Fasciitis ossificans: imaging features, histology, and differential diagnosis.

AIM: To describe the imaging features of fasciitis ossificans and its histopathological features. MATERIALS AND METHODS: Using a word search of existing pathology reports at the Mayo Clinic, six cases of fasciitis ossificans were identified. The clinical history, histology, and available imaging of the affected area were reviewed. RESULTS: Imaging consisted of radiographs, mammograms, ultrasound images, bone scintigraphs, computed tomography (CT), and magnetic resonance imaging (MRI) images. All cases demonstrated a soft-tissue mass. The characteristic MRI appearance was a T2 hyperintense enhancing mass with surrounding soft-tissue oedema. Peripheral calcifications were seen on radiographs, CT, and/or ultrasound. Histological sections showed distinct zonation, with nodular fasciitis-like zones of myofibroblastic proliferation, which merged with osteoblasts that rim the ill-defined trabeculae of woven bone and became continuous with the mature lamellar bone surrounded by a thin layer of compressed fibrous tissue. CONCLUSION: Imaging features of fasciitis ossificans are that of an enhancing soft-tissue mass located within a fascial plane with prominent surrounding oedema and mature peripheral calcification. Imaging and histology are that of myositis ossificans but occurring within the fascia. It is important that radiologists are aware of the diagnosis of fasciitis ossificans and appreciate its similarity to myositis ossificans. This is particularly important in anatomical locations with fascias but no muscle. Given the radiographic and histological overlap between these entities, nomenclature that encompasses both could be considered in the future.

Does gadolinium improve magnetic resonance arthrography of the hip beyond fluid distension alone?

AIM: To determine if there is a difference in the detection of labral tears and cartilage lesions using the T2-weighted sequences of magnetic resonance arthrography (MRA) only, which simulate MRA with saline alone, compared with the full examination including T1-weighted sequences. MATERIALS AND METHODS: One hundred hip MRA examinations performed at 3 T were identified retrospectively. Each study was reviewed by a musculoskeletal radiologist using either the T2-weighted sequences only (without gadolinium-based contrast agent [GBCA] effect) or the entire examination, including T1-weighted sequences (with GBCA effect). Receiver operating characteristic (ROC) curves were calculated for both groups and compared within a non-inferiority framework, using - 0.10 as the limit of non-inferiority. RESULTS: For labral tears, the difference area under the curve (AUC) was -0.004 (95% confidence interval [CI]: -0.064-0.056, p=0.90). For acetabular cartilage lesions, the difference AUC was 0.011 (95% CI: -0.073-0.096, p=0.79). For femoral cartilage lesions, the difference AUC was -0.059 (95% CI: -0.206-0.087, p=0.43). CONCLUSION: T2-weighted sequences alone were not inferior in diagnostic accuracy compared with the full examination (including T1-weighted sequences with intra-articular GBCA) in detecting acetabular cartilage lesions and labral tears. Further study with prospective comparison of saline injectate to GBCA-containing injectate may help clarify the necessity of continued GBCA use in MRA of the hip.

[Ear aid in Myanmar]. / Ohrenhilfe in Myanmar.

As the example of a small team working in Myanmar since 2010 has demonstrated, it is possible to achieve sustainable success in medical education and training in foreign countries with relatively little effort, in this case in middle ear surgery. The main requirements are outstanding communication within the team as well as with the authorities, organizations, and colleagues on site. Equally important is mindful and respectful work in the hosting country and the consideration of cultural particularities. Essential contributing factors for long-term success are intercultural exchange, interpersonal relationships, and especially the motivation of colleagues on site, which together create a foundation of trust. This trust combined with regular exchange visits enables constant professional development according to the current needs.

Fluoroscopically guided transforaminal epidural steroid injections at a quaternary-care teaching institution: effect of trainee involvement and patient body mass index on fluoroscopy time and patient dose.

AIM: To investigate whether there are differences in fluoroscopy time and patient dose for fluoroscopically guided lumbar transforaminal epidural steroid injections (TFESIs) performed by staff radiologists versus with trainees and to evaluate the effect of patient body mass index (BMI) on fluoroscopy time and patient dose, including their interactions with other variables. MATERIALS AND METHODS: Single-level lumbar TFESIs (n=1844) between 1 January 2011 and 31 December 2013 were reviewed. Fluoroscopy time, reference point air kerma (Ka,r), and kerma area product (KAP) were recorded. BMI and trainee involvement were examined as predictors of fluoroscopy time, Ka,r, and KAP in models adjusted for age and gender in multivariable linear models. Stratified models of BMI groups by trainee presence were performed. RESULTS: Increased age was the only significant predictor of increased fluoroscopy time (p<0.0001). Ka,r and KAP were significantly higher in patients with a higher BMI (p<0.0001 and p=0.0009). When stratified by BMI, longer fluoroscopy time predicted increased Ka,r and KAP in all groups (p<0.0001). Trainee involvement was not a statistically significant predictor of fluoroscopy time or Ka,r in any BMI category. KAP was lower with trainees in the overweight group (p=0.0009) and higher in male patients for all BMI categories (p<0.02). CONCLUSION: Trainee involvement did not result in increased fluoroscopy time or patient dose. BMI did not affect fluoroscopy time; however, overweight and obese patients received significantly higher Ka,r and KAP. Male patients received a higher KAP in all BMI categories. Limiting fluoroscopy time and good collimation practices should be reinforced in these patients.

MRgFUS ablation of a recurrent tenosynovial giant cell tumor in the foot using ExAblate 2100 system in combination with patient immobilization device.

INTRODUCTION: Magnetic Resonance-guided Focused Ultrasound (MRgFUS) treatment for certain anatomy locations can be extremely challenging due to patient positioning and potential motion. This present study describes the treatment of a recurrent tenosynovial giant cell tumor of the plantar forefoot using the ExAblate 2100 system in combination with patient immobilization device. METHODS: Prior to the treatment, several patient immobilization devices were investigated. Vacuum cushions were selected and tested for safety and compatibility with the treatment task and the MR environment. RESULTS: During the treatment, one vacuum cushion immobilized the patient's right leg in knee flexion and allowed the bottom of the foot to be securely positioned on the treatment window. Another vacuum cushion supported the patient upper body extended outside the scanner bore. 19 sonications were successfully executed. The treatment was judged to be successful. No immediate complications were observed. CONCLUSIONS: MRgFUS treatment of a recurrent tenosynovial giant cell tumor of the right plantar forefoot was successful with the use of patient immobilization vacuum cushions. IMPLICATIONS FOR PRACTICE: The immobilization system could be utilized to aid future MRgFUS treatment of lesions in challenging anatomic locations. Various sizes of the vacuum cushions are available to potentially better accommodate other body parts and treatment configurations.

Familial association of intracranial aneurysms and multiple congenital anomalies.

The familial occurrence of intracranial aneurysms and the possible relationship with connective tissue disease are discussed. We studied a large family in which seven members presented with aneurysms. Another family member presented with a subarachnoidal hemorrhage. Two other family members each presented with Marfan's syndrome and an unclassified multiple congenital anomalies syndrome, respectively. The multiplicity of the aneurysms in four members is in excess of that found in sporadic or familial cases with intracranial aneurysms. We suggest a common cause, eg, a connective tissue disorder for both the intracranial aneurysms, the Marfan's syndrome, and the unclassified syndrome.

Red blood cell enzymes in the diagnosis of genetic disease.

Erythrocytes are uniform cells which contain only those proteins that are synthesized during the reticulocyte stage. The relationship of red cell enzymes to gene dosage and gene expression enables the use of red cell enzyme assays to determine the presence or absence of gene defects causing enzyme deficiencies leading to various metabolic diseases; in addition, the mode of inheritance of these defects can frequently be ascertained by analyzing red cell enzymes. However, indirect evidence favoring other enzyme deficiency states can sometimes be obtained from a study of red cell enzyme activities, because apparent enzyme deficiencies may result from the accumulation of inhibitory metabolites formed due to an enzyme deficiency in other tissues. The polymorphic expression of many red cell enzymes lends itself to biochemical analysis which can produce highly accurate and specific diagnostic information.

Simultaneous occurrence of xanthine oxidase and sulfite oxidase deficiency. A molybdenum dependent inborn error of metabolism?

In a 3-week old female child with clinical features including neurologic abnormalities and lens dislocation, xanthinuria co-existed with increased excretion of sulfur compounds (sulfite, S-sulfocysteine, taurine and thio-sulfate). Low xanthine oxidase and absent sulfite oxidase activities were found on liver biopsy. No abnormality was detected in either parent. Both the above enzymes are molybdenum-flavoproteins. Normal serum molybdenum concentration seemed to rule out dietary deficiency or impaired absorption. A defect in the incorporation of the metal into flavoproteins is postulated in this case.

Some kinetic properites of liver ornithine carbamoyl transferase (OCT) in a patient with OCT deficiency.

Some kinetic properties of liver OCT from a patient with OCT deficiency were studied. Contrary to controls, in which two pH optima were observed (pH 7.7 and pH 8.5), only the pH optimum of 8.5 could be demonstrated in our patient. From KM studies at pH 7.7 and pH 8.5, the most striking abnormalities in comparison with human controls were (a) a strongly increased KM (ornithine) at pH 7.7, but less pronounced at pH 8.5, (b) a higher VMAX at pH 8.5 compared with the VMAX at pH 7.7 and (c) the absence of substate inhibition at pH 8.5 to ornithine was elevated up to a concentration above approximately 1.5 mM.

Esterase D: evaluation of a potential derived gene marker for hereditary retinoblastoma.

The gene locus of esterase D is closely linked to that coding for retinoblastoma. When the occurrence of retinoblastoma is based on a chromosome deletion, red cell esterase D might be a potential tumor marker for diagnosing retinoblastoma. This diagnostic utility was tested by measuring total esterase D and differentiating esterase D to its different phenotypes in red cells of patients with bilateral retinoblastoma, unilateral retinoblastoma having a positive family history and unilateral sporadic retinoblastoma. These results are compared with the findings within a group of first degree relatives of these patients and of a reference group of apparently healthy controls. A poor sensitivity and a low positive predictive value were found. So, the conclusion might be drawn that screening of all retinoblastoma patients for esterase D in order to get insight in the weighting of risk of retinoblastoma is very much open to question and due to high cost efficiency ratio might not be recommended.

Permanent chemical phenylketonuria and a normal phenylalanine tolerance in two sisters with a normal mental development.

A positive ferric chloride reaction was found at routine examination of the urine of a 14 year old mentally normal girl, admitted for complaints of headache and other meningitis-like symptoms. It turned out that she excreted permanently increased amounts of phenylpyruvic, phenyllactic and o-hydroxyphenylacetic acids, but phenylacetic acid (free plus conjugated) was normal. Fasting serum phenylalanine was not increased nor was urinary phenylalanine. On loading with L-phenylalanine (100 mg/kg) a normal serum phenylalanine response followed, but urinary phenylpyruvic, phenyllactic and o-hydroxyphenylacetic acids increased further. Phenylacetic acid responded too, but remained in the normal range. In addition to the above-mentioned abnormalities the urine contained a still unidentified abnormal acid, which also increased after loading with phenylalanine. Her 12 year old healthy sister showed the same chemical abnormality. Two older brothers and the parents had normal excretions. The enzyme defect has not been identified. As a possibility the defective decarboxylation of phenylpyruvic acid is proposed.

Tyrosinemia and tyrosyluria in healthy prematures: time courses not vitamin C-dependent.

Tyrosyluria and for a part also tyrosinemia were studied in 60 healthy prematures of various birth weights and gestational ages. The first analyses were performed between the 6th and the 14th day after birth. A normal milk diet was given and the protein-intake was between 3 and 4 g/kg. After the first collection of urine half the patients received extra ascorbic acid, 100 mg/kg daily. Urinary analyses of tyrosine and p-hydroxyphenyl metabolites were performed once a week, until the excretion of p-hydroxyphenylpyruvic plus p-hydroxyphenyllactic acids was lower than 5 mmoles per gram creatinine. In 22 out of the 60 prematures (or 37%) a tyrosyluria of more than 5 mmoles/g creatinine and in 19 out of 44 (43%) patients analysed serum tyrosine was higher than 5 mg/100 ml at first analysis. No inverse correlation between tyrosyluria and tyrosinemia on the one hand and birth weight and gestational age on the other hand existed. But in children with a delayed intra-uterine development the incidence of tyrosyluria was higher as prematurity was more pronounced. Ascorbic acid had no effect on the rate of disappearance of tyrosyluria. It was concluded that the addition of extra vitamin C to the diet of prematures is not useful for the normalization of tyrosine metabolism.

Determination of remifentanil in human heparinised whole blood by tandem mass spectrometry with short-column separation.

For the use in pharmacokinetic studies, a fast and sensitive assay method was developed for the determination of remifentanil in human heparinised whole blood samples of 0.5 ml. The assay method is based on tandem mass spectrometry detection (LC-MS/MS). The limit of quantification is 0.1 ng/ml and linear up to 50 ng/ml. The precision, accuracy, recovery and applicability were found to be adequate for pharmacokinetic studies.

High temperature RF SQUIDs for biomedical applications.

We have been investigating the feasibility of radio-frequency RF, low-noise superconducting quantum interference device (SQUID) magnetometers and gradiometers operating in liquid nitrogen at 77 K. Using flux-focusing structures fabricated from epitaxial YBa2Cu3O7-chi films, we have attained a magnetic field resolution for a magnetometer of better than 200 fT Hz-1/2 at less than 1 Hz, i.e. over the low signal-frequency range important for biomedical diagnostics. At 77 K, this magnetometer recorded diagnostically useful heart signals, voluntary eye-blink signals, and also the first evoked response of a human brain. These and similar results were obtained in a magnetically shielded room. We were also able to record heart signals in the absence of any shielding when using a first-order gradiometer. An improvement in the magnetic field resolution of our magnetometers and gradiometers by, at least, another order of magnitude is possible and probable.

Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients.

BACKGROUND AND PURPOSE: Immunoglobulin G4-related disease is a systemic fibroinflammatory process of unknown etiology, characterized by tissue infiltration by immunoglobulin G4 plasma cells. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in immunoglobulin G4-related disease of the orbit. MATERIALS AND METHODS: This study included 27 patients with biopsy-proved immunoglobulin G4-related disease of the orbit and either a CT or MR imaging of the orbits. These CT or MR imaging examinations were evaluated for the following: extraocular muscle size, extraocular muscle tendon enlargement, lacrimal gland enlargement, infiltrative process in the orbital fat (increased attenuation on CT or abnormal signal on MR imaging), infraorbital nerve enlargement, mucosal thickening in the paranasal sinuses, and extension of orbital findings intracranially. RESULTS: Extraocular muscles were enlarged in 24 of 27 (89%) patients, 21 (88%) bilaterally. In 32 of 45 (71%) affected orbits, the lateral rectus was the most enlarged muscle. In 26 (96%) patients, the tendons of the extraocular muscles were spared. Nineteen (70%) patients had lacrimal gland enlargement. Twelve (44%) patients had an infiltrative process within the orbital fat. Infraorbital nerve enlargement was seen in 8 (30%) patients. Twenty-four (89%) patients had sinus disease. Cavernous sinus or Meckel cave extension was seen in 3 (11%) patients. CONCLUSIONS: In patients with extraocular muscle enlargement, particularly when the tendons are spared and the lateral rectus is the most enlarged, and even more so when other noted findings are present, immunoglobulin G4-related disease should be a leading differential consideration, even over more commonly known etiologies of extraocular muscle enlargement.