Chronic Recurrent Multifocal Osteomyelitis (ECR 2010 Case of the Day)

Clinical Cases 18.10.2010
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 18 years, female
Authors: Gonçalves LP, Reijnierse M.
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Details
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AI Report

Clinical History

An 18-year-old female presented with pain in the left clavicle. She was known with intermittent pain and swelling on the medial clavicle and right ribs in the last four years, nonresponsive to local corticoid. The histology and culture of two previous percutaneous biopsies were inconclusive. The laboratory tests were unremarkable.

Imaging Findings

Radiographs (anteroposterior clavicle and posteroanterior chest) revealed predominantly sclerotic lesions with a minor lytic component, and marked expansion and solid periosteal reaction on the medial two-thirds of the left clavicle and anterior aspect of the 6th right rib (Fig. 1-3).
Tc-99m methylene diphosphonate bone scintigraphy (anterior projection) demonstrated increased uptake in the locations previously described as well as in the 5th right rib, the latter being radiographically occult (Fig. 4).
Magnetic resonance imaging (MRI) of the left clavicle depicted a medullary lesion with sclerosis and multiple patchy and coalescent areas isointense in T1-weighted images (WI), hyperintense in T2-WI, and strongly enhancing after gadolinium on the medial two-thirds of the clavicle. The solid periosteal reaction is also appreciated as well as soft tissue edematous changes (Fig. 5-8).

Discussion

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare, autoinflammatory disorder typically affecting children and adolescents. CRMO belongs to the spectrum of aseptic osteomyelitis-like conditions in which SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis) syndrome is included. Its cause remains unknown though recently a genetic component has been suggested. Its incidence is estimated to be 2–5% of all osteomyelitis cases [1, 2]. CRMO is twice as common in females and the age of onset is usually 4–14 years although it has been reported in adulthood [1, 2, 3]. The main complaints are localized pain and variable swelling, possibly associated with mild malaise and dermatologic affections as palmoplantar pustulosis [2, 3].
Lesions are typically bilateral and multifocal at presentation and involve the metaphyses of tubular bones and medial clavicle, and also commonly the spine, ribs, mandible and pelvis [1, 2, 4]. CRMO has a prolonged course with relapses and remissions, involving the same or a different location. Histopathology is that of a non-specific inflammation, with fibrosis and/or hyperostotic regeneration [5].
The mainstays of imaging diagnosis are radiography and bone scintigraphy. Initially, the lesions are purely osteolytic developing variable sclerosis and periosteal reaction over time. After clinical resolution, radiographic appearance can normalize in 0,5-5 years or maintain remnants of the bony alteration. Complete healing of the clavicular lesions is rare [2, 3, 4]. MRI is highly sensitive for diagnosing occult lesions and whole body MRI is increasingly used for evaluation of multifocal bone lesions. MRI is extremely accurate to evaluate disease activity and local extent, being helpful for biopsy guidance and evaluation of long-standing disease. Active disease manifests as areas of high signal on T2-WI and enhancement. Quiescent disease correlates with low signal on T1- and T2-WI corresponding to sclerosis, possibly with scattered areas of high signal related to fatty marrow [1, 2].
Owing to the lack of specific tests the diagnosis of CRMO is one of exclusion. Although CRMO can be self-limited, it can have a protracted course and result in significant morbidity. The multifocal involvement of typical sites particularly the anterior thoracic wall, as in the case in appreciation, is crucial to accurate diagnosis.
Radiologists can be the first to suggest CRMO diagnosis given its characteristic radiographic appearance and distribution, thus preventing unnecessary multiple biopsies and long-term antibiotic treatment.

Differential Diagnosis List

Chronic Recurrent Multifocal Osteomyelitis
Polyostotic Eosinophilic Granuloma
Ewing sarcoma
Fibrous Dysplasia
Leukemia

Final Diagnosis

Chronic Recurrent Multifocal Osteomyelitis

Liscense

Figures

anteroposterior clavicle radiograph

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anteroposterior clavicle radiograph

posteroanterior chest radiograph

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posteroanterior chest radiograph

rib detail

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rib detail

Tc-99m methylene diphosphonate bone scintigraphy

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Tc-99m methylene diphosphonate bone scintigraphy

MRI of the left clavicle. Axial T1W image

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MRI of the left clavicle. Axial T1W image

Axial T2W fat sat image

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Axial T2W fat sat image

Axial T1W fat sat after i.v. contrast

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Axial T1W fat sat after i.v. contrast

Sagittal T1W fat sat after i.v. contrast

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Sagittal T1W fat sat after i.v. contrast