Magnetic resonance imaging in teenagers and young adults with limited haemophilic arthropathy: baseline results from a prospective study.

The clinical relevance of subtle changes on magnetic resonance imaging (MRI) for evaluating haemophilia treatment is unknown. To determine the relationship of findings on MRI with joint function and bleeding in joints with apparently very mild arthropathy, a prospective study was performed. Knees and ankles of 26 patients, 13-26 years, were scanned. Two blinded radiologists scored the MRI (IPSG consensus score) and the radiography [Pettersson score (PS)]. Clinical function (HJHS) was scored by one physiotherapist. Life-time number of bleeds was collected from patient files. Of 104 joints scanned, three were excluded because of previous arthrodesis or trauma. Remaining 101 MRI scores correlated weakly with clinical function (r = 0.27, P = 0.01) and less with lifetime number of bleeds (r = 0.16, P = 0.14). MRI scores were 0 in 58 joints, including 27 with major bleeds. In three joints of patients playing intensive sports MRI showed minor changes (MRI score = 1) in the absence of bleeds. Agreement was reasonable between PS and MRI score (r = 0.41, P < 0.01). In 30% of joints, MRI detected abnormalities in soft-tissue and cartilage, while PS was 0 points. No evidence of occult haemorrhages was found. Instead, we found no abnormalities on MRI in 43 joints with a history of repeated joint bleeding. Haemosiderin seemed associated with the time between assessment and last bleed; joints that had suffered a bleed long before MRI had hardly haemosiderin, while those with a recent bleed showed haemosiderin, suggesting joint damage may be reversible. Abnormalities detected by MRI, but not by PS were minor and their clinical implications are not yet clear.

MR mammography is useful in the preoperative locoregional staging of breast carcinomas with extensive intraductal component.

PURPOSE: To determine the role of magnetic resonance (MR) mammography in detection and assessment of extent of tumors with extensive intraductal component (EIC+). MATERIAL AND METHODS: In a prospective study, 233 consecutive women with a suspicious lesion underwent preoperative MR mammography and 209 invasive ductal carcinomas were detected. We studied the prediction of intraductal spread on mammography (MX), ultrasound (US) and MR. We compared the size of the total lesion on MX, US and MR and correlated it with histopathology. Enhancement patterns on MR were described. RESULTS: Of 209 invasive ductal carcinomas, 50 were EIC+ (24%). MX predicted intraductal spread in EIC+ carcinomas in 48.5%, US in 34.2% and MR in 68%. Compared to MX and US, MR was best in assessment of total tumor size. On MR, ductal spread in EIC+ tumors presented as ductal or linear enhancement, long spicules, a regional enhancing area or nodules adjacent to a mass. CONCLUSION: MR had the highest sensitivity to predict intraductal spread and was superior in assessing total tumor size.

Vascular disease of the spleen.

A wide range of vascular disorders can affect the spleen. Although clinical presentation is often nonspecific, early diagnosis and treatment are mandatory in most conditions. Noninvasive imaging techniques are well suited to meet these objectives. Familiarity with normal macroscopic and microscopic vascular anatomy is a prerequisite to understand the pathophysiology of vascular disorders of the spleen. This article deals with diseases of the splenic vasculature (aneurysms, arteriovenous fistula, splenic vein thrombosis, collateral circulation in portal hypertension) as well as vascular disorders affecting the splenic parenchyma (splenic infarct, Gamna-Gandy bodies). Primary vascular tumors and tumor-like conditions of the spleen will be discussed very briefly.

Early diagnosis of cerebral fat embolism syndrome by diffusion-weighted MRI (starfield pattern).

BACKGROUND: Cerebral fat embolism syndrome is a rare, but potentially lethal, complication of long bone fractures. Neurological symptoms are variable, and the clinical diagnosis is difficult. The purpose of this case study is to demonstrate the value of diffusion-weighted MRI of the brain for early diagnosis of fat embolism syndrome. Case Description- A non-head-injured 18-year-old woman suffered acute mental status changes 21 hours after an uncomplicated fracture of the left tibia. MRI of the brain was performed 48 hours after injury. T2-weighted images showed multiple nonconfluent areas of high signal intensity, which, on the diffusion-weighted scans, were revealed as bright spots on a dark background ("starfield" pattern). We suggest that this indicates areas of restricted diffusion that are due to cytotoxic edema, resulting from multiple microemboli. CONCLUSIONS: High-intensity lesions in the brain on diffusion-weighted images may serve as an early-appearing and more sensitive indicator of the diagnosis of fat embolism in the clinical context of long bone injury without head trauma.

Acute superior vena cava obstruction after rupture of a bronchial artery aneurysm.

A patient was referred to our hospital with a superior vena cava syndrome after the rupture of a bronchial artery aneurysm. To our knowledge, this complication has not been reported before. An urgent thoracotomy was performed after embolization had failed.

Amyloidoma of the skull base.

We report a case of a primary amyloidoma of the skull base. Plain radiography and CT showed a lytic, highly destructive lesion with multiple scattered calcifications within. MR imaging revealed that the tumor was isoto hypointense to muscle on T1-weighted images and extremely hypointense on T2-weighted images. In contrast to two previous reports, marked enhancement after the administration of contrast material was absent. Bone amyloidomas are very rare and are frequently misinterpreted as chondrosarcomas.

MR of the brain in Sjögren-Larsson syndrome.

Cerebral MR was performed in three patients with Sjögren-Larsson syndrome. In each case, a 1.5-T system was used, and the patient was under general anesthesia. The MR findings included confluent hyperintense white matter lesions in the periventricular and deep white matter of the centrum semiovale, with sparing of the subcortical U fibers. The topography of the white matter abnormalities correlated well with the clinical signs and symptoms.

Cerebral complications of murine monoclonal CD3 antibody (OKT3): CT and MR findings.

Treatment of acute renal allograft rejection with mouse monoclonal antibody (OKT3) is associated with systemic and neurologic side effects. We describe cerebral abnormalities in a 13-year-old boy with steroid-resistant renal allograft rejection. After treatment with OKT3, an acute neurologic syndrome developed, including seizures, lethargy, and decreased mental function. CT and MR imaging revealed confluent cerebral lesions at the corticomedullary junction. Contrast-enhanced MR images showed patchy enhancement, indicating blood-brain barrier dysfunction. The diagnosis of OKT3-induced encephalopathy with cerebral edema and capillary leak syndrome was made. Although CT and MR findings are nonspecific, neuroradiologists should be aware of this condition in transplant patients treated with OKT3.

Effects of a continuing education program on nurses' pain assessment practices.

Surgical nurses from five Dutch general hospitals participated in a continuing education program on pain assessment and management. A pretest-posttest controlled intervention study revealed that the program led to an increase in the quality of activities relevant to taking pain histories. Although this increase in quality was most apparent 1 month after the program, it was still observable 6 months after the program. There were, however, no effects on the number of activities relevant to taking pain histories, or on the number of nurses who used direct questioning as a method to determine pain. There may be several explanations for these results, including nurses' limited openness to new approaches, a lack of support from physicians and nurse superiors, and that program items were not translated into ward policy.

Contrast-enhanced MR imaging of breast lesions and effect on treatment.

PURPOSE: To assess the value of local staging with preoperative magnetic resonance imaging (MRI) in patients with suspect breast lesions and the effect on therapeutic approach. MATERIALS AND METHODS: Two hundred and four consecutive women with suspect breast lesions on clinical examination (CE) and/or mammography (MX) and/or ultrasound (US) underwent preoperative contrast-enhanced MRI. Detection of multifocal, multicentric and bilateral breast cancer by all three imaging modalities was evaluated. Results of preoperative breast MRI were discussed with the treating surgeons. The type of therapeutic change after preoperative MRI was marked on a questionnaire (none, additional fine needle aspiration, core biopsy, open biopsy, wider excision, mastectomy) and considered 'necessary' or 'unnecessary' using final histopathological results as gold standard. RESULTS: In 170 patients, breast cancer was diagnosed. MRI detected 96% of multifocal disease and 95% of multicentric disease, whereas MX depicted 37 and 18%, and US 41 and 9% of them, respectively. All bilateral breast cancers were seen on MRI; both MX and US detected 56%. Findings of more extensive disease and unsuspected multiple breast cancer foci identified on MRI only, changed the therapeutic approach correctly in 30.6% of breast cancer patients. Nine unnecessary wider excisions and three unnecessary FNA/core biopsies were performed because MRI overestimated the number or size of malignant lesions. CONCLUSION: Preoperative breast MRI is an important adjunct to conventional imaging in the loco-regional staging of breast cancer and a useful tool in treatment planning.

Computed tomography of the brain in unipolar depression.

Several authors have reported enlarged lateral brain ventricles in major depressive patients as compared to healthy controls. Also, the enlargement of brain lateral ventricles has been related to delusions, psychomotor retardation and some biochemical data such as cortisol secretion and L-tryptophan serum levels. The present study was undertaken to investigate if melancholic depressives are characterised by a higher degree of brain atrophy than normal controls and minor depressives, the origin of any brain atrophy, and whether measures of brain atrophy are related to cortisol secretion and L-tryptophan serum levels. We investigated 10 healthy controls and 35 depressive patients categorised according to DSM-III. In contrast to previous studies, we determined a combination of indices which makes it possible to differentiate between central and cortical diffuse atrophy. We found no evidence for the existence of abnormal atrophy of the brain in melancholics; nor did we find any correlation between CT scan measurements and cortisol or tryptophan.

Computed tomographic diagnosis of dilated ovarian veins in a case of "ovarian vein syndrome".

Ovarian vein dilatation in the so called "ovarian vein syndrome" is classically demonstrated by selective phlebography. The diagnostic value of computed tomography as a non invasive alternative for selective phlebography in a case of ovarian vein syndrome is reported. A definition of the ovarian vein syndrome and a personal view on the pathogenesis of this syndrome are given.

Unusual cystic liver lesions: a pictorial essay.

Cystic liver lesions are being increasingly detected by ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI). These lesions produce a wide spectrum of radiological features. Unfortunately their appearance is not always specific and they may resemble and be mistaken for other lesions. Cystic lesions can be classified into congenital, inflammatory, neoplastic and miscellaneous groups. In this pictorial essay a number of unusual cystic lesions are illustrated with a brief analysis of morphological features of the individual lesion and a diagnostic approach for cystic liver lesions is suggested.

Tuberous sclerosis: CT and MRI characteristics.

Tuberous sclerosis is a disease characterized by the presence of hamartomatous nodules in various organs, including the brain. Thirteen CT scans and four MR investigations were performed in 13 patients. CT is superior to MR in demonstrating the presence of subependymal nodules, thereby confirming or establishing the diagnosis of tuberous sclerosis. MR is superior to CT in demonstrating the peripheral lesions, which are characterized by a long T2-relaxation time. Differentiation between cortical tubers and subcortical lesions is not yet accurate.

Congenital skeletal abnormalities: an introduction to the radiological semiology.

Despite the recent advances in the molecular diagnosis of congenital abnormalities, the initial identification and the decision to refer a patient for further molecular analysis and expensive genetic tests still relies frequently on clinical and radiological criteria. The radiological identification of syndromes, dwarfs and dysplasias is a difficult task, because there are so many findings to consider and so many syndromes to remember that the problem is overwhelming. There is a definite need for an easy and systematic analysis system, in order to try to categorize a skeletal dysplasia in a certain group. In this brief review, we suggest an approach to the evaluation of skeletal syndromes, based on the analysis of cardinal criteria, from which the most useful information is derived, and additional criteria, making further differentiation possible. Generally, cardinal information is derived from analysis of the long bones, hands, pelvis and the spine, whereas the analysis of other skeletal elements, like the skull, feet, and other flat bones is of additional value.

Molecular and radiological diagnosis of sclerosing bone dysplasias.

Bone mineral density (BMD) is a quantitative trait for which the heritability of the variance is estimated to be up to 80%, based on epidemiological and twin studies. Further illustration of the involvement of genetic factors in bone homeostasis, is the existence of an extended group of genetic conditions associated with an abnormal bone density. The group of conditions with increased bone density has long been poorly studied and understood at the molecular genetic level but recently, thanks to recent developments in molecular genetics and genomics, for some of them major breakthroughs have been made. These findings will make the molecular analysis of such patients an additional tool in diagnostics and in genetic counseling. However, the initial identification of affected patients is still largely dependent upon recognition of clinical and radiological stigmata of the disease. Therefore, in this overview of sclerosing bone dysplasias, the classical clinical and radiological signs of this group of disorders will be discussed along with the new molecular insights.

Hereditary multiple exostoses: from genetics to clinical syndrome and complications.

OBJECTIVE: To give an overview of genetic, clinical and radiological aspects in two families over four generations with known hereditary multiple exostoses (HME). METHODS AND MATERIAL: After linkage analysis in both families to localize the defective gene, mutation analysis was performed in these genes to identify the underlying mutation. In the 31 affected individuals, location, number and morphology and evolution of exostosis, evolution of remodeling defects at the metaphysis, and the extent of possible complications were evaluated on clinical and imaging (plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI)) data over a lifetime period. RESULTS AND CONCLUSIONS: Both families demonstrate the gene defect in the same EXT-2 gene locus on chromosome 11p. Exostoses are preferentially located in the lower extremity (hip, knee and lower leg), humerus, and forearm. Any other bone may be involved, except for the calvaria of the skull and the mandible. Exostoses are rather sessile than pedunculated. Exostosis is rarely present at birth but develops gradually and may persist to grow slowly after closure of the growth plates. Preferential expression of the remodeling defect was seen in the hip, distal femur (trumpet-shaped metaphysis) and forearm (shortening of the ulna with secondary bowing of the radius and development of a pseudo-Madelung deformity). These radiological manifestations start at the age of 4-5 years and become more obvious as the enchondral bone formation progresses with age. Reported complications in these families consist of local entrapment phenomenons (vessel, tendon, nerve), frictional bursitis, and sarcomatous transformation. MRI was able to suggest these complications and is the imaging technique of choice in the evaluation of symptomatic exostoses.

CT diagnosis and differential diagnosis of otodystrophic lesions of the temporal bone.

The purpose of this study was to assess the diagnostic and differential value of high-resolution computed tomography in the evaluation of temporal-bone dystrophies. The study group included 55 patients with osseous abnormalities of the temporal bone in general, and the labyrinthine capsule in particular. In 27 patients the CT scan revealed evidence of otodystrophic lesions. The CT findings in patients with otosclerosis (21 patients), osteogenesis imperfecta (two patients), fibrous dysplasia (one patient), Paget's disease (one patient) and osteopetrosis (two patients) are described. The CT scans of 17 patients revealed secondary osseous lesions due to metastasis (five patients), post-inflammatory changes (10 patients) or labyrinthitis ossificans (two patients). Normal variants and congenital mineralization defects were diagnosed in nine patients, Down's syndrome in two. Our results indicate the importance of high-resolution computed tomography as the primary imaging modality in evaluating osseous lesions of the temporal bone and labyrinth.

Reject analysis: a pilot programme for image quality management.

The radiographic film wastage and the different parameters affecting this wastage were analysed for a 9-week period at a 600-bed University Hospital. An overall reject rate of 7.6% was found. The different reasons for rejection were evaluated, while retake rate, relation between working experience of the personnel, amount of rejected films and total film wastage in surface (m2), were registered and analysed.

Epidural and subdural contrast in myelography and CT myelography.

A retrospective study of 624 lumbar myelographic and CT myelographic procedures was undertaken to evaluate the frequency, radiographic and clinical relevance of contrast medium outside the dural sac. Our findings indicate that epidural contrast is frequently identified on CT scans with highly sensitive window settings (in 35.4% of all CT myelographic studies). Standard myelographic films are less sensitive for the detection of epidural contrast (9.6%). Subdural contrast is far less often encountered (in 2.4% of CT myelographic studies and in 3.2% of standard myelographic films). We believe leakage of contrast to occur through the puncture site into the epidural and subdural space.