Chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis was first described in 1972 and to date 33 cases have been reported, all but one from outside the United States. This unusual osteomyelitis is characteristically recurrent and multifocal with a predilection for the metaphyses. Cultures are persistently negative and antibiotics do not appear to affect the course of the disease, which may be as long as 15 years. Females are affected twice as frequently as males and half the cases are less than ten years old. Antistreptolysin 0 titers are elevated in a quarter of the patients, and there may be a history of previous throat infection. There is an association with pustulosis palmoplantaris. We present two additional cases from the United States.     

Multifocal osteomyelitis in childhood. Review by radionuclide bone scan.

Multifocal osteomyelitis is considered an uncommon complication of acute osteomyelitis. Over a 3-year period, 136 infants and children who had a final diagnosis of acute osteomyelitis were reviewed, and multifocal osteomyelitis was detected in 27 (19%) patients. The major age peak of acute osteomyelitis was between 6 weeks and 3 years (46%). Two age peaks were found for multifocal disease-less than 6 weeks (38%), and 9 to 12 years (44%). Three patients with multifocal disease had septicemia and photon-deficient areas on bone scans. Another adolescent group had nonspecific bone and joint pain that in some cases persisted for more than 3 months and were finally diagnosed as multifocal osteomyelitis. Organisms were isolated in 15/27 (56%). Multifocal osteomyelitis is well recognized in the neonatel age group. However, it occurs more commonly than previously described in older patients. This higher incidence can most likely be attributed to the higher use of the radionuclide bone scan early in the disease and the high sensitivity of the scan for the detection of osteomyelitis.     

Infection of the appendicular skeleton

In relation to the clinical course, infection in bone can be divided into acute, subacute and chronic osteomyelitis. The diagnosis of acute osteomyelitis is often challenging but can best be made by correlating radiography, bone scintigraphy and MRI with clinical information. Radiography should routinely be supplemented by sonography in the newborns and infants, if applicable. Brodie's abscess, which is clinically a subacute form of osteomyelitis, is best diagnosed by the combination of radiography and MRI. Chronic osteomyelitis is divided into primary haematogenous forms and exogenous, mostly post-traumatic, osteomyelitis. In the majority of patients, post-traumatic osteomyelitis is a clinical diagnosis; however, in a number of patients only the correlation of clinical findings, blood tests and imaging reveals the correct diagnosis. Often, MRI and scintigraphic methods, such as scanning with labeled leucocytes, together establish the diagnosis. Chronic recurrent multifocal osteomyelitis may mimic bacterial osteomyelitis but is a distinct disease probably associated with the SAPHO syndrome.     

Infection of the appendicular skeleton

In relation to the clinical course, infection in bone can be divided into acute, subacute and chronic osteomyelitis. The diagnosis of acute osteomyelitis is often challenging but can best be made by correlating radiography, bone scintigraphy and MRI with clinical information. Radiography should routinely be supplemented by sonography in the newborns and infants, if applicable. Brodie’s abscess, which is clinically a subacute form of osteomyelitis, is best diagnosed by the combination of radiography and MRI. Chronic osteomyelitis is divided into primary haematogenous forms and exogenous, mostly post-traumatic, osteomyelitis. In the majority of patients, post-traumatic osteomyelitis is a clinical diagnosis; however, in a number of patients only the correlation of clinical findings, blood tests and imaging reveals the correct diagnosis. Often, MRI and scintigraphic methods, such as scanning with labeled leucocytes, together establish the diagnosis. Chronic recurrent multifocal osteomyelitis may mimic bacterial osteomyelitis but is a distinct disease probably associated with the SAPHO syndrome.     

Chronic recurrent multifocal osteomyelitis involving the mandible: case reports and review of the literature

Chronic recurrent multifocal osteomyelitis (CRMO) is an aseptic inflammatory disorder of unknown cause occurring in children and adolescents. It is characterized by multifocal bone lesions with pain and swelling recurring over months to years. Lesions usually involve the metaphyses of the long bones and involvement of the jaw is rare. The clinical presentation, radiographic appearance and histology of a case of CRMO involving the mandible in an 8-year-old girl are documented. The radiographic appearance of another three cases is also described.     

Chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis (CRMO) is a clinical entity distinct from bacterial osteomyelitis. It occurs mainly in children and adolescents and is characterized by a prolonged, fluctuating course with recurrent episodes of pain occurring over several years. CRMO is often multifocal and most often seen in tubular bones, the clavicle, and less frequently the spine and pelvic bones; other locations are rare. The radiographic appearance suggests subacute or chronic osteomyelitis. Histopathological and laboratory findings are nonspecific and bacterial culture is usually negative. CRMO is often diagnosed by exclusion of the two main differential diagnoses--bacterial infections and tumor--by assessing for a characteristic course and the findings by conventional radiography, if necessary supplemented by scintigraphy and/or magnetic resonance imaging (MRI). The MRI appearance of CRMO lesions in tubular bones and the spine is often rather characteristic and support the diagnosis. It is important to diagnose CRMO to avoid unnecessary diagnostic procedures and initiate an appropriate therapy.     

全身MRI在慢性复发性多灶性骨髓炎中的诊断进展

慢性复发性多灶性骨髓炎(CRMO)是好发于儿童和青少年的自身炎症性骨病,临床表现及实验室检查缺乏特异性。CRMO早期X线表现可正常,中晚期呈进行性长骨干骺端骨质破坏、硬化或骨皮质增厚等骨髓炎改变。全身MRI检查发现多灶性、对称性骨病,或锁骨、下颌骨单发病变对该病的诊断具有重要提示作用。就CRMO的临床表现、发病机制、全身MRI表现、鉴别诊断及治疗进展进行综述。     

Chronic recurrent multifocal osteomyelitis: an evolving clinical and radiological spectrum

 Objective. To describe unusual clinical and radiological features in patients with chronic recurrent multifocal osteomyelitis (CRMO). Design and subjects. Based on radiographic and microscopic findings, three patients were prospectively diagnosed as having chronic recurrent multifocal osteomyelitis (CRMO). They form the basis of this report because of either the unusualness of the clinical presentation, hitherto undescribed associated diseases or the unusual age of presentation and sites of lesions. Results. One patient developed pyoderma gangrenosum at the site of one of the skeletal lesions and then went on to develop ulcerative proctitis. A second patient presented with a soft tissue mass, which on MRI mimicked a sarcoma. The final patient presented with lesions in the wrist and phalanges of the toes at the unusual age of 38. None of the patients was treated with steroids or antibiotics for the skeletal lesions. Steroids were administered to one patient for treatment of pyoderma gangrenosum. Conclusions. The pattern and distribution of skeletal lesions in CRMO are well recognized in the pediatric age group. The unusual clinical and/or radiological features discussed herein suggests that this is a disease that continues to evolve with a broader spectrum of features than recognized.     

Chronic sclerosing osteomyelitis of the iliac bone. Etiological possibilities

An adolescent with chronic recurrent multifocal osteomyelitis located to both iliac bones and an adult with pustulotic arthro-osteitis, involving the left iliac bone, an invertebral space, and the sternoclavicular, first sternocostal, manubriosternal and single peripheral joints are described. Their iliac bone changes were identical, except for the occurrence of bilateral changes in the adolescent, and ankylosis of the sacroiliac joint in the adult. It is suggested that chronic recurrent multifocal osteomyelitis and pustulotic arthro-osteitis may be related diseases with age-dependent differences.     

Synovitis,Acne, Pustulosis,Hyperostosis and Osteitis (SAPHO) and chronic recurrent multifocal osteomyelitis (CRMO): Role of imaging in diagnosis

There is a spectrum of musculoskeletal disorders which can be associated with dermatologic findings, the fundamental component of which is a nonbacterial osteitis. CRMO (Chronic recurrent multifocal osteomyelitis) and SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis) describe paediatric and adult conditions, respectively, of inflammatory osteitis that can be associated with palmoplantar pustulosis and acne. Imaging findings are similar and a key component to the diagnosis in both conditions. This report describes two patients with strikingly similar radiologic presentations of clavicular osteitis in whom the diagnosis was made predominantly on the basis of imaging findings. The typical imaging features and radiographic hallmarks of both conditions will also be discussed.     

Chronic recurrent multifocal osteomyelitis in children. A roentgenologic and scintigraphic investigation

Thirty cases of chronic recurrent multifocal osteomyelitis have been reported in the literature. A radiologic evaluation of thirty-one additional cases, 25 of whom also underwent bone scintigraphy, is presented. Bone biopsy specimens were obtained in 16 patients. Most lesions were located in the metaphyses of the long bones adjacent to the physis and had a characteristic, probably pathognomonic appearance. Extension into the epiphyses was rare. Lesions in the vertebral bodies, clavicle and pelvis had possibly a less specific radiographic appearance. Lesions in short tubular bones were non-specific. Bone scintigraphy had a practical value in evaluating the global distribution of lesions including asymptomatic lesions and lesions in the spine or pelvis, the latter being somewhat hard to detect with conventional radiography. All biopsies showed acute, subacute or chronic unspecific osteomyelitis, sometimes mixed in the same lesion. Staining for bacteria and fungi was negative.     

Vertebral osteomyelitis without disc involvement

Vertebral osteomyelitis is most commonly due to pyogenic or granulomatous infection and typically results in the combined involvement of the intervertebral disc and adjacent vertebral bodies. Non-infective causes include the related conditions of chronic recurrent multifocal osteomyelitis (CRMO) and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome. Occasionally, these conditions may present purely within the vertebral body, resulting in various combinations of vertebral marrow oedema and sclerosis, destructive lesions of the vertebral body and pathological vertebral collapse, thus mimicking neoplastic disease. This review illustrates the imaging features of vertebral osteomyelitis without disc involvement, with emphasis on magnetic resonance imaging (MRI) findings.     

Recurrent multifocal chronic osteomyelitis: scintigraphy or MRI. Apropos of 2 cases]

We report two cases of chronic recurrent multifocal osteomyelitis. In both cases, MRI demonstrated the presence of asymptomatic lesions that the technetium bone scan failed to show. The asymptomatic lesions were located in the acetabelum and the distal femur. Despite the actual literature, MRI was more specific than bone scan in these two cases.     

Chronic infections multifocal osteomyelitis vs chronic recurrent multifocal osteomyelitis: with reference to one case

We present a rare case of chronic infectious multifocal osteomyelitis with affectation symmetric in the both femures in a patient with spondyarthropathic secondary Inflammatory Bowel Disease. The diagnosis was confirmed with culture of the bone biopsy. The aim of this work is the revision of the Chronic Infectious Multifocal Osteomyelitis and the Chronic Recurrent Multifocal Osteomyelitis into SAPHO syndrome that both were the possibilities diagnoses.     

Chronic recurrent multifocal osteomyelitis demonstrated by Tc-99m methylene diphosphonate bone scan

A 12-year-old Middle Eastern girl presented with abdominal pain, weight loss, and intermittent pain in both thighs. She was initially suspected of Crohn disease, but this diagnosis was excluded after extensive gastromedical evaluation. Plain x-rays of the femora were normal, whereas the initial Tc-99m methylene diphosphonate (MDP) bone scan showed several foci with increased activity in both femurs. MR scan showed excessive periostal inflammatory and mild intramedullary changes in both femurs. A percutaneous bone biopsy demonstrated changes consistent with chronic recurrent multifocal osteomyelitis (CRMO). The patient's symptoms disappeared spontaneously, but reappeared 1.5 years later, which led to a new MDP bone scan that showed normal findings.     

Imaging of chronic recurrent multifocal osteomyelitis of childhood first presenting with isolated primary spinal involvement

OBJECTIVE: Initial presentation with primary spinal involvement in chronic recurrent multifocal osteomyelitis of childhood (CRMO) is rare. Our objective was to review the imaging appearances of three patients who had CRMO who initially presented with isolated primary spinal involvement. DESIGN AND PATIENTS: The imaging, clinical, laboratory and histology findings of the three patients were retrospectively reviewed. Imaging included seven spinal MR imaging scans, one computed tomography scan, nine bone scans, two tomograms and 16 radiographs. These were reviewed by two musculoskeletal radiologists and a consensus view is reported. All three patients presented with atraumatic spinal pain and had extensive bone spinal pathology. The patients were aged 11, 13 and 12 years. There were two females and one male. RESULTS AND CONCLUSIONS: The initial patient had thoracic T6 and T8 vertebra plana. Bone scan showed additional vertebral body involvement. Follow-up was available over a 3 year period. The second patient had partial collapse of T9 and, 2 years later, of C6. Subsequently extensive multifocal disease ensued and follow-up was available over 8 years. The third patient initially had L3 inferior partial collapse and 1 year later T8 involvement with multifocal disease. Follow-up was available over 3 years. The imaging findings of the three patients include partial and complete vertebra plana with a subchondral line adjacent to endplates associated with bone marrow MR signal alterations. Awareness of the imaging appearances may help the radiologist to include this entity in the differential diagnosis in children who present with spinal pathology and no history of trauma. Histopathological examination excludes tumor and infection but with typical imaging findings may not always be necessary.     

Osteomyelitis in the neonate. Clinical aspects and the role of radiography and scintigraphy in diagnosis and management

Based on experience with 22 cases of neonatal osteomyelitis in 10 years, the authors suggest these patients can be divided into two groups depending on severity of disease. Premature infants requiring umbilical catheterization and severely ill full-term infants constitute a high-risk group; signs are more overt, multifocal infection and joint involvement more frequent, and severe skeletal deformities more common. The patients in the low-risk group had much milder disease but also presented more difficulty in diagnosis because of the vagueness of the presenting signs. Radiographic examination is essential for diagnosis and follow-up of osteomyelitis, particularly limb deformities. Bone scans should be reserved for situations in which the clinical and radiographic findings are equivocal.     

Scintigraphic aspect of infantile cortical hyperostosis (Caffey's disease)

Two patients with infantile cortical hyperostosis (Caffey's disease) are presented in whom radionuclide imaging with 99mTc-MDP and 67Gallium-citrate was performed to eliminate multifocal osteomyelitis as a diagnosis. The scintigraphic appearance of this relatively uncommon disease of children was similar to that obtained in multifocal osteomyelitis; all osseous lesions showed a marked uptake of both radiotracers. However, the distribution of abnormalities (mandible, scapulae) and the degree of extension (mainly diaphyseal) of the lesions are important characteristics in the scintigraphic differential diagnosis but bone radiographs are best used in making the diagnosis.     

Successful treatment of chronic recurrent multifocal osteomyelitis using low-dose radiotherapy

Background

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory disease, which lacks an infectious genesis and predominantly involves the metaphysis of long bones. Common treatments range from nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids at first onset of disease, to immunosuppressive drugs and bisphosphonates in cases of insufficient remission. The therapeutic use of low-dose radiotherapy for CRMO constitutes a novelty.

Case report

A 67-year-old female patient presented with radiologically proven CRMO affecting the right tibia/talus and no response to immunosuppressive therapy. Two treatment series of radiation therapy were applied with an interval of 6 weeks. Each series contained six fractions (three fractions per week) with single doses of 0.5 Gy, thus the total applied dose was 6 Gy. Ten months later, pain and symptoms of osteomyelitis had completely vanished.

Conclusion

Radiotherapy seems to be an efficient and feasible complementary treatment option for conventional treatment refractory CRMO in adulthood. The application of low doses per fraction is justified by the inflammatory pathomechanism of disease.

     

Premature epiphyseal fusion and degenerative arthritis in chronic recurrent multifocal osteomyelitis

A 9-year-old boy was diagnosed with chronic recurrent multifocal osteomyelitis affecting multiple sites. During an 8-year follow-up he developed premature closure of a distal radial epiphysis and degenerative changes in the adjacent radiocarpal joint. Received: 26 July 1999 Revision requested: 24 August 1999 Revision received: 27 September 1999 Accepted: 8 October 1999