Fig. 6). Biopsy of the lesion revealsno arguments in favor of malignancy on histopathological examination. Culture of the biopsyspecimen is negative with absence of growth of micro-organisms. Upon careful retro-anamnesis, thepatient remembers having felt vague pain episodes at the right knee and ankle. Complementaryconventional radiography show inhomogenous sclerosis at the distal femoral metadiaphysis (Fig. 7)and distal tibial metadiaphysis (Fig. 8).DiscussionCRMO is an auto-immune disease with recurrent inflammatory lesions of the bone and can beclassified in the spectrum of spondyloarthropathy. The first case was reported by Giedion in1972[1]. Since then 250 cases have been reported but the true prevalence is estimated to be higher.CRMO typically affects children while the adult counterpart of the disease is known as SAPHOsyndrome (Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis). Patients with CRMO typically present with complaints of progressive swelling and pain. Theproximal or distal tibial metaphysis, distal femoral metaphysis or medial clavicle are sites ofpredilection. If the clavicle is affected this may lead to reduced shoulder mobility and thoracicoutlet symptoms. CRMO is the most frequent non-neoplastic disease of the clavicle in children lessthan 20 years old [2]. In most patients, multiple bones are synchronously or metachronouslyaffected with both active and 'silent' lesions. Search for multifocality and recurrence is of utmostimportance for a correct diagnosis of the disease. No infectious etiology has been proven. Positive microbiology tests result from contamination ofskin organisms. Histology shows inflammatory changes and granulocytic infiltration withpolymorphonuclear leukocytes and osteoclastic bone resorption in the active phase of CRMO [3].Radiographic findings depend on the stage of the lesions. Initially, an osteolytic lesion is foundsometimes associated with periosteal reaction having a so-called onion ring" appearance. Later on,there is progressive sclerosis of the bone. CRMO of the clavicle typically affects the medial part,whereas the lateral portion is spared. The sternoclavicular joint is normal in children whereas adultsmay show arthritic changes or ligamentous ossification as part of the SAPHO syndrome. Activelesions show bone marrow edema which is hypo-intense on T1-weighted images (WI) andhyperintense on FS T2-WI MRI images. Transphyseal invasion may be visualized. Progressivesclerosis results in hypointense signal of the lesion on both pulse sequences as seen in subacute andchronic lesions. There is no strict correlation of activity on imaging with clinical symptoms.Clinically silent lesion can be active on imaging. Whole body MRI or 99mTC bone scintigraphymay be useful tools for demonstrating multifocality [4].Nonsteroidal anti-inflammatory drugs will relieve pain and swelling in 80% of the patients.Bisphosphonates can be added in case of failure of NSAIDS, but caution is warranted for possiblejaw necrosis. TNF blockers are a third option but are avoided due to associated risk of infectionsand malignancy [5].Final DiagnosisChronic Recurrent Multifocal OsteomyelitisDifferential Diagnosis ListEwing's sarcoma, Osteosarcoma, Infectious osteomyelitis, Langerhans cell histiocytosisFiguresFigure 1 Clinical imageClinical image of the patient with a marked swelling of the medial side of the clavicle (whitearrow) © Department of Radiology, AZ Sint-Maarten, Duffel, Belgium Area of Interest: Musculoskeletal bone;  Imaging Technique: Conventional radiography;  Procedure: Localisation;  Special Focus: Inflammation; Figure 2 Standard radiographyStandard radiograph shows an expansion of the bone at the medial side of the right clavicle.There is osteosclerosis of the bone with a solid periosteal reaction (arrow). © Department of Radiology, AZ Sint-Maarten, Duffel, Belgium Area of Interest: Musculoskeletal bone;  Imaging Technique: Conventional radiography;  Procedure: Diagnostic procedure;  Special Focus: Inflammation; Figure 3 Computed TomographyComputed tomography(coronal reformatted image). Inhomogeneous and sclerotic aspectwith a solid periosteal reaction. © Department of Radiologie, AZ Sint-Maarten, Duffel, Belgium Area of Interest: Musculoskeletal bone;  Imaging Technique: CT;  Procedure: Diagnostic procedure;  Special Focus: Inflammation; Figure 4 Coronal T1-weighted MRI ImageCoronal MRI image. The lesion is hypo-intense compared to bone marrow on T1-weightedimages. © Department of Radiology, AZ Sint-Maarten, Duffel, Belgium Area of Interest: Musculoskeletal bone;  Imaging Technique: MR;  Procedure: Diagnostic procedure;  Special Focus: Inflammation; Figure 5 Coronal T2 - weighted MRI imageCoronal MRI image. The lesion remains relatively hypointense compared to fluid within thesternoclavicular joint on fatsuppressed T2-weighted images. Note osseous expansion of thelesion beyond the original cortex of the clavicle (black arrow). © Department of Radiology, AZ Sint-Maarten, Duffel, Belgium Area of Interest: Musculoskeletal bone;  Imaging Technique: MR;  Procedure: Diagnostic procedure;  Special Focus: Inflammation; Figure 6 Coronal Fatsuppressed T1-weighted image after intravenous administration ofgadoliniumcontrastNote slightly heterogeneously contrast enhancement within the affected part of the clavicle,which is most prominent adjacent to the sternoclavicular joint. Note also subtle enhancementof the soft tissues adjacent to clavicle (arrow). © Department of Radiology, AZ Sint-Maarten, Duffel, Belgium Area of Interest: Musculoskeletal bone;  Imaging Technique: MR-Diffusion/Perfusion;  Procedure: Diagnostic procedure;  Special Focus: Inflammation; Figure 7 Standard radiography femurStandard radiography shows inhomogeneous sclerosis of the distal metaphysis and diaphysisof the right femur (predominantly of the dorsal portion) (arrow). © Department of Radiology, AZ Sint-Maarten, Duffel, Belgium Area of Interest: Musculoskeletal bone;  Imaging Technique: Conventional radiography;  Procedure: Diagnostic procedure;  Special Focus: Inflammation; Figure 8 Standard radiography tibiaStandard radiography shows inhomogeneous sclerosis of the distal metaphysis and diaphysisof the right tibia (predominantly of the anterior portion) (arrow). © Department of Radiology, AZ Sint-Maarten, Duffel, Belgium Area of Interest: Musculoskeletal bone;  Imaging Technique: Conventional radiography;  Procedure: Diagnostic procedure;  Special Focus: Inflammation; References[1] Giedion A, Holthusen W, Masel LF, Vischer D (1972) Subacute and chronic Ann Radiol(Paris). 15(3):329-42[2] Marino C1, McDonald E, Megna D, Brennessel D, Reddy KS, Jain PC (1992) Chronic recurrentmultifocal osteomyelitis in adult women. N Y State J Med. 92(8):360-2[3] Solheim L, Paus B, Liverud K, Støen E (1980) Chronic Recurrent Multifocal Osteomyelitis: ANew Clinical-Radiological Syndrome Acta Orthopaedica Scandinavica 51: 37-41[4] Khanna G, Sato TS, Ferguson P (2009) Imaging of Chronic recurrent Multifocal Osteomyelitis.Radiographics Radiographics 29(4):1159-77[5] Ferguson PJ, Sandu M (2012) Current understanding of the pathogenesis and management ofchronic recurrent multifocal osteomyelitis Curr Rheumatol Rep. 14(2):130-41CitationJulie Desimpel , Filip M. Vanhoenacker1, 2 1, 2, 31. Department of radiology, Antwerp University Hospital, Edegem, Belgium2. Department of radiology, General Hospital Sint-Maarten, Duffel-Mechelen, Belgium3. Department of radiology, Gent University Hospital, BelgiumUniversity Hospital Antwerp, Department of Radiology, University Hospital Antwerp;Wilrijkstraat, 10 2650 Antwerp, Belgium; Email:filip.vanhoenacker@telenet.be (2017, Jan. 7)  Chronic Recurrent Multifocal Osteomyelitis (CRMO) of the clavicle {Online}URL: http://www.eurorad.org/case.php?id=14212

Chronic Recurrent Multifocal Osteomyelitis (CRMO) of the clavicle

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Chronic Recurrent Multifocal Osteomyelitis (CRMO) of the clavicle

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