Long-term follow-up of the cervical spine with conventional radiographs in patients with rheumatoid arthritis.

OBJECTIVES: To investigate the prevalence of cervical spine damage due to rheumatoid arthritis (RA) in the long term and to investigate which disease-specific factors are related to this damage. METHOD: Patients with early RA from the Nijmegen inception cohort with 6 to 12 years of follow-up were included. Conventional radiographs of the cervical spine were obtained at baseline, 3, 6, 9, and 12 years and scored for erosions of C1 and C2, anterior atlantoaxial subluxation (AAS) and atlantoaxial impaction (AAI). Disease-specific factors, such as disease activity, functionality, and peripheral joint damage, at baseline, 3, 6, and 9 years, were compared between patients with and without cervical spine damage at 9 years. RESULTS: A total of 196 patients were included, of whom 134 had radiographs at 9 years. Cervical spine damage was present in 16% (22/134) of the patients at 9 years. During the total 12 years of follow-up, AAS and erosions of C2 were observed most frequently. Erosions of C1 and AAI were very rare. Patients with cervical spine damage at 9 years had a higher number of erosions of the peripheral joints and failed more disease-modifying anti-rheumatic drugs (DMARDs) at 3, 6, and 9 years. Patients without peripheral erosive disease at 3 years were unlikely to develop cervical spine damage within 9 years of disease duration. CONCLUSIONS: The prevalence of cervical spine damage due to RA was 16% at 9 years. Patients without peripheral erosive disease at 3 years were unlikely to develop cervical spine damage at 9 years.

Prekallikrein deficiency in a 15-year-old boy with Ménière's disease: a case report.

Congenital prekallikrein deficiency is a rare disorder in which there is an in vitro clotting defect despite absence of bleeding or thrombotic tendency. In this report, a 15-year-old boy with an unexpected markedly prolonged activated partial thrombin time, a normal prothrombin time, and without personal nor familial history of bleeding or thrombosis is presented. Laboratory investigation revealed a severe prekallikrein deficiency. This case highlights the importance of following a diagnostic algorithm to establish the correct diagnosis. Moreover, by selecting appropriate laboratory tests, unnecessary and repeatedly testing can be avoided.

Myelofibrosis patients in Belgium: disease characteristics.

OBJECTIVE: To date, only a small number of epidemiological studies on myelofibrosis have been performed. The current study aimed to characterize the myelofibrosis patient population in Belgium according to pre-defined disease parameters (diagnosis, risk categories, hemoglobin <10 g/dl, spleen size, constitutional symptoms, platelet count, myeloblast count), with a view to obtaining a deeper understanding of the proportion of patients that may benefit from the novel myelofibrosis therapeutic strategies. METHODS: A survey was used to collect data on prevalence and disease parameters on all myelofibrosis patients seen at each of 18 participating hematologic centers in 2011. Aggregated data from all centers were used for analysis. Analyses were descriptive and quantitative. RESULTS: A total of 250 patients with myelofibrosis were captured; of these, 136 (54%) were male and 153 (61%) were over 65 years old. One hundred sixty-five (66%) of myelofibrosis patients had primary myelofibrosis and 85 (34%) had secondary myelofibrosis. One hundred ninety-three myelofibrosis patients (77%) had a palpable spleen. About a third of patients (34%) suffered from constitutional symptoms. Two hundred twenty-two (89%) myelofibrosis patients had platelet count ≧50 000/µl and 201 (80%) had platelet count ≧100 000/µl. Of 250 patients, 85 (34%) had a myeloblast count ≧1%. Six (2%) patients had undergone a splenectomy. Thirteen (5·2%) patients had undergone radiotherapy for splenomegaly. CONCLUSIONS: The results of this survey provide insight into the characteristics of the Belgian myelofibrosis population. They also suggest that a large proportion of these patients could stand to benefit from the therapies currently under development.

Nuclear arthrography: combined scintigraphic and radiographic procedure for diagnosis of total hip prosthesis loosening.

UNLABELLED: Radiographic arthrography and bone scintigraphy are common diagnostic procedures used for evaluating total hip prostheses. In this study, both techniques are combined, and nuclear contrast imaging (nuclear arthrography) is added. The efficacy of the procedures is evaluated. METHODS: After intravenous injection of 99mTc-methylene diphosphonate (MDP), standard radiographic arthrography was performed in 105 patients (107 prostheses). The radiographic contrast medium was mixed with insoluble 111In-colloid (5 MBq/20 ml). After completion of the radiographic arthrography, nuclear arthrography was performed, and multiple-view dualisotope images (111In, 247-keV peak only) were recorded. Images were interpreted by superposition of the 111In image and the corresponding 99mTc-MDP image, the latter serving as a landmark for the position of the prosthesis and osseous structures. Findings at surgery were used as the gold standard. RESULTS: In both cemented and uncemented acetabular and femoral components, nuclear arthrography performed better than or equal to radiographic arthrography (70%-90% and 60%-75%, respectively). Nuclear arthrography had higher diagnostic accuracy than 99mTc-MDP images alone. CONCLUSION: Nuclear arthrography is a sensitive technique for detection of loosening of prostheses, offering added value over radiographic arthrography and bone scanning alone, especially for evaluation of the femoral component. Radiographic arthrography remains necessary not only for adequate deposition of contrast agents but also for detailed evaluation of osseous structures.

Evaluation of infectious diabetic foot complications with indium-111-labeled human nonspecific immunoglobulin G.

Osteomyelitis of the foot is a well-known complication of diabetes mellitus. In this study, the validity of 111In-labeled human nonspecific immunoglobulin G (IgG) scintigraphy was studied in 16 diabetic patients with foot ulcers, gangrene or painful Charcot joints. In all patients, plain radiographs, conventional bone scan images and 111In-IgG images were recorded. The results were verified by histologic examination of surgical specimens in patients who did not respond to antibiotic treatment within 2-3 wk (10 lesions) or long-term clinical follow-up of at least 6-mo (16 lesions). On the bone scans, all seven osteomyelitic foci were detected. However, 19 additional foci not due to osteomyelitis were seen. The absence of true-negative bone scans in this study resulted in a specificity of 0%. On the plain radiographs, four of seven osteomyelitis foci were detected; for 111In-IgG scintigraphy, six of seven (sensitivity 57% and 86%, respectively). Plain radiographs correctly ruled out osteomyelitis in 15 of 19 lesions, 111In-IgG scintigraphy in 16 of 19 (specificity 79% and 84%, respectively). All imaging procedures gave false-positive results in penetrating ulcers over the calcaneus in two patients and in one patient with a Charcot joint, most likely due to recent fractures. A false-negative 111In-IgG study was observed in a patient with severe arterial angiopathy. Accurate estimation of probable osteomyelitis was not possible from the results of soft-tissue cultures, since in only 6 of 12 positive cultures, osteomyelitic foci could be proven. Indium-111-IgG scintigraphy can contribute to adequate evaluation of osteomyelitis in diabetic foot complications because it improves specificity when compared to bone scan and radiographic findings and improves sensitivity in comparison to plain radiographs.

Scintigraphic evaluation of experimental chronic osteomyelitis.

UNLABELLED: Assessment of disease activity and disease extent in chronic osteomyelitis remains a difficult diagnostic problem. Radiography is not particularly sensitive. Scintigraphic techniques can be more helpful, but the routinely available agents lack specificity (99mTc-methylene diphosphonate [MDP], 67Ga-citrate) or are laborious to prepare (111In-leukocytes). We evaluated the performance of 2 new radiopharmaceuticals, 99mTc-polyethyleneglycol (PEG) liposomes and 99mTc-hydrazinonicotinamide (HYNIC)-immunoglobulin G (IgG), in an experimental model of chronic osteomyelitis. METHODS: Chronic osteomyelitis was induced in rabbits by inserting S. aureus into the right reamed and washed femoral canal. The canal was closed with cement. A sham operation was performed on the left femur. Routine radiographs were obtained immediately after surgery and before scintigraphy. Four weeks after surgery, each rabbit was injected with 37 MBq 99mTc-PEG liposomes, 99mTc-HYNIC-IgG, and 99mTc-MDP on 3 consecutive days and imaged up to 4 (MDP) or 22 (liposomes and IgG) h after injection. On day 4, rabbits received either 18 MBq 111In-granulocytes or 67Ga-citrate and were imaged up to 44 h after injection. Uptake in the infected femur was determined by drawing regions of interest. Ratios of infected-to-sham-operated femur were calculated. After the last image, the rabbits were killed, and the left and right femur were scored for microbiologic and histopathologic evidence of osteomyelitis. RESULTS: 99mTc-PEG liposomes and 99mTc-HYNIC-IgG correctly identified all 6 rabbits with osteomyelitis. 11In-granulocytes and 67Ga-citrate gave equivocal results in 1 infected rabbit. 99mTc-MDP missed 1 case of osteomyelitis. The uptake in the affected region did not differ significantly between the agents, although 99mTc-MDP tended to have higher values (MDP, 4.75 +/- 1.23 percentage injected dose per gram [%ID/g]; 67Ga, 2.05 +/- 0.54 %ID/g; granulocytes, 1.56 +/- 0.83 %ID/g; liposomes, 1.75 +/- 0.76 %ID/g, and IgG, 1.96 +/- 0.27 %ID/g). The ratios of infected-to-normal femur were also not significantly different for the respective radiopharmaceuticals. Radiography visualized only severe osteomyelitis. CONCLUSION: In this rabbit model, 99mTc-PEG liposomes and 99mTc-HYNIC-IgG performed at least as well as 111In-granulocytes and 67Ga-citrate in the localization of chronic osteomyelitis. The ease of preparation, the better image quality, and the lower radiation dose suggest that 99mTc-PEG liposomes and 99mTc-HYNIC-IgG might be suitable alternatives for 67Ga-citrate and 111In-granulocytes in the scintigraphic evaluation of osteomyelitis.

Nomenclature of Raynaud's phenomenon: a capillary microscopic and hemorheologic study.

Hemorheologic parameters and nailfold capillaries were investigated in 72 patients with ischemic hand phenomena and 25 normal subjects. Red blood cell (RBC) aggregation and plasma viscosity were normal in all groups except in patients with secondary Raynaud's phenomenon; in this group, these parameters were significantly increased (p less than 0.001). The capillary density in the nailfold was significantly lower in patients with secondary Raynaud's phenomenon or acrocyanosis (p less than 0.001). The capillary diameters were slightly increased in patients with primary Raynaud's phenomenon, moderately increased in patients with acrocyanosis (more than twofold), and markedly increased in patients with secondary Raynaud's phenomenon (fourfold to fivefold). Nailfold velocity, before and after cold provocation, was decreased in all patients (p less than 0.001). The findings in this study support the conclusion that patients with primary and secondary Raynaud's phenomenon, acrocyanosis, or asphyxia digitorum can be distinguished by combining hemorheologic observations with morphologic and functional microscopic investigations of the nailfold capillaries. The nomenclature currently employed in our clinic can be used to define these different phenomena.

Treatment of benign and low-grade malignant intramedullary chondroid tumours with curettage and cryosurgery.

AIMS: To shed light on the controversy surrounding the methods of evaluating, staging and final treatment of intramedullary chondroid lesions. Controversy particularly exists for enchondroma and low-grade chondrosarcoma located in the extremities, because their accurate distinction is hampered by their radiographical and histological similarity. METHODS: Since 1991 we have treated 22 patients (mean age: 39.6 years) with 26 lesions (three chondroblastomas, 14 enchondromas and nine grade 1 chondrosarcomas) with curettage, cryosurgery and bone grafting. RESULTS: After a mean follow-up of 26 months no recurrences were observed. Complications consisted of two post-operative fractures, one wound infection and one intraoperative venous gas embolism. All bone grafts incorporated, resulting in full weight-bearing capacity and excellent functional results. CONCLUSION: The usefulness of a combination of curettage and cryosurgery as adjuvant therapy is considered to be equal to marginal resection according to orthopaedic oncological principles. The pre-operative assessment of these lesions and cryosurgical technique is described in detail.

Synovial sarcoma: oncological and functional results.

AIM: To evaluate the oncological and functional result of the treatment of patients with a synovial sarcoma. This paper gives a retrospective review of 20 patients (15 male and five female) treated for synovial sarcoma at the Nijmegen University Hospital, The Netherlands. METHODS: The median age of the patients was 30 years (range: 14-71, mean 37 years). RESULTS: The tumour locations were: lower extremity in 12 patients; upper extremity in three; pelvic and groin region in four; and the retroperitoneal space in one. Surgical stages according to Enneking (Clin Orthop 1986; 204: 9-24) were IIA in five cases; IIB in seven; and IIIB in eight. The surgical margin was intralesional in three cases; marginal in three; wide in six; and radical in six. In one case the surgical margin could not be assessed and one patient was not operated. One patient developed a recurrent tumour and one developed nodal metastases. Eight patients who did not have metastases at the time of diagnosis developed metastase during follow-up. Fourteen patients died of metastatic disease; one patient died of diabetes; one is alive with disease; and four presently do not have evidence of disease. CONCLUSIONS: The outcome was poor, especially due to the occurrence of pulmonary metastases. The functional result according to the MSTS was 100% in four patients; in one the result was 93% because of the scar and venous insufficiency.

Single- versus dual-energy quantitative computed tomography for spinal densitometry in patients with rheumatoid arthritis.

Lumbar bone mineral density was measured by both single- and dual-energy quantitative computed tomography in 109 patients with rheumatoid arthritis. The results were corrected for the age-related increase in vertebral fat content by converting them to percentages of expected densities, using sex and energy-level specific regression equations obtained in a normal reference population. The percentages of expected density are approximately 10% lower in the single- than in the dual-energy mode, both in the patients with and without prednisone therapy. This difference is statistically highly significant, and is positively correlated with the duration of the disease and with the degree of radiological joint destruction. The data suggest that the vertebral fat content may be increased in patients with rheumatoid arthritis, as a consequence of disease-dependent mechanisms.

Grading medial collateral ligament injury: comparison of MR imaging and instrumented valgus-varus laxity test-device. A prospective double-blind patient study.

PURPOSE: The role of MR imaging in grading medial collateral ligament (MCL) injury of the knee in comparison to other grading methods (clinical findings and instrumental measurement) is hardly documented in the literature. The purpose of this study is to compare the results of MR imaging in grading acute MCL injuries to the results of a clinical grading by an instrumented valgus-varus laxity tester (VVLT). MATERIALS AND METHODS: Twenty-one patients clinically suspected of acute MCL injury were tested by VVLT, a well documented and instrumented test-device. All patients subsequently underwent MR imaging of the knee. MCL injury was graded independently by VVLT and MR imaging using a classification method with reference to Petermann. RESULTS: Nineteen patients had corresponding grading results by VVLT and MR imaging (kappa, 0.83; S.E., 0.10); 14 patients had a Grade I, four a Grade II and two patients had a Grade III MR imaged MCL injury. Associated lesions were also depicted on MR imaging (bone contusion (n = 3), ACL disruption (n = 2) and medial meniscal rupture (n = 1)). CONCLUSIONS: This study shows a very high degree of agreement between the results in grading acute MCL injuries with MR imaging and an instrumented valgus-varus laxity tester (VVLT). MR imaging depicted important, clinically undetected, additional lesions which can determine the treatment of MCL injury.

The use of subtraction arthrography in total hip arthroplasties.

The results of plain film radiography and subtraction arthrography in 24 patients prior to revision surgery for a loosened total hip arthroplasty (T.H.A.) were compared with operative findings. Loosening of both the acetabular and the femoral components was evaluated. In plain film radiography the overall accuracy for evidence of loosening in 22 acetabular and 23 femoral components was 58%. The overall accuracy with arthrography was 93%. Three results were false-negatives; arthrography showing no evidence of loosening, while the arthroplasty was found to be loose on surgical evaluation. The results of this study are compared with findings reported in the literature. Arthrography was performed by a lateral puncture technique. There were no complications. The use of the puncture technique has not been described previously. The extent of contrast leakage into the interfaces is described and discussed.

MR imaging of 22 Charnley-Müller total hip prostheses.

To find out whether MR imaging is contraindicated in patients with metallic implants or can be a routine diagnostic procedure, MR investigations in 18 patients with 22 Charnley-Müller total hip prostheses were performed on a 0.5 T Gyroscan S 5, Philips. No adverse reactions during or post MR investigation were encountered. The imaging of the soft tissue was superior to CT and showed less distortion. The diagnosis of loosening, by detection of demarcation lines at the interfaces was at its best in the distal part of the femoral stem prosthesis and was poor in the acetabular component and in the upper part of the stem prosthesis due to artifacts.

Aneurysmal bone cysts treated by curettage, cryotherapy and bone grafting.

We treated 26 patients with 27 aneurysmal bone cysts by curettage and cryotherapy and evaluated local tumour control, complications and functional outcome. The mean follow-up time was 47 months (19 to 154). There was local recurrence in one patient. Two patients developed deep wound infections and one had a postoperative fracture. We compared our results with previous reports in which several different methods of treatment had been used and concluded that curettage with adjuvant cryotherapy had similar results to those of marginal resection, and that no major bony reconstruction was required. We recommend the use of cryotherapy as an adjuvant to the surgical treatment of aneurysmal bone cysts. It provides local tumour control. Combination with bone grafting achieved consolidation of the lesion in all our patients.

Sacroiliitis and acne conglobata.

A 24-year old man developed a bilateral sacroiliitis in association with acne conglobata. The acne conglobata was followed after the use of prednisone during 9 months. The prednisone was taken for a severe form of sarcoidosis. This may be a new instance of a relatively uncommon and poorly understood relationship between a particular form of acne and associated arthritis.

Swelling of hand and forearm caused by Mycobacterium bovis.

A 75-year-old native Dutch farmer presented with a painless swelling of his right hand extending into his forearm, accompanied by general malaise and low grade fever. His medical history revealed coxitis tuberculosa in 1954 and injury of the same hand in 1978. His present swelling appeared to be caused by Mycobacterium bovis, probably due to endogenous reactivation. Whether the mycobacteria reached the hand hematogenously or were directly inoculated at the time of injury of his hand in 1978 remains unclear.

Was isolation of Veillonella from spinal osteomyelitis possible due to poor tissue perfusion?

From a needle biopsy of the body of the 12th thoracic vertebra in a 74-year-old man with spinal osteomyelitis a Veillonella parvula was isolated. The significance of this bacterium as lactic acid indicator has been considered and discussed. Since this strictly anaerobic bacterium mainly uses lactic acid for energy generation, lactic acid must continuously and sufficiently have been produced in or near to the vertebrate body to permit outgrowth of this bacterium. Since neither microbial infections nor tumours could be demonstrated, we finally hypothesised that in this patient poor tissue perfusion has been the primary cause of lactic acid production.

Eosinophilic granuloma of bone: results of treatment with curettage, cryosurgery, and bone grafting.

Intralesional instillation of steroids is currently the first choice of treatment for eosinophilic granuloma of bone. However, some lesions fail to respond or are unsuitable for injection therapy due location, pending pathologic fracture, and soft tissue intrusion. The authors treated six patients with these lesion characteristics using curettage, cryosurgery, and bone grafting. After a mean follow-up of 34.3 months, all lesions healed completely, although one femoral fracture occurred 8 months after the operation. In selected cases of eosinophilic granuloma of bone, a primary surgical treatment seems feasible. The use of cryosurgery as adjuvant treatment extends the surgical margin and is of value in averting local recurrence.

[Chronic recurrent multifocal osteomyelitis]. / Chronische recidiverende multifocale osteomyelitis.

In a 9-year-old girl chronic recurrent multifocal osteomyelitis was diagnosed, a rare disease mostly affecting children of which the aetiology remains obscure. It is usually diagnosed around the age of II years. The patient suffers from exacerbations and remissions for an average of 2.9 years. Awareness and recognition of the disease is of importance to avoid unnecessary diagnostic procedures and treatment. The prognosis is good.

Aneurysmal bone cyst. Long-term results and functional evaluation.

A study was performed on 13 patients with 16 primary aneurysmal bone cysts (ABC) to assess long-term results and functional outcome. The average age at diagnosis was 19.8 years. The anatomical sites were femur (4), tibia (5), humerus (2), fibula (2) and other (3). Four ABC's required no treatment. One patient underwent radiotherapy for an ABC of the spine. Eleven ABC's were treated surgically, mostly by intracapsular resection and bone grafting. Three patients had a total of 6 recurrences, which needed further treatment. At the time of follow-up (average period: 102 months), the average functional evaluation rate of 13 patients was 96%. These functional results were graded as excellent in 9 patients and good in 4.