Chronic recurrent multifocal osteomyelitis: report of five cases

Chronic recurrent multifocal osteomyelitis is a rare disease of unknown etiology that mainly affects children. We present a retrospective review of the medical records of five patients with a diagnosis of chronic recurrent multifocal osteomyelitis in an infectious diseases clinic at a tertiary center. We describe the epidemiological, clinical and radiological features, laboratory, microbiological and histological findings, treatment, course and prognosis in these patients. There is no specific treatment for chronic recurrent multifocal osteomyelitis. There are new treatment options such the combination of azithromycin and calcitonin in children without clinical improvement with non-steroidal anti-inflammatory drugs.     

Chronic recurrent multifocal osteomyelitis of the spine and mandible: case report and review of the literature

At initial presentation, chronic recurrent multifocal osteomyelitis may mimic acute hematogenous osteomyelitis; however, cultures of affected bone are sterile. Nuclear scintigraphy identifies additional foci of involvement that present concurrently or sequentially. Unlike acute bacterial osteomyelitis, chronic recurrent multifocal osteomyelitis seems unaffected by antibiotic therapy and typically responds to treatment with antiinflammatory drugs. Surgical decortication has been reported for refractory cases. The case presented here illustrates the rare involvement of the mandible after initial presentation in the spine of a 4-year-old girl and the refractory nature of the disease over 6 years despite treatment with various medical and surgical therapies.     

Successful management of chronic multifocal Q fever Osteomyelitis with adjuvant interferon-gamma therapy

We present a 3-year-old girl who had chronic recurrent multifocal osteomyelitis caused by Coxiella burnetii despite long-term dual antibiotic therapy. Excellent clinical response was achieved and sustained when immunomodulatory therapy with interferon-γ was initiated. This is the case of a first child who was successfully treated with interferon-γ as adjuvant therapy for chronic multifocal Q fever osteomyelitis.     

Bacterial bone and joint infections in childhood--a review. 4. Subacute and primary-chronic osteomyelitis, rare forms of osteomyelitis

This is an overview of the most important aspects of pathogenesis, etiology, diagnostics, therapy and differential diagnostics of the subacute and primary chronic osteomyelitis in children. This group of disease includes Brodie's abscess, plasma cellular osteomyelitis, sclerosing osteomyelitis (Garré) and the chronic recurrent multifocal osteomyelitis. The treatment of children with these not completely understood diseases requires a close cooperation between pediatricians, pediatric surgeons and radiologists.     

Chronic recurrent multifocal osteomyelitis: a noninfectious inflammatory process

We report seven patients with chronic recurrent multifocal osteomyelitis, an uncommon childhood disease of unknown etiology. These patients presented with insidious onset of bone pain at one or more sites associated with erythema, swelling and tenderness. Scintigraphy and radiography were consistent with osteomyelitis at multiple sites. Bone biopsies confirmed osteomyelitis but no organisms were consistently isolated. During a 1- to 3-year follow-up, most patients developed new symptomatic lesions. The disease was unaffected by antimicrobial therapy. Two of our patients had psoriasis and all were rheumatoid factor-, antinuclear factor- and HLA-B27-negative. We speculate that chronic recurrent osteomyelitis is a noninfectious inflammatory condition, a seronegative spondyloarthropathy. Chronic recurrent osteomyelitis is a clinical entity that should be recognized so that invasive diagnostic procedures and antimicrobial therapy are appropriately used. The patient may be reassured that this is not a malignant condition although there may be exacerbations over many years.     

Die chronisch rezidivierende multifokale Osteomyelitis (CRMO) Übersicht und erste Befunde einer genetisch-rheumatologischen Studie

Chronic recurrent multifocal osteomyelitis (CRMO) is a nonbacterial osteomyelitis and most commonly occurs during childhood. Associations with palmoplantar pustulosis (PPP) have been reported in about 20%. Since the etiology of the disease is unknown it is suspected to be an autoimmune osteomyelitis. Own studies [27] demonstrate evidence for a genetic basis of CRMO for the first time. The clinical features, diagnostic procedures and therapeutic options are demonstrated.     

Primary multifocal osseous lymphoma in a child

We report a case of primary multifocal osseous lymphoma in a 6-year-old girl presenting with multifocal osteolytic lesions without systemic symptoms or identifiable non-osseous primary tumor. The differential diagnoses for such a presentation include histiocytosis X, chronic recurrent multifocal osteomyelitis, acute lymphoblastic leukemia, metastatic disease, and primary bone lymphoma. Although non-Hodgkin lymphoma is common in the pediatric population, its presentation as a primary bone tumor, especially with multifocal disease, is extremely rare and is frequently misdiagnosed. We hope that awareness of this entity will help radiologists achieve timely diagnosis and intervention.     

Chronic recurrent multifocal osteomyelitis of the mandible. A case report]

Chronic recurrent multifocal osteomyelitis (CRMO) is a disorder rarely localized to the lower jaw. CASE REPORT: A fourteen-year-old boy complained of a swollen of his lower jaw. After a CT Scan, a bone biopsy was performed and yielded S. oralis against which an adapted intravenous antibiotherapy was administered without efficacy. The absence of malignant process and the revelation of an other focus of fixation at the Tc bone scan localized on humerus called to mind the diagnosis of CRMO. CONCLUSION: The diagnosis of this disease is difficult and based on a number of concording arguments:clinical and radiological signs of osteomyelitis, multifocal presentation, recurrent relapses and remissions, inaction of antibiotics, elimination of the other differential diagnosis, in particular the infectious osteitis.     

The syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia. Report of a new family and a review

A new autosomal recessive syndrome of chronic recurrent multifocal osteomyelitis (CRMO) and congenital dyserythropoietic anaemia (CDA) with microcytosis has recently been described in four children (two sibships) of one consangineous Arab family. In this report, we describe the clinical features and course of the syndrome of CRMO and CDA in two additional patients (one sibship) from another consanguineous Arab family and review the literature. The two patients (brother and sister), the products of a consanguineous marriage, developed the syndrome at an early age of 3 weeks and 2 months respectively. The diagnosis of CRMO was confirmed by radiological and technetium isotope bone scans. Bone marrow studies confirmed the diagnosis of CDA. Peripheral blood films showed hypochromia and microcytosis. The sites involved by CRMO were periarticular, mainly around the elbow, knee, wrist and small joints of the hand. The brother is now 21 years old and the sister 3.5 years old and CRMO is still active with frequent relapses. The brother developed flexion deformities at the age of 13 years. Both patients failed to thrive; weight and height were below the 5th percentile. CONCLUSION: This is the second report of the syndrome of chronic recurrent multifocal osteomyelitis and microcytic congenital dyserythropoietic anaemia, confirming it as a clinical entity, inherited as an autosomal recessive trait. The disease is characterised by an early onset, long clinical course of remissions and relapses, and seems to be different from the sporadic form of chronic recurrent multifocal osteomyelitis.     

Physeal involvement in chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis is a recently recognized disease characterized by remissions and exacerbations of multiple bone lesions which radiographically and pathologically have the appearance of hematogenous osteomyelitis. No consistent etiology can be identified, and antimicrobial agents seem to have no beneficial effect. A review of the appearances of imaging modalities and the clinical and pathologic manifestations is undertaken in seven cases of CRMO. No therapeutic regimen resulted in consistent clinical or radiographic improvement. We suggest the sequelae of this process are not as benign as previously reported.     

Chronic recurrent multifocal osteomyelitis – report of eight patients

The authors undertook a retrospective review of the clinical records and radiologic findings of eight children with chronic, recurrent multifocal osteomyelitis (CRMO). This disease is a recognized clinicopathologic entity with typical radiographic findings, mostly in the metaphyses of the long bones. The diagnosis is one of exclusion without pathognomonic findings. The patients were treated with non-steroidal anti-inflammatory drugs. There was no evidence of altered bone growth or abnormal joint development. It is concluded that CRMO is a distinct clinical entity that is different from acute or subacute bacterial osteomyelitis. Recognition of this condition is important to avoid treatment with antibiotics and repeated operations. Accepted: 8 April 1998     

Chronic recurrent multifocal osteomyelitis with unusual features

We describe two children with chronic recurrent multifocal osteomyelitis who showed unusual extraskeletal manifestations. The first patient developed an asymptomatic pulmonary consolidation; the second had an atypic localization of the associated pustulosis, which involved the trunk, groin, thighs and buttocks, but spared the palms and soles. We conclude that this disease is clinically more heterogeneous than previously thought.     

Ostéomyélite chronique multifocale récurrente. À propos d'un cas de la mandibuleChronic recurrent multifocal osteomyelitis of the mandible. A case report

Chronic recurrent multifocal osteomyelitis (CRMO) is a disorder rarely localized to the lower jaw.Case report. – A fourteen-year-old boy complained of a swollen of his lower jaw. After a CT Scan, a bone biopsy was performed and yielded S. oralis against which an adapted intravenous antibiotherapy was administered without efficacy. The absence of malignant process and the revelation of an other focus of fixation at the Tc bone scan localized on humerus called to mind the diagnosis of CRMO.Conclusion. – The diagnosis of this disease is difficult and based on a number of concording arguments:clinical and radiological signs of osteomyelitis, multifocal presentation, recurrent relapses and remissions, inaction of antibiotics, elimination of the other differential diagnosis, in particular the infectious osteitis.     

Rare forms of chronic osteomyelitis in children (author's transl)

4 cases of chronic osteomyelitis in children and adolescents are described. The first one concerns a girl with severe plasmacellular osteomyelitis of the right upperarm. As the treatment with antibiotics failed a partial resection of the right humerus, followed by plastic covering of the resulting defect was successful. Two further cases of chronic metatarsal osteomyelitis of both feet could be classified as a kind of "sclerosing osteomyelitis Garré", mostly corresponding to the clinical feature of the "chronic recurrent multifocal osteomyelitis" (Bj?rkstén et al., 1978). Furthermore, a case of chronic ostitis localized in the symphyseal area is described, the clinical symptoms of which were resembling to the cortical osteoid, a special kind of sclerosing osteomyelitis. It is suggested that these different forms of chronic osteomyelitis in children are caused by bacterial infections. Nevertheless, this hypothesis remains to be proved. The problems of the differential diagnosis are discussed. Generally, the only therapy with antibotics is not successful, whereas the combination of removing of the inflammatory process with plastic covering of the bone-defect usually results in recovery.     

Vertebral collapse in an 8-year-old girl

We present a case of an 8-year-old girl with collapse of her T6 and T7 vertebrae secondary to chronic recurrent multifocal osteomyelitis. She presented with chronic abdominal pain and was found to have multiple bony lesions involving her spine, clavicle and mandible. Extensive investigations, including tissue biopsy, were unable to identify an infective cause and there was no response to a prolonged course of intravenous antibiotics. She made a good response to regular non-steroidal anti-inflammatory medication.     

Dramatic pain relief and resolution of bone inflammation following pamidronate in 9 pediatric patients with persistent chronic recurrent multifocal osteomyelitis (CRMO)

Background  

Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory, non-infectious osteopathy that affects predominantly patients ≤ 18 years of age. There is no uniformly effective treatment. Our objective is to describe clinical, magnetic resonance imaging (MRI), and bone resorption response to intravenous pamidronate in pediatric CRMO.     

Acute polyosteal osteomyelitis in a patient with congenital ichthyosis and acute lymphoblastic leukemia.

Osteomyelitis can develop after bacteremia in children and is a rare complication of chemotherapy for acute lymphoblastic leukemia. A child with congenital ichthyosis, acute lymphoblastic leukemia and multifocal hematogenous osteomyelitis is described herein. A breached skin barrier secondary to ichthyosis during induction chemotherapy, coupled with impaired immunity, likely provided the opportunity for bacterial seeding, leading to an extensive multifocal osteomyelitis.     

Multifocal Tuberculous Osteomyelitis: Possible Inherited Interferon Gamma Axis Defect

Multifocal Tuberculous (TB) osteomyelitis is an extremely rare entity in immunocompetent individuals. The authors report a 19-mo-old girl with multifocal TB osteomyelitis which resolved completely following institution of four drug antituberculous treatment for 1 y and detailed immunological evaluation was found to be normal but as the response of interferon gamma (IFN γ) in terms of production of IL12p40 was severely impaired, she is suggested to have a possible defect in the interferon gamma axis even though the exact defect in the pathway could not be delineated. This case report reiterates the need for detailed immunological evaluation in children presenting with multifocal TB osteomyelitis.     

Chronic recurrent multifocal osteomyelitis with MR correlation: A case report

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare disorder of unknown etiology. The main characteristics on plain X-rays are a lytic destruction in the metaphyseal region of the long bones followed by progressive sclerosis. The symmetrical pattern and the frequent involvement of the sternoclavicular joints and vertebrae are typical. An association with palmoplantar pustulosis has also been described. Laboratory findings are nonspecific. Although MRI is not necessary to make the diagnosis of chronic osteomyelitis, it is useful in assessing the extent and the evolution of the lesions. CRMO of the tibial and fibular bones is described in a 14-year-old girl, who suffered from pain and soft tissue swelling in both ankles. Initial plain X-rays and laboratory findings were normal. After relapsing clinically, progressive sclerosis of both fibular bones occurred. Lytic lesions in the left tibia and both fibular bones were visible. Scintigraphic examination showed pathologic tracer accumulation in both legs. The diagnosis of CRMO was suggested based on CT and MRI findings. CRMO was confirmed after curettage and bone biopsy. Histopathological findings were consistent with active chronic osteomyelitis.     

Successful treatment of chronic recurrent multifocal osteomyelitis with tumor necrosis factor-alpha blockage

We describe the case of an 18-year-old girl with chronic recurrent multifocal osteomyelitis (CRMO) over a period of 10 years. She had suffered predominantly from very painful recurrent swelling of her cheeks. Various therapeutic regimens including nonsteroidal antiinflammatory drugs and steroids had shown only a partial or temporary response. Because tumor necrosis factor-alpha-blocking agents have been successfully applied in Crohn's-associated CRMO and the related SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome, tumor necrosis factor-alpha-blocking therapy with infliximab was initiated. Thereafter, apart from 1 mild episode, no additional recurrences were observed during 21 months of follow-up. Infliximab was well tolerated, and steroids were tapered off. Our observation indicates that infliximab may be an effective therapeutic option in CRMO.