Dysphagia caused by diffuse idiopathic skeletal hyperostosis

Summary Diffuse idiopathic skeletal hyperostosis (DISH) of the spine is often a hazardous radiological finding. Dysphagia, caused by ossification of the anterior longitudinal ligament, may be one of the most important clinical symptoms disturbing the patient. Diagnosis is done by esophagogram, and cervical decompression restores esophageal function. The case of a successfully operated patient is reported and a short overview of the literature is given.     

Tibial hyperostosis: A diagnostic approach

Tibial hyperostosis may be encountered in musculoskeletal imaging, incidentally or during the investigation of a leg pain. Hyperostosis involves the exuberant production of osseous tissue and results in cortical, periosteal and/or endosteal thickening of the bone. As a long bone with thick cortices, the tibia has a significant probability of being affected by ubiquitous bone diseases. As a tubular long bone, the tibia is likely to be involved in extensive infectious conditions such as osteomyelitis. As a bone of the lower limb, the tibia undergoes high stresses and may be affected by decrease in bone strength or repetitive submaximal stress. The tibia is also particularly involved in some bone sclerosing dysplasias and Paget's disease. In this work, we aim at highlighting the main conditions leading to tibial hyperostosis and try to provide key elements to narrow down the several diagnostic possibilities. Osteoid osteomas, fatigue or insufficiency fractures, infectious conditions, vascular lesions, sclerosing bone dysplasias and Paget's disease represent the main challenging diagnoses to discuss.     

Surgical Outcomes of Sphenoid Wing Meningioma with Periorbital Invasion

ObjectiveThe aim of this study was to evaluate the clinical outcome of sphenoid wing meningioma with periorbital invasion (PI) after operation. MethodsSixty one patients with sphenoid wing meningioma were enrolled in this study. Their clinical conditions were monitored after the operation and followed up more than 5 years at the outpatient clinic of a single institution. Clinical and radiologic information of the patients were all recorded including the following parameters : presence of PI, presence of peri-tumor structure invasion, pathologic grade, extents of resection, presence of hyperostosis, exophthalmos index (EI), and surgical complications. We compared the above clinical parameters of the patients with sphenoid wing meningioma in the presence or absence of PI (non-PI), then linked the analyzed data with the clinical outcome of the patients. ResultsOf 61 cases, there were 14 PI and 47 non-PI patients. PI group showed a significantly higher score of EI (1.37±0.24 vs. 1.00±0.01, p<0.001), more frequent presence of hyperostosis (85.7% vs. 14.3%, p<0.001), and lower rate of gross total resection (GTR) (35.7% vs. 68.1%, p=0.032). The lower score of pre-operative EI, the absence of both PI and hyperostosis, smaller tumor size, and the performance of GTR were associated with lower recurrence rates in the univariate analysis. However, in the multivariate analysis, the performance of GTR was the only significant factor to determine the recurrence rate (p=0.043). The incidences of surgical complications were not statistically different between the subtotal resection (STR) and GTR groups, but it was strongly associated tumor size (p=0.017). ConclusionThe GTR group showed lower recurrence rate than the STR group without differences in the surgical complications. Therefore, the GTR is strongly recommended to treat sphenoid wing meningioma with PI for the better clinical outcome.     

Morbidity and mortality of hospitalized patients with diffuse idiopathic skeletal hyperostosis

Diffuse idiopathic skeletal hyperostosis (DISH) has been associated with various metabolic disorders considered to be cardiovascular risk factors such as obesity, diabetes mellitus, hyperinsulinemia, and hyperlipidemia. To evaluate morbidity and mortality of hospitalized patients with DISH admitted to the department of medicine. One hundred patients from a cohort of 1020 consecutive patients, aged 45 years and more, admitted to the department of medicine were diagnosed as suffering from DISH. Another group of 100 patients, age- and gender matched, admitted without DISH, served as controls. Clinical and demographic characteristics, diagnoses on admission, previous chronic diseases, chronic medical therapy, laboratory tests, and the rates of in-hospital mortality and readmissions within 1 month of discharge were collected from the hospital database, for the two groups. Uncompensated or paroxysmal atrial fibrillation was more often encountered on admission in patients with DISH (p=0.038). Patients with DISH were more likely to suffer from elevated body mass index, arterial hypertension, diabetes mellitus, and previous cerebral vascular accidents, although the differences did not reach statistical significance. However, significantly more patients had an electrocardiographic evidence of left ventricular hypertrophy (p=0.03). The mortality rate was similar between the two groups. The lack of significant associations for cardiovascular risk factors such as diabetes mellitus, hypertension, and high BMI should be interpreted cautiously considering the characteristics of the control group. Identification of comorbid conditions and proper therapeutic interventions may prove useful in reducing the morbidity and mortality associated with this disorder.     

Van Buchem disease: lifetime evolution of radioclinical features

Objective The purpose of this study was to evaluate the lifetime evolution of the radioclinical features in a large family with van Buchem disease.Design and patients The study population included 13 patients, ranging between 6 and 69 years. The evolution of the clinical features has been assessed by retrospective analysis of the clinical records of the patients. The age-related evolution of the cortical hyperostosis and defective modeling at the tubular bones was evaluated by morphometric analysis of hand films in 9 patients, compared with 9 control individuals. Progression of sclerosis of the craniofacial bones was evaluated by analysis of the skull radiographs of eleven van Buchem patients, taken at different age.Results and conclusions Radioclinical features, including sclerosis of the cranial and tubular bones and cranial nerve deficit, become more prominent in older patients. Defective modeling of tubular bones, cortical thickness and medullary width progress with age. Radioclinical abnormalities of van Buchem patients become more prominent in older patients, which suggests that the van Buchem gene is very actively involved in bone metabolism throughout life. Morphometric analysis of the plain films supports the hypothesis that the physiological function of the van Buchem gene is to inhibit bone formation and possibly to regulate bone remodeling.     

Acquired hyperostosis syndrome: Spectrum of manifestations at the sternocostoclavicular region. Radiologic evaluation of 34 cases

Summary Thirty-four patients with chest wall hyperostosis, a condition which has been designated by various terms in the literature were evaluated radiologically. We prefer the name acquired hyperostosis syndrome (AHS), which we categorize into the complete, incomplete and possible form. In complete AHS, sternocostoclavicular hyperostosis is associated with axial and/or peripheral (endosteal, periosteal, enthesopathic, metaplastic) hyperostosis and with psoriasiform or acneform dermatosis. In addition, these three manifestations are accompanied by erosive or nonerosive peripheral and/or axial arthritis to a variable degree. Sometimes, concomitant findings which are consistent with ankylosing spondylitis are also to be found in the axial skeleton. AHS is manifested at 11 different sites on the anterior chest wall. Ossification forms of the costal cartilage, inflammatory enthesopathies (three different insertions) and focal hyperostoses as well as processes of remodelling of the ribs, clavicles and sternum which are described in detail have particular diagnostic significance. AHS can start simultaneously at one, two or several sites on the anterior chest wall. Conventional tomography (possibly supplemented by CT) is necessary for early diagnosis and for analysis of the various findings on the anterior chest wall.     

Is SAPHO syndrome a target for antibiotic therapy?

The etiology of the synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome remains unclear. Infectious factors are proposed to be relevant in the etiopathogenesis of the disease. To our knowledge, this is the first reported case of a proposed relationship between Staphylococcus aureus cultured from plantar pustule and SAPHO syndrome, which was successfully treated with co-trimoxazole (CTM) (sulfamethoxazole/trimetoprim). CTM might be the drug of choice for therapy for SAPHO syndrome because of combined antibiotic and immunomodulatory properties. Hypersensitivity testing of the medication in vitro was performed to identify, in the preclinical stage, the hypersensitivity reaction to CTM, which may have been severe.     

Schnitzler’s syndrome

We report on a case of Schnitzler’s syndrome, focussing on pattern of bone involvement and its differential diagnosis.     

Melorheostosis

Summary Melorheostosis is a rare, benign disease, usually affecting bones of the extremities by a characteristic hyperostosis causing typical findings on X-ray examination. We report an 18-year-old male with melorheostosis of the right hand. A review of the literature is given.