Chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis is a rare disorder of unknown etiology, characterized by multiple bone lesions and a variable clinical course. We present a 10 year old boy with chronic recurrent multifocal osteomyelitis who improved after treatment with naproxen.     

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.     

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.     

Blurring the lines in interferon {gamma} receptor deficiency: an infant with near-fatal airway disease

Deficiencies of the interferon γ (IFN-γ) pathway have become a well-recognized cause of nontuberculous mycobacterial infection. We report here a case of autosomal dominant IFN-γ receptor 1 (IFN-γ-R1) deficiency presenting at the unusually young age of 16 months with a severe clinical course. Mycobacterium avium complex was cultured from bronchial washings of a child who presented with primary endobronchial disease after a 4-month history of rhinorrhea, wheeze, and acute lobar consolidation. A maternal history of multifocal Mycobacterium kansasii osteomyelitis and cutaneous M avium complex led to genetic confirmation of IFN-γ-R1 818del4 deletion (a 4 base pair deletion at nucleotide position 818) in both family members. This case demonstrates the link between mycobacterial disease and IFN-γ pathway deficiency, the diagnosis of which facilitates more accurate therapy and genetic counseling. The case also raises questions about the reported distinct presentation, treatment, and prognosis of autosomal dominant and recessive IFN-γ-R1 phenotypes.     

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.     

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.     

Small rounded B-cell lymphoma of bone presented by limp, with a positive multifocal 99mTc MDP bone scintigraphy pattern and a negative 99mTc HMPAO-labeled leukocytes study

In this article, we present a rare case of B-cell bone lymphoma in a child with multifocal asymptomatic lesions detected by bone scintigraphy and a chronic clinical history characterized by limping and fever episodes for over a year. Initially, multifocal osteomyelitis was suspected and antibiotic therapy was administered with no clinical improvement. The biopsy of the main lesion in the left distal femur along with bone marrow cytology established the final diagnosis. This rare case illustrates the utility of routinely low cost-effective nuclear medicine studies like whole body bone scan and 99mTc hexamethylpropyleneamine oxime-labeled leukocytes to orientate similar cases to a correct diagnosis.     

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.     

Sweet's syndrome with multifocal sterile osteomyelitis

Sweet's syndrome is a rare condition characterized by fever, leukocytosis, dense dermal infiltration of leukocytes, and painful indurated cutaneous plaques. We describe a 23-month-old male infant with the typical cutaneous manifestations of Sweet's syndrome and painful extremities that limited ambulation. Evaluation revealed multifocal sterile osteomyelitis of both tibia. After the institution of systemic corticosteroid therapy, the patient's symptoms resolved, and bone scans normalized. After one year of follow-up, the patient has intermittent recrudescences that resolve during corticosteroid therapy. Sterile multifocal osteomyelitis represents another clinical manifestation of Sweet's syndrome.     

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.     

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.     

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.     

Locations of osteomyelitis in children with sickle-cell disease at Tokoin teaching hospital (Togo)

Background  The sickle-cell children are particularly affected by osteomyelitis in specific locations. This study was done in order to point out the locations of osteomyelitis in children with sickle-cell disease. This direct clinical examination for a quick diagnosis. Materials and methods  This is a retrospective study done by examining files of 43 children (15 girls and 28 boys), aged from 0 to 15 years, treated for osteomyelitis between January 1998 and December 2006. Their phenotypes included 18 SS, 14 SC and 11 AS. Osteomyelitis was acute in 20 cases and chronic in 23 cases. The different localisations are classified according to the type of osteomyelitis and the kind of bones concerned. Results  The 43 children presented 63 locations: 57 on long bones and 6 on short bones. The osteomyelitis was unifocal in 32 cases, and multifocal in 11 cases. The locations on long bones were humeral (18 cases), tibial (12 cases), femoral (9 cases), fibular (7 cases), radial (7 cases) and ulnar (4 cases). The 6 short bones included 3 metacarpus and 3 phalanxes. The 11 multifocal locations concerned 8 SS, 2 AS and 1 SC. In chronic osteomyelitis, 7 patients had sequestrum and 2 had pathological fracture. Conclusion  During examination of sickle-cell children with fever, particular attention must be shown, respectively to the arm, the leg and the thigh, in order to track down quickly, an acute osteomyelitis. Early diagnosis and quick treatment permit to avoid complications and heavy sequela in these children.     

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.     

Acute multilocular osteomyelitis in infancy and childhood

38 cases of acute multilocular osteomyelitis out of a total of 165 osteomyelitis patients were treated between 1974 and 1986 at the Department of Paediatric Surgery and Paediatric Diseases at the University of Mainz. Osteomyelitis in infants was separated from juvenile osteomyelitis because of the different problems inherent to this disease depending upon the age; likewise, multilocular remote involvement was separated from regional multiple involvement. It was found that distant involvement is of special importance in infancy. 6 of the 8 cases in this group presented with symmetrical involvement. This interesting phenomenon is described in this article for the first time in acute multilocular osteomyelitis; so far, it was known to occur only in chronic recurrent multifocal osteomyelitis (CRMO). The infants in this group were predominantly newborn who were particularly severely handicapped by predisposing factors such as necessary intensive-care measures or surgical interventions. Our findings showed that in this age group most of the problems occurred, namely: 3 deaths and 2 healing defects in 8 children. For this reason it is imperative to effect particularly close monitoring of multilocular acute osteomyelitis in infancy. This must be considered as a paediatric and paediatric-surgical emergency requiring not only accurate diagnostic measures but also early internistic and surgical treatment to improve the prognosis of this specific disease pattern.     

Pyoderma gangrenosum in a six-month-old boy

Pyoderma gangrenosum (PG) is an uncommon, chronic ulcerative condition of the skin that was first described in 1930. It can occur in any age group, but only 4% of the patients are infants or children. An underlying systemic disease is present in approximately 50% of the patients with PG. The most common associations include inflammatory bowel disease, arthritis, lymphoproliferative disorders and chronic recurrent multifocal osteomyelitis (CRMO). PG has been reported in association with CRMO in only a few children whose ages were between 18 months and 12 years. We report a six-month-old boy who was diagnosed as CRMO based on his clinical examination and histological findings. This is the youngest case reported in the literature (under 12 months of age) with PG associated with CRMO.     

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     

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.     

Multifocal sclerotic BCG spondylitis in a 13-year-old girl

A case is reported of multifocal BCG osteomyelitis in a 13-year-old girl. The lesions in the skull, ribs and in several vertebrae were mainly sclerotic and healed with antituberculotic therapy. The case is unusual because of the late onset, sclerotic changes, and involvement of the spine.