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Purpose: The purpose of this study was to evaluate the diagnostic value of 18 F-fluorodeoxyglucose (FDG) positron emission tomography and computed tomography (PET/CT scan) and magnetic resonance imaging (MRI) in diagnosing spondylodiscitis and its complications, such as epidural and paraspinal abscesses., Methods: From January 2006 to August 2013 patients with a clinical suspicion of spondylodiscitis, with an infection, or with fever of unknown origin were retrospectively included if 18 F-FDG-PET/CT and MRI of the spine were performed within a 2-week time span. Imaging results were compared to the final clinical diagnosis and follow-up data were collected., Results: Sixty-eight patients were included of whom 49 patients were diagnosed with spondylodiscitis. MRI showed an overall sensitivity of 67 % and specificity of 84 %. Diagnostic accuracy was 58 %, when MRI was performed within 2 weeks after the start of symptoms and improved to 82 %, when performed more than 2 weeks after onset of symptoms. 18 F-FDG-PET/CT showed a sensitivity of 96 % and a specificity of 95 %, with no relation to the interval between the scan and the start of symptoms., Conclusions: As compared to MRI, 18 F-FDG-PET/CT has superior diagnostic value for detecting early spondylodiscitis. After 2 weeks both techniques perform similarly., Competing Interests: Compliance with ethical standards Ethical standards According to the Dutch law, this study was exempt from approval by an ethics committee, because of the retrospective character of this study and the anonymous storage of data. Conflict of interest The authors declare that they have no conflict of interest.

Background and Objectives: Both enchondroma and atypical cartilaginous tumors (ACT) are not considered malignant, so inactive and asymptomatic tumors might not need surgery. To the best of our knowledge, this is the first study that has been done to evaluate the natural course of conservative-treated enchondroma and ACT in the long bones., Methods: For this retrospective study, we analyzed the results of patients in whom we refrained from surgery and only regularly performed radiological follow-up of the tumor. Minimal follow-up after initial diagnosis was 24 months., Results: Forty-nine patients were included in this study. Eight out of forty-nine cases received surgical treatment during follow-up of the tumor. The reasons for this surgery were radiologic growth of the tumor in two cases, pain in one case, patient request in three cases, another indication for surgery in the same limb in two cases., Conclusion: In this small series of conservatively treated enchondroma and ACT, only 6% of the patients had a medical indication for surgery. This study shows that indication for surgery should be discussed more thoroughly. Based on our results, we would recommend annual radiologic follow-up for asymptomatic enchondroma or ACT in the long bones, irrespective of tumor size. J. Surg. Oncol. 2016;114:987-991. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Synovial sarcoma is the fourth most common type of soft-tissue sarcoma (following undifferentiated pleomorphic sarcoma, liposarcoma, and rhabdomyosarcoma), and should be considered a high-grade neoplasm with a high number of local recurrences and late metastases. Synovial sarcoma predominantly occurs in adolescents and young adults, and typically arises near the joints of the lower extremity. However, this tumor can also occur at uncommon sites such as the abdominal wall, which is illustrated in this article. Furthermore, we reviewed the available literatures on the clinical, pathological and radiological appearances, as well as the current knowledge concerning treatment options and prognosis.

Facioscapulohumeral muscular dystrophy is a slowly progressive hereditary disorder resulting in fatty infiltration of eventually most skeletal muscles. Weakness of trunk and leg muscles causes problems with postural balance and gait, and is associated with an increased fall risk. Although drop foot and related tripping are common problems in FSHD, gait impairments are poorly documented. The effect of ankle plantarflexor involvement on gait propulsion has never been addressed. In addition to ankle plantarflexion, gait propulsion is generated through hip flexion and hip extension. Compensatory shifts between these propulsion sources occur when specific muscles are affected. Such a shift may be expected in patients with FSHD since the calves may show early fatty infiltration, whereas iliopsoas and gluteus maximus muscles are often spared for a longer time. In the current study, magnetic resonance imaging was used to assess the percentage of unaffected calf, iliopsoas and gluteus maximus muscles. Joint powers were analyzed in 10 patients with FSHD at comfortable and maximum walking speed to determine the contribution of ankle plantarflexor, hip flexor and hip extensor power to propulsion. Associations between muscle morphology, power generation and gait speed were assessed. Based on multivariate regression analysis, ankle plantarflexor power was the only factor that uniquely contributed to the explained variance of comfortable (R(2)=80%) and maximum (R(2)=86%) walking speed. Although the iliopsoas muscles were largely unaffected, they appeared to be sub-maximally recruited. This submaximal recruitment may be related to poor trunk stability, resulting in a disproportionate effect of calf muscle affliction on gait speed in patients with FSHD., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Background: Although it is known that muscle weakness is a major cause of postural instability and leads to an increased incidence of falls in patients with neuromuscular disease, the relative contribution of lower extremity and trunk muscle weakness to postural instability has not been studied well., Methods: We determined the relationship between muscle fatty infiltration and sagittal-plane balance in ten patients with facioscapulohumeral muscular dystrophy. Sagittal-plane platform translations were imposed in forward and backward directions on patients with facioscapulohumeral muscular dystrophy and healthy controls. Stepping thresholds were determined and kinematic responses and center-of-mass displacements were assessed using 3 dimensional motion analysis. In the patients, magnetic resonance imaging was used to determine the amount of fatty infiltration of trunk and lower extremity muscles., Findings: Stepping thresholds in both directions were decreased in patients compared to controls. In patients, significant correlations were found for fatty infiltration of ventral muscles with backward stepping threshold and for fatty infiltration of dorsal muscles with forward stepping threshold. Fatty infiltration of the rectus abdominis and the back extensors explained the largest part of the variance in backward and forward stepping thresholds, respectively. Center-of-mass displacements were dependent on intensity and direction of perturbation. Kinematic analysis revealed predominant ankle strategies, except in patients with lumbar hyperlordosis., Interpretation: These findings indicate that trunk muscle involvement is most critical for loss of sagittal-plane postural balance in patients with facioscapulohumeral muscular dystrophy. This insight may help to develop rehabilitation strategies to prevent these patients from falling., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Afibrinogenaemia is an autosomal recessive disease with an estimated prevalence of approximately one in a million. The most common symptoms of afibrinogenaemia are umbilical cord bleeding, bleeding into skin, mouth, muscles, gastrointestinal and genitourinary tracts and the central nervous system. Other recognized complications include; haemarthroses, spontaneous splenic rupture, epistaxis, menorrhagia, recurrent abortion and venous and arterial thromboembolism. Bone cysts have also been described as a rare complication of afibrinogenaemia. The aim of this study was to conduct a systematic literature review, summarize the reported cases and to report two new cases. Three electronic databases were searched for relevant publications: PubMed, Medline and EMBASE. The following search criteria were used: '(bone cysts OR intraosseous haematoma OR intraosseous haemorrhage) AND (afibrinogenaemia OR fibrinogen deficiency)'. The reference lists of the selected papers were searched for more relevant literature. In total, eight patients had bone cysts as complication of afibrinogenaemia and six of them suffered from pain in their extremities. Bone cysts were primarily located in the vicinity of the cortex or trabeculae in the diaphysis of the long bones, especially in the femora, tibiae and humeri. Some were regressive, probably due to reactive bone remodelling. A number of cysts were filled with serosanguinous fluid. It might be useful to check for bone cysts when patients with congenital afibrinogenaemia complain of 'rheumatic' pains in their extremities. Whole body magnetic resonance imaging is the diagnostic imaging technique of choice. Recurrent episodes of pain, but not radiological deterioration, appear to benefit from prophylactic therapy with fibrinogen concentrate., (© 2013 John Wiley & Sons Ltd.)

Facioscapulohumeral muscular dystrophy (FSHD) is an untreatable disease, characterized by asymmetric progressive weakness of skeletal muscle with fatty infiltration. Although the main genetic defect has been uncovered, the downstream mechanisms causing FSHD are not understood. The objective of this study was to determine natural disease state and progression in muscles of FSHD patients and to establish diagnostic biomarkers by quantitative MRI of fat infiltration and phosphorylated metabolites. MRI was performed at 3T with dedicated coils on legs of 41 patients (28 men/13 women, age 34-76 years), of which eleven were re-examined after four months of usual care. Muscular fat fraction was determined with multi spin-echo and T1 weighted MRI, edema by TIRM and phosphorylated metabolites by 3D (31)P MR spectroscopic imaging. Fat fractions were compared to clinical severity, muscle force, age, edema and phosphocreatine (PCr)/ATP. Longitudinal intramuscular fat fraction variation was analyzed by linear regression. Increased intramuscular fat correlated with age (p<0.05), FSHD severity score (p<0.0001), inversely with muscle strength (p<0.0001), and also occurred sub-clinically. Muscles were nearly dichotomously divided in those with high and with low fat fraction, with only 13% having an intermediate fat fraction. The intramuscular fat fraction along the muscle's length, increased from proximal to distal. This fat gradient was the steepest for intermediate fat infiltrated muscles (0.07±0.01/cm, p<0.001). Leg muscles in this intermediate phase showed a decreased PCr/ATP (p<0.05) and the fastest increase in fatty infiltration over time (0.18±0.15/year, p<0.001), which correlated with initial edema (p<0.01), if present. Thus, in the MR assessment of fat infiltration as biomarker for diseased muscles, the intramuscular fat distribution needs to be taken into account. Our results indicate that healthy individual leg muscles become diseased by entering a progressive phase with distal fat infiltration and altered energy metabolite levels. Fat replacement then relatively rapidly spreads over the whole muscle.

This systematic review of studies compared magnetic resonance imaging (MRI), (18) F-fluorodeoxyglucose positron emission tomography (FDG-PET), FDG-PET with computerized tomography (PET-CT) and CT with whole body X-Ray (WBXR) or (whole body) CT in order to provide evidence-based diagnostic guidelines in multiple myeloma bone disease. A comprehensive search of 3 bibliographic databases was performed; methodological quality was assessed using Quality Assessment of Diagnostic Accuracy Studies (QUADAS) criteria (score 1-14). Data from 32 directly comparative studies were extracted. The mean QUADAS score was 7·1 (3-11), with quality hampered mainly by a poor description of selection and execution criteria. All index tests had a higher detection rate when compared to WBXR, with up to 80% more lesions detected by the newer imaging techniques; MRI (1·12-1·82) CT (1·04-1·33), PET (1·00-1·58) and PET-CT (1·27-1·45). However, the modern imaging techniques detected fewer lesions in the skull and ribs. In a direct comparison CT and MRI performed equally with respect to detection rate and sensitivity. This systematic review supports the International Myeloma Working Group guidelines, which recommend that WBCT can replace WBXR. In our opinion, the equal performance of MRI also indicates that it is a valuable alternative. As lesions of the skull and ribs are underdiagnosed by modern imaging techniques we advise additional X-rays of these regions. The consequences of this approach are discussed., (© 2013 John Wiley & Sons Ltd.)

Ten years after chemoradiation for primary lymphoma of the left pelvic bone, a 38-year-old man presented with a 4-month history of gradually increasing pain in his left upper leg and thigh. Initial radiographs and contrast-enhanced magnetic resonance imaging were consistent with recurrent lymphoma, infection, or postirradiation sarcoma. Subsequent F-18 fluorodeoxyglucose positron emission tomography/computed tomography demonstrated a focal area of F-18 fluorodeoxyglucose-avidity within the previously irradiated bone consistent with tumor and the location was confirmed by advanced magnetic resonance imaging techniques and histopathology, thus optimizing treatment planning.

Introduction: Mazabraud's syndrome is a rare but well-described disorder characterized by fibrous dysplasia in single or multiple bones associated with one or more soft-tissue myxomas. In this report, we describe what is, to the best of our knowledge, the first case involving an intra-osseous myxoma. This finding supports, and could provide new insight into, the pathological association between fibrous dysplasia and myxomas., Case Presentation: In this report, we describe the case of a 49-year-old Caucasian woman known for years to have fibrous dysplasia in the left femur and tibia who presented with progressive pain in her left leg and soft swelling in the left quadriceps region. After surgical intervention with excision of the soft-tissue mass, the diagnosis of Mazabraud's syndrome was confirmed. During follow-up, our patient presented with a painless mass located on the lateral side of the left knee, next to a second, intra-osseous lesion with the same characteristics in the left lateral tibial plateau. The histopathological examination was consistent with a soft-tissue intra-osseous myxoma., Conclusion: In the international literature, 67 cases of Mazabraud's syndrome have been described so far.To our knowledge, the present case report is the first to describe the combination of polyostotic fibrous dysplasia and intra-muscular as well as intra-osseous myxoma.

The aim of this study was to investigate anteroposterior instability in the CKS and the PFC total knee arthroplasty (TKA) designs. Physical examinations, including VAS, IKS and WOMAC were performed in combination with a detailed fluoroscopic measurement technique for three-dimensional kinematic assessment of TKA design function. Anteroposterior instability rated with the IKS was not significantly different (p=0.34), but patients with a CKS design showed more limitations according to the WOMAC joint stiffness total score, and for items regarding higher flexion activities in the WOMAC score for knee disability. Kinematic analyses showed that the CKS design tended to have more anterior sliding of the femur on the tibia during mid- and deep flexion activities. The sliding distance was larger at the medial than at the lateral side. This phenomenon has also been described for posterior cruciate ligament deficient knees. Furthermore, the CKS design showed a significantly lower range of tibial rotation (p<0.05) from maximum extension to maximum flexion during deep knee bend activities. Kinematic differences can be ascribed to posterior cruciate ligament deficiency/laxity or differences in TKA designs.

Background: Aneurysmal bone cysts (ABCs) are most often treated with intralesional surgery (curettage) and additional bone grafting. There is debate on whether or not to use adjuvant therapy to decrease the local recurrence rate. This study is done to assess the outcome of curettage and cryosurgery as a treatment of ABC., Methods: We analyzed 80 consecutive cases of ABC treated with curettage and cryosurgery. Patients were followed minimal 24 months after surgery (average 55 months, range 24-122 months) with physical examination and radiographs. Functional outcome was evaluated using the musculoskeletal tumor society score (MSTS)., Results: The 80 patients were all treated with curettage and cryosurgery. Additional bone grafting was used in 73 patients, osteosynthesis in 12 and spondylodesis in 1 patient. Four local recurrences were found in this study, a recurrence rate of 5%. All local recurrences were treated successfully with curettage and cryosurgery again 7-33 months after the initial surgery. Postoperative one fracture, one wound infection, and three transient nerve palsy occurred. The average MSTS score was 29.2 at follow-up., Conclusions: The use of cryosurgery as adjuvant therapy results in a lower local recurrence rate when compared to other publications for the treatment of ABC and excellent functional results., (Copyright 2009 Wiley-Liss, Inc.)

Multiple myeloma is a malignant B-cell neoplasm that involves the skeleton in approximately 80% of the patients. With an average age of 60 years and a 5-years survival of nearly 45% Brenner et al. (Blood 111:2516-2520,35) the onset is to be classified as occurring still early in life while the disease can be very aggressive and debilitating. In the last decades, several new imaging techniques were introduced.The aim of this review is to compare the different techniques such as radiographic survey, multidetector computed tomography (MDCT), whole-body magnetic resonance imaging (WB-MRI), fluorodeoxyglucose positron emission tomography-(FDG-PET) with or without computed tomography(CT), and 99mTc-methoxyisobutylisonitrile (99mTc-MIBI) scintigraphy. We conclude that both FDG-PET in combination with low-dose CT and whole-body MRI are more sensitive than skeleton X-ray in screening and diagnosing multiple myeloma. WB-MRI allows assessment of bone marrow involvement but cannot detect bone destruction, which might result in overstaging. Moreover,WB-MRI is less suitable in assessing response to the rapythan FDG-PET. The combination of PET with low-dose CT can replace the golden standard, conventional skeletal survey. In the clinical practise, this will result in upstaging,due to the higher sensitivity.

Objective: To evaluate the effect of implant location and skin thickness on the frequency and degree of adverse skin reactions around the abutment., Design: Retrospective multivariate analysis of implant position related to skin thickness and clinical variables., Setting: Tertiary referral center., Patients: Random sample of 248 patients with bone-anchored hearing aids., Interventions: Bone-anchored hearing aid implant placement by means of the linear incision technique., Mean Outcome Measures: Type and number of skin reactions and implant loss., Results: The mean (SD) distance from the external auditory ear canal to implant was 48.8 (8.0) mm (range, 29-84 mm). The mean skin thickness was 5.5 (1.9) mm. Severe skin reactions (Holgers classification, 2-4) were seen in 46 of the 248 patients (18.5%). Implant loss occurred in 4 patients (1.6%). Three implants were lost owing to failed osseointegration (1.3%), and another implant was removed because of deterioration of cochlear function (0.9%). No implant was lost as a result of infection., Conclusion: Implant location and skin thickness were not correlated with implant loss or the frequency or degree of adverse skin reactions around the abutment.

Background and Objectives: Cryosurgery using liquid nitrogen is used as adjuvant treatment after intralesional resection of bone tumours to induce cell death. It is applied to enlarge the oncological margins of resection and to reduce the local recurrence rate. The objective of this study is to analyze the oncological and functional results., Methods: We studied the oncological and functional results of curettage and cryosurgery in 123 patients with 130 tumors. There were 75 enchondromas and 55 chondrosarcomas grade 1. The minimal follow-up period for all patients was 2 years (range 24-119 months)., Results: During follow-up one local recurrence of an active enchondroma and one local recurrence of an aggressive enchondroma occurred. They were treated with curettage and cryosurgery again. Both patients were disease-free at minimum of 3 years follow-up. No local recurrences after treatment of chondrosarcoma grade 1 were seen. Functional scores, according to the MSTS scoring system, showed an average score of 28 points (94%) at 2 years follow-up. Post-operative fractures were seen in 18 patients (14%)., Conclusions: Curettage and cryosurgery for enchondroma and chondrosarcoma grade 1 has excellent oncological and functional results. The post-operative management has been adjusted to reduce the number of fractures., ((c) 2008 Wiley-Liss, Inc.)

A correct histological diagnosis, careful staging and detection of tumour response to treatment are all crucial in the management of sarcomas. Imaging is important in all of these stages. Sarcomas have distinct biological and treatment-related features posing challenges for imaging. For example, size measurements may not adequately reflect response rates. Techniques which can measure tissue function rather than generate merely anatomical data such as positron emission tomography (PET) are rapidly gaining interest. We discuss the importance of imaging in different stages of patient management, emphasising the unique characteristics of sarcoma. Furthermore, we discuss the potential of PET for the various indications, focussing on therapy evaluation.

The aim of this study was to determine the optimal combination of preoperative examination methods to predict mandibular invasion by squamous cell carcinoma of the oral cavity. Data were gathered prospectively but evaluated retrospectively. The preoperative results of clinical examination, conventional radiography, bone single photon emission computed tomography (SPECT), computed tomography and magnetic resonance imaging were compared to the histological results of resection specimens from 67 patients with tumours, adjacent or fixed to the mandible, histologically confirmed as squamous cell carcinoma. The examination methods with acceptable sensitivity and specificity were selected and diagnostic algorithms were constructed using all possible combinations. The preferred diagnostic algorithm was found to be either computed tomography or magnetic resonance imaging, followed by a bone SPECT in cases where the first scan is negative. A negative bone SPECT rules out mandibular invasion (100% sensitivity). This algorithm accurately predicted mandibular invasion in 85% of the patients, without yielding false negative results. In this study group, application of such an algorithm would have resulted in a reduction of the number of unnecessary mandibular resections by 50%. The suggested, preferred, diagnostic algorithm shortens the preoperative screening process, avoiding unnecessary costs, as well as considerably reducing the number of unnecessary mandibular resections.

Background: Chondroblastoma of bone is a rare tumor that occurs most often in the epiphysis or apophysis of long bones. This benign tumor sometimes shows aggressive or malignant behaviour, and rarely metastases occur. Since wide resection often leads to growth impairment, intra-lesional curettage followed by an adjuvant therapy is developed to decrease the negative effects on growth and function without increased recurrence rates., Patients and Methods: Between 1980 and 2001, 18 patients were treated for chondroblastoma of bone. Since 1989 cryosurgery has been used as an adjuvant treatment. The functional and oncologic results at follow-up were studied., Results: Four patients were treated with curettage, and four with wide resection without adjuvant therapy. One patient developed a local recurrence and distant metastases. Ten patients were treated with curettage and cryosurgery. One patient developed a local recurrence 6 years later that was treated with curettage and cryosurgery again. No serious complications from cryosurgery occurred and the functional results at follow-up were excellent [mean MSTS score at follow-up 29]., Conclusions: Chondroblastoma of bone is a benign tumor with a relatively high recurrence rate. The local recurrence rates can be decreased with cryosurgery as a local adjuvant therapy with excellent functional results., ((c) 2007 Wiley-Liss, Inc.)

Background: The condition of pneumatosis intestinalis is characterized by gas in the intestinal wall, with a variety of causes., Case: A septic patient with intestinal pneumatosis, locally advanced ovarian cancer, and Sister Joseph's Nodule., Conclusion: The clinical significance of this rare combination of symptoms and signs is discussed.

Nail-patella syndrome (NPS) is characterized by developmental defects of dorsal limb structures, nephropathy, and glaucoma and is caused by heterozygous mutations in the LIM homeodomain transcription factor LMX1B. In order to identify possible genotype-phenotype correlations, we performed LMX1B mutation analysis and comprehensive investigations of limb, renal, ocular, and audiological characteristics in 106 subjects from 32 NPS families. Remarkable phenotypic variability at the individual, intrafamilial, and interfamilial level was observed for different NPS manifestations. Quantitative urinanalysis revealed proteinuria in 21.3% of individuals. Microalbuminuria was detected in 21.7% of subjects without overt proteinuria. Interestingly, nephropathy appeared significantly more frequent in females. A significant association was established between the presence of clinically relevant renal involvement in an NPS patient and a positive family history of nephropathy. We identified normal-tension glaucoma (NTG) and sensorineural hearing impairment as new symptoms associated with NPS. Sequencing of LMX1B revealed 18 different mutations, including six novel variants, in 28 families. Individuals with an LMX1B mutation located in the homeodomain showed significantly more frequent and higher values of proteinuria compared to subjects carrying mutations in the LIM domains. No clear genotype-phenotype association was apparent for extrarenal manifestations. This is the first study indicating that family history of nephropathy and mutation location might be important in precipitating individual risks for developing NPS renal disease. We suggest that the NPS phenotype is broader than previously described and that NTG and hearing impairment are part of NPS. Further studies on modifier factors are needed to understand the mechanisms underlying phenotypic heterogeneity.

The diagnosis of osteomyelitis remains a difficult diagnostic dilemma. In this article, which is particularly aimed at those whose practice does not include a large paediatric population, we review the pathophysiology of paediatric osteomyelitis and contrast it with the available imaging modalities. We examine the role of the radiologist as well as the usefulness of each modality. Secondly, we review the different clinical scenarios such as acute, subacute and chronic, as well as specific forms of osteomyelitis; the latter includes subacute chronic ostemyelitis, toxic synovitis, spondylodiscitis as well as the congenital inflammatory disorders such as rubella and syphylis. The most useful imaging findings to look for and their significance are assessed and we evaluate their usefulness in each case. Close cooperation between clinicians and imagers remains the key to early and adequate diagnosis of paediatric osteomyelitis.

Equinovarus foot deformity as seen in neurologic disease is often caused by imbalance in muscle tone. One of the possible operative procedures to correct this deformity is transposition of the posterior tibial tendon. The effect of this intervention is disputed in the orthopaedic literature. The results of this procedure are presented in a retrospective study of 9 children (11 feet). The mean follow-up period was 19 months (8-40). In all cases but one the results were satisfactory. The indications and complications of this operation are discussed.