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.     

Salmonella vertebral osteomyelitis and epidural abscess in a child with sickle cell anemia

A case of Salmonella vertebral osteomyelitis with epidural abscess in a child with sickle cell anemia is presented. Spinal osteomyelitis is a rare event in children. Although osteomyelitis in sickle cell anemia may occur in any bone, it has most often been documented as beginning in the medullary cavity of the long and tubular bones. This is in contrast to the clinical presentation of osteomyelitis in the normal individual, who is likely to have infection beginning in and restricted to the metaphyseal regions of bones. Nonspecific or constitutional symptomatology may obscure the diagnosis of vertebral infection with ensuing cord compression. This case stresses the rapidity of development of paralysis or other neurologic complications, as well as the difficulty and emergent nature of the diagnosis of epidural abscess in this situation.     

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.     

Osteoid osteoma mimicking Brodie's abscess in a 13‐year‐old girl

Osteoid osteoma is a solitary, benign lesion of bone causing significant nocturnal pain. Magnetic resonance imaging (MRI), computed tomography (CT), and bone scan are commonly used in this diagnosis. A case of osteoid osteoma of the distal femur mimicking chronic osteomyelitis with Brodie's abscess is reported and discussed. Initial radiographs and MRI showed a lesion of the distal femur consistent with subacute osteomyelitis with a Brodie's abscess. Because primary malignant tumor could not be eliminated, surgical biopsy was carried out. Histological examination showed a typical nidus consistent with the diagnosis of osteoid osteoma. Subacute osteomyelitis (Brodie's abscess) may be difficult to distinguish from other malignant or benign bone lesions as osteoid osteoma. CT usually is recommended as the best imaging procedure to identify the nidus and confirm the diagnosis. MRI also can be used for this purpose. Surgical biopsy remains mandatory for unclear lesions before deciding on appropriate treatment.     

新生儿骨髓炎13例临床分析

目的 探讨新生儿骨髓炎的临床特点。方法 对重庆医科大学附属儿童医院2004—2012年诊断为新生儿骨髓炎的13例患儿的临床表现、实验室检查、影像学特征及治疗方案进行回顾性分析。 结果 本组13例。10例继发于败血症,3例由邻近感染扩散而来;四肢长骨尤其是股骨、肱骨最易受累,6例并发关节感染,最常见受累关节为髋关节、膝关节;最常见临床表现为局部红肿及肢体活动受限（假瘫痪）;血或脓液、关节液培养出致病菌12例,其中金黄色葡萄球菌7例,革兰阴性（G-）杆菌4例;所有患儿均行抗生素治疗, 4例联合手术治疗,随访7例均无后遗症发生。结论 新生儿骨髓炎并不罕见,临床表现不典型,应尽早完善影像学和病原学检查,早期使用足量敏感抗生素和必要时联合手术治疗可减少后遗症的发生。     

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.     

SAPHO osteomyelitis and sarcoid dermatitis in a patient with DiGeorge syndrome

We report the development and spontaneous resolution of annular erythematous skin lesions consistent with sarcoid dermatitis in a child with DiGeorge syndrome (DGS) carrying the 22q11.2 microdeletion. The skin lesion developed after she was treated with isoniazid (INH) following exposure to active tuberculosis (TB). After resolution of the skin lesions, this child developed sterile hyperplastic osteomyelitis consistent with SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) osteomyelitis in her right mandible triggered by an odontogenic infection. This child had congenital heart disease, dysmorphic facies, recurrent sinopulmonary infection, gastroesophgeal reflux disease, scoliosis, reactive periostitis, and developmental delay. She had a low CD4 and CD8 T cell count with a normal 4/8 ratio, but normal cell proliferation and T cell cytokine production in response to mitogens. When she was presented with sterile osteomyelitis of right mandible, she revealed polyclonal hypergammaglobulinemia with elevated erythrocyte sedimentation rate (ESR)/angiotensin converting enzyme (ACE) levels, but negative CRP. Autoimmune and sarcoidosis workup was negative. Inflammatory parameters gradually normalized following resolution of odontogenic infection and with the use of non-steroidal anti-inflammatory drugs (NSAIDs). The broad clinical spectrum of DGS is further expanded with the development of autoimmune and inflammatory complications later in life. This case suggests that patients with the DGS can present with unusual sterile inflammatory lesions triggered by environmental factors, further broadening the clinical spectrum of this syndrome.     

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??Abstract??Objective To expore the clinical features of acute osteomyelitis of neonate. Methods A retrospective study was done about clinical symptoms??radiologic features and treatments in 13 neonates who were diagnosed with osteomyelitis in Children’s Hospital Affliated to Chongqing University of Medical Sciences from 2004 to 2012. Results There were eleven male and two female infants??10 babies were related to bacteraemia and 3 cases were due to infection nearby.Limb bones were most commonly compromised. Six babies had joint involvement. Local swelling and disorder of limb’s activity were the most common clinical symptoms. Twelve patients got positve culture results from blood??pus or joint fluid samples. The most common pathogens were Staphylococcus aureus and Gram-negative bacteria. All of the babies accepted antibiotic treatment while 4 cases also underwent surgeries. All osteomyelitis patients had good outcomes. None had any sequelae among the 7 cases during the following 2 months to 2 years. Conclusion Neonatal osteomyelitis is not a rare disease with atypical clinical features. Radiologic and bacteriologic examination should be carried out once osteomyelitis is suspected. Appropriate antibiotics and necessary surgery are important to avoid further damage as well as long-term sequelae.     

Generalisatio BCG familiaris semibenigna,Osteomyelitis salmonellosa und Pseudotuberculosis intestinalis—Folgen eines familiären Makrophagendefektes?

Two siblings became very ill after BCG inoculation in the newborn period. A generalized BCG infection was assumed from the clinical symptomatology and the histological findings. Later on the boy suffered from enteric salmonellosis and osteomyelitis caused by the same organism, and as a todler he had intestinal pseudotuberculosis. Prompt recovery took place under selective antibiotic therapy. No known immunodeficiency disease could be correlated with this familial syndrome of increased susceptibility to intracellular vital microbes. A defective function of the patients macrophage system is suspected.     

Vertebral Langerhans-cell histiocytosis in childhood--a differential diagnosis of spinal osteomyelitis

BACKGROUND: The Langerhans cell histiocytosis has lots of different manifestations. Symptoms are not specific enough to identify the disease. In cases of vertebral Langerhans cell histiocytosis it is sometimes difficult to differentiate the lesions from spinal osteomyelitis. PATIENTS: Six children with Langerhans cell histiocytosis who had an operative treatment at our clinic in 1981 to 1995 have been reviewed. All patients had a localised vertebral presentation at the onset of the disease. METHODS: In this study we have been showed the clinical, radiological, histological and laboratory findings of our patients with Langerhans cell histiocytosis for an average of six years. Especially the use of diagnostic tools has been examined. RESULTS: Diagnostic tools such as radiogram, computerised tomography, bone scan and MRI and their value in finding the correct diagnosis have been described. Especially the use of MRI in early diagnosing has been discussed. Early changes in MRI have been presented as well as the useful radiological signs and MRI signs to differentiate Langerhans cell histiocytosis and spinal osteomyelitis. CONCLUSIONS: Vertebral Langerhans cell histiocytosis in childhood is a possible differential diagnosis to spinal osteomyelitis.     

Rib osteomyelitis in children. Early radiologic and ultrasonic findings

The earliest radiographic changes of osteomyelitis in the long bones is deep-seated edema manifesting as soft tissue swelling and obliteration of the intermuscular planes adjacent to the affected bone. Similarly, the early change of rib osteomyelitis is pericostal edema demonstrated by soft tissue swelling of the thoracic wall accompanied by an adjacent inward pleural displacement. In both osteomyelitis of the rib and the long bones, the bony changes will appear 1–2 weeks later. Pericostal edema can be readily diagnosed by ultrasound scan. Pericostal edema, although non specific and can occur in other conditions, yet it is a strong warning sign, set within the overall clinical picture of osteomyelitis.     

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.     

Osteomyelitis in the neonate

Osteomyelitis is defined as a bacterial infection of the musculoskeletal system. Osteomyelitis in the newborn is relatively rare, but if missed can have devastating, lifelong consequences for the growing infant. Those of us who care for neonates in intensive care units should have an understanding of this disease and be equipped to recognize it and begin treatment quickly to prevent long-term sequelae. This article reviews the pathophysiology of osteomyelitis and explains why the neonate is at an extraordinary risk for this disease. It reviews risk factors, clinical presentation, etiology and pathophysiology, diagnostic evaluation, treatment and monitoring of treatment, and long-term outcomes. A case study of a 27-week gestational age infant who presented with osteomyelitis and septic hip is presented, looking at the infant's hospital course and outcome.     

Yersinia enterocolitica osteomyelitis in a child

Yersinia enterocolitica systemic infections are uncommon and osteomyelitis due to this organism is very rare. We report a 9-year-old girl with thalassemia major, liver cirrhosis, and hemosiderosis who developed osteomyelitis of the rib caused by Y enterocolitica type 0:3. Serologic response to the infection was confirmed. No definite source of infection was identified.     

Oral alendronate in pediatric chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis (CRMO) primarily affects children and adolescents, and is characterized by episodic sterile osteomyelitis over several years. No definitive treatment is available. Non‐steroidal anti‐inflammatory drugs (NSAID) are common first‐line agents, but provide limited improvement in bone pain and do not affect disease duration. Several agents are utilized in the case of non‐response to NSAID, including corticosteroids, methotrexate, and tumor necrosis factor‐blocking agents. Bisphosphonates are increasingly being used. Most case series involve cyclic i.v. pamidronate, but this restricts the social lives of children and their families. Although oral medication has advantages over cyclic i.v. infusion because it does not require repeated hospital admissions, there have been no reports on treatment with oral bisphosphonates, such as alendronate, in pediatric CRMO patients. This case report describes the use of oral bisphosphonate as an alternative treatment in CRMO patients in whom standard therapy has failed.     

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.     

Culture-negative osteomyelitis

OBJECTIVE: To examine the natural history, clinical manifestations, laboratory changes and outcomes of children with culture-negative osteomyelitis and to compare findings with cases of culture-positive infection treated during the same time period. DESIGN: Retrospective review of consecutive admissions for osteomyelitis during the 4-year period 1998 through 2001, with a minimum follow-up of 1 year. SETTING: A single urban children's hospital with a large orthopedic referral service. CLINICAL AND LABORATORY MEASURES: Age, gender, predisposing factors, clinical manifestations, maximum temperature, duration of pain, bone involved, laboratory changes, results of cultures from infected bone and blood and outcome after treatment. RESULTS: A total of 85 patients fulfilled study criteria, of whom 40 were culture-negative. Compared with culture-positive cases, culture-negative osteomyelitis patients were less likely to have antecedent trauma (P = 0.0357) and overlying skin changes (P = 0.0001), duration of pain and other symptoms was longer (P = 0.0396) and skeletal residua were rare. They were also older, with this difference approaching statistical significance (P = 0.0586). CONCLUSIONS: Children with culture-negative osteomyelitis present initially differently from culture-positive cases and can be managed as presumed staphylococcal disease with excellent long term results.     

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.     

Ultrasonic signs of pelvic osteomyelitis in children

The ultrasonic findings were reviewed in 13 children in whom pelvic osteomyelitis was diagnosed by a positive^99mtechnetium methylene diphosphonate (MDP) bone scan in conjunction with clinical and laboratory features of osteomyelitis. All patients presented with pain in the region of the hip joint. In six patients the ultrasound study was confined to the hip joint, and all six had normal findings. In seven patients the ultrasound study was extended to include the pelvis. Deep soft tissue swelling was demonstrated in six of these, including a periosseous abscess in one case. Ultrasonography was negative in one patient with a 5-week history, whose pelvic osteomyelitis was resolving at the time of the ultrasound study. Oedema of the obturator internus and externus muscles was observed in osteomyelitis affecting the pubis and ischium, and of the iliacus and/or the gluteus medius muscle in osteomyelitis of the ilium. In children presenting with hip pain who have a normal hip ultrasound study, extension of the ultrasound examination to include these four pelvic muscles may help to identify and document the progression of acute pelvic osteomyelitis.     

Chronische nichtbakterielle und bakterielle Osteomyelitis

Inflammatory bone diseases in childhood and adolescence can lead to substantial impairment of the quality of life as well as mental and physical damage. In contrast to the previous assumption that osteomyelitis exclusively is the result of infections, noninfectious (auto) inflammatory causes are becoming more appreciated. The most frequent autoinflammatory bone disease is chronic nonbacterial osteomyelitis (CNO), which predominantly affects the skeleton but can also cause inflammatory alterations of the skin, intestines and joints. The broad clinical spectrum ranges from timely limited inflammation of individual bones to chronically active or recurrent courses with symmetrical manifestation in several bones. This article summarizes data on differential diagnoses to infectious and noninfectious osteomyelitis and provides evidence-based recommendations for differential diagnostics and treatment of CNO.