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 sclerosing osteomyelitis (so-called Garré's). Review of 12 cases

This study reports 12 cases of chronic sclerosing osteomyelitis in children. The authors recall the past history of this disease and describe the clinical, radiological and pathological picture found nowadays. They insist upon the salient features that allow to differentiate this lesion from other types of infectious osteomyelitis and osteoid osteoma.     

Chronic recurrent multifocal osteomyelitis-- I. Review

Juvenile and adolescent "Chronic Recurrent Multifocal Osteomyelitis" (CRMO) is described on the basis of literature and analysis of 43 own cases (23 cases in children or adolescents).This systemic, non-purulent inflammatory disease occurs mainly metaphyseal in long bones, in pelvic bones or as spondylitis and is not as rare as it seemed. Basis of the disease is a primarily chronic, sterile, in phase of onset often monotopic (e.g. clavicle) and later frequently polytopic osteomyelitis, possibly triggered by an immuno-pathological process (e.g. Proprionibacterium acnes), and showing histologically plasmacellular invasion and a sclerosing process in different stages. Association with pustulous dermatosis (psoriasis, acne, palmo-plantar pustulosis) is found in about 25 % of children and adolescents and in more than 50 % of the adult patients. 5 differents types of distribution of osteomyelitic lesions can be found by using Te99m-bone scan primarily, of which the "pelvic type" is the most common. Because of the close neighbourhood of meta-/epiphyseal osteomyelitic focuses, "sympathetic arthritis" with synovitis is seen frequently. A therapeutic approach with azithromycine and calcitonine is presented.     

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.     

Chronic multifocal osteomyelitis

The cases of three female Guinean children are described. Bloods tests were nonspecific, showing a moderately high globular sedimentation rate. The patients received combined therapy with systemic antibiotic therapy (including local gentamicin administration in two of the three patients) and surgery. One patient returned to Guinea and was lost to follow-up. The second patient showed severe sequelae and the third patient had a favorable outcome. In recent years, the prevalence of chronic osteomyelitis in Africa has increased. Most patients have multiple bone involvement and multiple etiology. Blood cultures are negative in 40 % of patients and severe radiologic abnormalities, most commonly fractures, are frequent. A successful therapeutic regimen must be based on antibiotic and surgical treatment.     

儿童慢性骨髓炎应用抗感染活性骨Ⅰ期植骨治疗的疗效评价

目的 验证抗感染活性骨(anti-infective reconstituted bone xenograft,ARBX)Ⅰ期植骨治疗儿童慢性骨髓炎的疗效.方法 自2001年9月对21例慢性骨髓炎患儿进行病灶清除,应用抗感染活性异种骨(anti-infective reconstituted bone xenograft,ARBX)Ⅰ期植骨治疗,其中获得12个月以上随访的儿童慢性骨髓炎患儿共16例.包括血源性慢性骨髓炎8例,原发性慢性骨髓炎3例,创伤性慢性骨髓炎5例.结果 16例中除1例骨髓炎复发,1例骨髓炎合并大段骨缺损在术后发生骨不连外,其余14例感染均获得控制无复发,病变部位均获得确实骨修复.本组感染治愈率(感染控制且无复发)为93.8%,完全治愈率(感染控制既无复发又完成骨修复)为87.5%.结论 ARBX具有高效诱导成骨活性和强效抗感染能力,是Ⅰ期植骨治疗儿童慢性骨髓炎的有效方法.     

Group A streptococcal osteomyelitis: severe presentation and course

AIM: To evaluate the course of group A streptococcal osteomyelitis associated with severe disease nowadays. METHODS: Three consecutive cases of severe group A streptococcal disease with osteomyelitis in children that were documented in Beer Sheva, Israel are described in detail. RESULTS: Two of the three cases were postvaricella. Early in the course of the disease, the presentation resembled that of severe cellulitis. All three patients had severe osteomyelitis and required surgery, and one patient developed chronic osteomyelitis. Sepsis was diagnosed in two cases. CONCLUSION: Our cases are distinguishable from typical haematogenous staphylococcal osteomyelitis by the severe course and the extensive involvement of bone and soft tissues. The increase in severity of invasive group A streptococcal infections documented throughout the world could account for the difference between our complex cases and the previous reports.     

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.     

Guillain-Barré syndrome presenting with severe musculoskeletal pain

Three children with Guillain-Barré syndrome are described who presented with musculoskeletal pain to an orthopaedic clinic or to a paediatric rheumatologist at a children's hospital. In each case, osteomyelitis of the spine was considered to be the most likely diagnosis and bone scans were performed in two of the three patients. Two of the three children required care in an intensive care unit, within hours of diagnosis. Outside of the specialty of neurology, the presentation of Guillain-Barré syndrome with severe muscle pain is not generally well known. With greater awareness of this particular presentation, dangerous delays in diagnosis and inappropriate investigations will be minimized.     

Unusual presentation of osteoid osteoma mimicking osteomyelitis in a 27-month-old infant

Osteoid osteoma is an uncommon, benign disorder of bone that is most often encountered in school-age children and in adolescents. It is quite rare in children under the age of 3 years. We report a case of osteoid osteoma with unusual clinical and imaging features in a 27-month-old toddler who presented with a limp and swelling of the right knee. Initial clinical evaluation, plain films, and computed tomography (CT) were strongly suggestive of chronic osteomyelitis involving the distal right femoral metadiaphysis. The appearance on nuclear medicine bone scan, however, was typical of osteoid osteoma. A biopsy of the lesion was taken and histological examination confirmed the diagnosis of osteoid osteoma. The misleading clinical and imaging features in this case are discussed.     

Clinical presentation and management of Pseudomonas osteomyelitis

To determine the incidence and clinical characteristics of Pseudomonas aeruginosa osteomyelitis in children, the records of 144 hospitalized patients under 19 years of age were reviewed; 104 fulfilled the study criteria for the diagnosis of acute or chronic osteomyelitis. Pseudomonas aeruginosa was recovered from 10.6 percent of the children and was the second most common pathogen isolated. In comparison to children with staphylococcal infections, patients with pseudomonal osteomyelitis were significantly older, gave an antecedent history of penetrating trauma, and lacked clinical and laboratory evidence of systemic illness. The data collected in this study suggest that osteomyelitis due to Pseudomonas aeruginosa is a distinct entity with clinical features differing from those of Staphylococcus aureus. Management should be directed at adequate surgical debridement followed by 10 to 21 days of antimicrobial therapy.     

Course and treatment of osteomyelitis in childhood (author's transl)]

In the years 1955-1972 132 children with osteomyelitis were treated in the Pediatric, Surgical and Orthopedic Department of the university of Kiel. There was no increase in the incidence of osteomyelitis during this period. Acute hematogenous osteomyelitis was diagnosed in 111 children, chronic hematogenous osteomyelitis in 11 children, traumatic and postoperative osteomyelitis in 10 children. Secondary chronic osteomyelitis occurred in 1 patient. Mainly staphylococci (in 90%) were the pathogenic bacteria, whereas haemophilus, pseudomonas, streptococci group A, E. coli and mixed infections occurred less frequently. In 17 of 111 patients with acute hematogenous osteomyelitis there were no roentgenological changes. Bacteriological investigations of blood and pus, and the antistaphylolysin reaction (repeated in the course of the disease) were helpful to establish the diagnosis in many cases. 107 of 111 patients with acute hematogenous osteomyelitis were cured (8 patients with defects). 4 children died in septic shock or because of complications (meningitis, pleural empyema, pneumonia). Bactericidal antibiotics in high dosage (penicillins, gentamicin) were superior to bacteriostatic antibiotics. Additional surgical treatment was necessary in 49 of 111 patients with acute hematogenous osteomyelitis. Recommendations for antibiotic therapy of osteomyelitis are given.     

Systematic review of systemic antibiotic treatment for children with chronic and sub‐acute pyogenic osteomyelitis

Background: There is a lack of consensus on the most appropriate antibiotics to treat children with chronic or sub‐acute pyogenic bacterial osteomyelitis and on the optimal duration of antibiotic therapy. Aim: To review the published evidence on the duration of antibiotic therapy and outcomes in children with chronic and sub‐acute pyogenic bacterial osteomyelitis. Methods: Systematic review of the literature. Results: We found no randomised controlled trials comparing different antibiotic regimens or comparing duration of antibiotic treatment for chronic or sub‐acute osteomyelitis in children. We found 14 observational case series published between 1973 and 2008. Most children with chronic osteomyelitis received 4–6 weeks of parenteral antibiotics followed by oral antibiotics to a total duration of 3–6 months. Small observational studies suggest that a shorter duration of parenteral and oral antibiotics may be equally effective. Conclusion: There is no high level evidence on the optimal duration of parenteral and oral antibiotics for children with chronic or sub‐acute osteomyelitis. A large randomised controlled trial is needed comparing short course parenteral and oral antibiotics with longer antibiotic duration.     

Vertebral osteomyelitis in children

Vertebral osteomyelitis is an uncommon disease in children, presenting with signs and symptoms that, at least initially, make a correct diagnosis difficult to be achieved. The authors report five cases of vertebral osteomyelitis in children, describing its clinical and laboratory peculiarities. The usefulness of the different radiologic studies for the diagnosis and follow up of these patients is discussed. We confirm previous reports according to which, with adequate clinical and therapeutic management, the prognosis is good for most patients.     

带血供腓骨移植Ⅰ期修复小儿慢性骨髓炎死骨缺损

目的：探讨小儿慢性骨髓炎大段死骨早期摘除后骨缺损的Ⅰ期修复效果。方法：16例胫骨慢性骨髓炎并大段死骨清除后，作吻合血管腓骨移植Ⅰ期重建；2例作炎性骨段切除，采用带血供腓骨移植Ⅰ期修复。结果：18例胫骨慢性骨髓炎在摘除大段死骨后，应用吻合血管腓骨移植或带血管蒂腓骨移植重建。术后平均随访26个月，见重建骨完全骨性愈合，累及的炎症得到完全控制；修复技体的功能，外形令人满意。结论：慢性骨髓炎所形成的大段死骨在作病灶清除的同时，进行早期的血管吻合或带血管腓骨移植Ⅰ期重建术，可免疫死骨爬行替代的漫长病程，改善受区血液循环，抑制炎症漫延，缩短了住院时间，阔手术次数，为骨髓炎早期死骨清除骨缺损的重建提供了有效外科治疗手段。     

Acute osteomyelitis and septic arthritis in children: a spectrum of disease

A spectrum of acute osteomyelitis and septic arthritis seen in children was studied, identifying four major groups, each with a different clinical presentation and different expected prognosis: (1) the usual group of osteomyelitis with a 10% incidence of poor results; (2) neonatal osteomyelitis (10% of osteomyelitis) with 25% poor results; (3) a group of severely ill patients with disseminated staphylococcal disease (10% of osteomyelitis) with 13% mortality and 38% poor results; and (4) the usual group of septic arthritis, with 5% poor results. Almost all cases of osteomyelitis were due to Staphylococcus aureus except in the neonatal group, where 28% were due to -haemolytic streptococci. Surgical drainage was performed in 91% of the usual osteomyelitis cases, in all with disseminated staphylococcal disease, and in 88% of babies with neonatal osteomyelitis, i.e. all the cases involving a joint. Cloxacillin was the drug of choice in osteomyelitis. In septic arthritis Staph. aureus was cultured in 29%, Haemophilus influenzae in 18%, streptococcus in 12%, pneumococcus in 6%, and no growth in 33%. Open arthrotomy was performed in all cases. Cloxacillin was used for cases infected with Staph. aureus, ampicillin for H. influenzae, and penicillin for streptococcal and pneumococcal infections. Offprint requests to: EB Hoffman     

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.     

Osteomyelitis in patient with sickle cell disease

From 1977 to 1989, 23 children with sickle cell disease were identified as having 21 episodes of acute and 3 episodes of chronic osteomyelitis, respectively. The responsible organisms were found in 17 cases: Salmonella (12 cases), coagulase-negative Staphylococcus (3 cases). Haemophilus influenzae (1 case), Escherichia coli (1 case). The mean age was 7 7/12 years. In 15 patients, osteomyelitis occurred in 1 bone; osteomyelitis of more than one bone was recorded in 9 cases. The most commonly affected bone was the femur (7 episodes); 5 episodes of hand-foot syndrome with osteomyelitis occurred in children in the first 2 years of life (mean age 16 months). Two patients had a Salmonella vertebral osteomyelitis. Incision and drainage were performed in 5 cases and bone aspiration in 9 cases. Etiologic agents were obtained with these two procedures in respectively 5 and 3 cases. Radionuclide scans were used in 7 episodes: uptake on bone scan was increased in 5 cases and normal in 2. In all cases, the outcome was satisfactory. Differentiation from acute bone infarcts in difficult. An extensive workup is required to confirm the diagnosis of infection: early scintigraphy, bone aspiration or surgical biopsy in patients with negative blood cultures should be performed. Until the results of cultures, the antimicrobial regimen chosen for initial therapy should be broad enough to treat the likely etiologic agents including Salmonella.     

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.     

Pelvic type of chronic recurrent multifocal osteomyelitis

We report about the juvenile and adolescent pelvic type of CRMO in 7 girls and 4 boys. The results show from solitary up to 7 pelvic lesions (average 3 lesions) predominantly in the acetabular and paraacetabular region (sometimes with hip joint effusion as a sign for concomitant coxitis).Tc99m-bone-scan is helpful to evaluate exactly the pattern of bone affection. We find 3 stages of an primarily chronic, non-purulent osteomyelitis going along with a "plasma-cell-sclerotic process", leading to a Garrè-type sclerosing end-stage, which probably heals after some years spontaneously, projecting on clinical symptoms and radiological appearance. The concomitant coxitis ("sympathetic coxitis") is clinically often in the foreground, but reversible. Pain in case of CRMO responds surprisingly well on medication with acithromycine.Knowing about CRMO in its different clinical appearances - especially concerning what we call "sympathetic coxitis" - can be a useful for pediatric rheumatologists and orthopedic surgeons, as well as MRI-focused radiologists and pathologists. Therapy might find a useful drug in acithromycine.In conclusion we d like to point out, that CRMO is one entity under the "roof" of the so called SAPHO-syndrome, which again shows us, that SAPHO-syndrome ist not a diagnosis itself but more a sign-post on the way to a correct diagnosis.