Frequency of inter-specialty consensus decisions and adherence to advice following discussion at a weekly neurovascular multidisciplinary meeting.

BACKGROUND/AIMS: Data are limited on the frequency of 'consensus decisions' between sub-specialists attending a neurovascular multidisciplinary meeting (MDM) regarding management of patients with extracranial carotid/vertebral stenoses and post-MDM 'adherence' to such advice. METHODS: This prospective audit/quality improvement project collated prospectively-recorded data from a weekly Neurovascular/Stroke Centre MDM documenting the proportion of extracranial carotid/vertebral stenosis patients in whom 'consensus management decisions' were reached by neurologists, vascular surgeons, stroke physicians-geriatricians and neuroradiologists. Adherence to MDM advice was analysed in asymptomatic carotid stenosis (ACS), symptomatic carotid stenosis (SCS), 'indeterminate symptomatic status stenosis' (ISS) and vertebral artery stenosis (VAS) patients, including intervals between index event to MDM + / - intervention. RESULTS: One hundred fifteen patients were discussed: 108 with carotid stenosis and 7 with VAS. Consensus regarding management was noted in 96.5% (111/115): 100% with ACS and VAS, 96.2% with SCS and 92.9% with ISS. Adherence to MDM management advice was 96.4% (107/111): 100% in ACS, ISS and VAS patients; 92% (46/50) in SCS patients. The median interval from index symptoms to revascularisation in 50-99% SCS patients was 12.5 days (IQR: 9-18.3 days; N = 26), with a median interval from MDM to revascularisation of 5.5 days (IQR: 1-7 days). Thirty patients underwent revascularisation. Two out of twenty-nine patients (6.9%) with either SCS or ISS had a peri-procedural ipsilateral ischaemic stroke, with no further strokes/deaths during 3-months follow-up. CONCLUSIONS: The high frequency of inter-specialty consensus regarding management and adherence to proposed treatment supports a collaborative/multidisciplinary model of care in patients with extracranial arterial stenoses. Service development should aim to shorten times between MDM discussion-intervention and optimise prevention of stroke/death.

Extracranial and Intracranial Vasculopathy With "Moyamoya Phenomenon" in Association With Alagille Syndrome.

Background: Alagille syndrome (AGS) is an autosomal-dominant, multisystem disorder caused by mutations in the JAG1 gene. Case Description: A 34-year-old man was referred to our service 10 years ago with focal seizures with impaired awareness and transient slurred speech. He had a 5-year history of intermittent left monocular low-flow retinopathy. He has a family history of AGS. General examination revealed mild hypertension, aortic regurgitation, and livedo reticularis. Neurological examination was normal. Investigations: He had mild hyperlipidaemia and persistently-positive lupus anticoagulant consistent with primary anti-phospholipid syndrome. Color Doppler ultrasound revealed low velocity flow in a narrowed extracranial left internal carotid artery (ICA). MR and CT angiography revealed a diffusely narrowed extracranial and intracranial left ICA. Formal cerebral angiography confirmed severe left ICA narrowing consistent with a left ICA "vasculopathy" and moyamoya phenomenon. Transthoracic echocardiogram revealed a bicuspid aortic valve and aortic incompetence. Molecular genetic analysis identified a missense mutation (A211P) in exon 4 of the JAG1 gene, consistent with AGS. Discussion: AGS should be considered in young adults with TIAs/stroke and unexplained extracranial or intracranial vascular abnormalities, and/or moyamoya phenomenon, even in the absence of other typical phenotypic features. Gene panels should include JAG1 gene testing in similar patients.

Imaging the Patient With Sacroiliac Pain.

Sacroiliac (SI) region pain is a common clinical presentation and is often due to pathology involving the SI joints, usually of inflammatory, infective, neoplastic, or post-traumatic etiology. The SI joints have a unique anatomic layout and composition and can be imaged with a variety of techniques including conventional radiographs, computed tomography, isotope bone scintigraphy, and magnetic resonance imaging. This article reviews a range of common SI joint conditions, illustrated by multimodality imaging findings. We also discuss strategies for choosing the optimal imaging modality, pearls, and pitfalls of imaging and discuss an algorithm for approaching the patient with suspected inflammatory back pain.

Magnetic resonance neurography of the pelvis and lumbosacral plexus.

Recent advances in magnetic resonance (MR) imaging have revolutionized peripheral nerve imaging and made high-resolution acquisitions a clinical reality. High-resolution dedicated MR neurography techniques can show pathologic changes within the peripheral nerves as well as elucidate the underlying disorder or cause. Neurogenic pain arising from the nerves of the pelvis and lumbosacral plexus poses a particular diagnostic challenge for the clinician and radiologist alike. This article reviews the advances in MR imaging that have allowed state-of-the-art high-resolution imaging to become a reality in clinical practice.

Multiphasic contrast-enhanced MRI: single-slice versus volumetric quantification of tumor enhancement for the assessment of renal clear-cell carcinoma fuhrman grade.

PURPOSE: To assess the association between clear-cell carcinoma pathology grade at nephrectomy and magnetic resonance imaging (MRI) tumor enhancement. MATERIALS AND METHODS: The Institutional Review Board approved this retrospective study and waived the informed consent requirement. In all, 32 patients underwent multiphase contrast-enhanced MRI prior to nephrectomy. MRI tumor enhancement was measured using two approaches: 1) the most enhancing portion of the tumor on a single slice and 2) volumetric analysis of enhancement in the entire tumor. Associations between pathological grade, tumor size, and enhancement were evaluated using the Kruskal-Wallis test and generalized logistic regression models. RESULTS: No significant association between pathology grade and enhancement was found when measurements were made on a single slice. When measured in the entire tumor, significant associations were found between higher pathology grades and lower mean, median, top 10%, top 25%, and top 50% tumor enhancement (P < 0.001-0.002). On multivariate analysis the association between grade and enhancement remained significant (P = 0.041-0.043), but tumor size did not make an additional contribution beyond tumor enhancement alone in differentiating between tumor grades. CONCLUSION: There is significant association between tumor grade and enhancement, but only when measured in the entire tumor and not on the most enhancing portion on a single slice.

Spectrum of central nervous system abnormalities in neurocutaneous melanocytosis.

AIM: Neurocutaneous melanocytosis is a rare neurocutaneous syndrome defined by the presence of large and/or multiple congenital cutaneous nevi and melanocytic deposits in the central nervous system. We sought to define the spectrum of central nervous system abnormalities in children with neurocutaneous melanocytosis. METHOD: We retrospectively reviewed cases of neurocutaneous melanocytosis referred to the pediatric neuro-oncology service at our center from 2003 to 2010. RESULTS: Of 14 patients (11 males, 3 females) identified, eight were living. Median age of survivors was 31 months (range 12mo-6y 10mo) while median age of death was 81 months (19mo-28y). Of the six patients who died, all had diffuse leptomeningeal melanocytic deposits and four had leptomeningeal melanoma. All patients had neuroimaging: six had findings suggestive of diffuse leptomeningeal melanocytosis; seven had multifocal melanocytic deposits; and one patient had normal neuroimaging but focal seizures. Spinal abnormalities were common: three patients had extensive dorsal spinal arachnoid cysts and one had a benign cervical spindle cell tumor. Seven patients had epilepsy. Three patients had profound developmental delay; the other 11 patients had no or mild delay. INTERPRETATION: Children with neurocutaneous melanocytosis exhibit a wide range of intracranial and intraspinal abnormalities and variable clinical outcomes.

Shall we report cardiomegaly at routine computed tomography of the chest?

PURPOSE: To examine the accuracy of the cardiothoracic ratio (CTR) at routine computed tomography (CT) of the chest to diagnose cardiomegaly by using echocardiography (ECHO) as a criterion standard, and secondarily, to search for CT cardiac measurements correlating with left ventricular hypertrophy (LVH) at ECHO. MATERIALS AND METHODS: We searched 2 databases: cancer patients who had chest CT who also underwent routine ECHO by one experienced cardiologist, from January to March 2008. Consecutive patients were enrolled who had chest radiography (CXR) within 1 month and ECHO within 6 months of CT. Cardiothoracic ratio was defined as the transverse greatest cardiac diameter from outer to outer myocardium on axial images divided by the transverse greatest thoracic diameter from inner to inner chest wall on axial images, as measured by 2 board-certified radiologists. Left ventricular short diameter on CT was measured from inner to inner myocardium. Left ventricular hypertrophy at ECHO was defined using the standard American Society of Echocardiography criteria. Other data captured included cardiac and chemotherapy history, and secondary signs of heart failure. The Pearson correlation coefficient (r) and Wilcoxon rank sum tests and receiver operating characteristic (ROC) curves were used for statistical analysis. RESULTS: 101 patients, 52 men and 49 women, with a mean age of 58 years met the entry criteria. Cardiothoracic ratio at CXR and CT were highly correlated (r = 0.802) (P < 0.001). There was moderate ability of CT CTR to identify LVH (area under the receiver operating characteristic curve AUC = 0.70; 95% CI, 0.51-0.90). The CT left ventricular short diameter showed moderate correlation with the ECHO left ventricular internal diameter (r = 0.49) and left ventricular mass (r = 0.37). CONCLUSION: In patients with cancer undergoing routine ECHO, the cardiothoracic ratio at routine CT scans was highly correlated with that at CXR. Preliminary estimates in this small study indicate a low likelihood of LVH when the CTR is less than 0.49.

Comparison of CT perfusion and digital subtraction angiography in the evaluation of delayed cerebral ischemia.

RATIONALE AND OBJECTIVES: Delayed cerebral ischemia (DCI) is a devastating condition that occurs secondary to aneurysmal subarachnoid hemorrhage (A-SAH). The purpose is to compare computed tomography perfusion (CTP) and digital subtraction angiography (DSA) for determining DCI in A-SAH. MATERIALS AND METHODS: A retrospective study of A-SAH patients admitted at our institution between December 2004 and December 2008 was performed. CTP and DSA were obtained at days 6-8 after aneurysm rupture. Both qualitative and quantitative analyses of CT perfusion deficits were performed. DSA was categorized as presence or absence of vasospasm. The reference standard for determining DCI was based on clinical deterioration or infarction on CT or MRI. The test characteristics of CTP and DSA were calculated and their graphs of conditional probabilities were constructed using Bayesian analysis. RESULTS: Fifty-seven patients were included; 79% (45/57) had DCI. Seventy percent (40/57) had CTP perfusion deficits; 80% (36/45) of the DCI and 33% (4/12) of no DCI patients. Sixty-three percent (36/57) had DSA demonstrating vasospasm; 73% (33/45) of the DCI and 25% (3/12) of no DCI patients. Quantitative analysis of the CTP data revealed a significant difference in cerebral blood flow values for the DCI (29.4 mL/100 g/minute) and no DCI groups (40.5 mL/100 g/minute, P = .0213). The sensitivity, specificity, and positive and negative predictive values for CTP were 0.80 (95% CI 0.68-0.92), 0.67 (95% CI 0.40-0.93), 0.90 (95% CI 0.82-0.96), 0.47 (95% CI 0.27-0.62), and for DSA were 0.73 (95% CI 0.60-0.86), 0.75 (95% CI 0.50-0.99), 0.92 (95% CI 0.82-0.98), and 0.43 (95% CI 0.26-0.53), respectively. CONCLUSION: CTP and DSA have similar test characteristics and Bayesian analysis for determining DCI in A-SAH patients.

Do all non-calcified echogenic renal lesions found on ultrasound need further evaluation with CT?

BACKGROUND: This is a study using Evidence Based Practice (EBP) technique to evaluate if non-calcified renal lesions detected with ultrasound, suspected to represent an angiomyolipoma (AML), need a CT to rule out a renal cell carcinoma (RCC). METHODS: The secondary and primary literature were searched for all relevant information. This was appraised for validity and strength. The results from the papers with the highest level of evidence were grouped together and analyzed. RESULTS: Three papers in the primary literature constituted the highest level of evidence. In total these three papers examined 220 lesions. The prevalence of AML was 45% in this sample. Overall, hyperechoic non-calcified renal lesions had a sensitivity of 0.99 (95% confidence interval (CI) 0.97-1.00), a specificity of 0.43 (95% CI 0.34-0.51), a positive predictive value (PPV) of 0.58 and a negative predictive value (NPV) of 0.98 for AMLs. 57.4% of RCCs were hyperechoic to renal parenchyma. Two of the studies found that posterior acoustic shadowing had a sensitivity of 0.34 (95% CI 0.40-0.56) and a specificity of 1.0 (95% CI 1.0-1.0) for AML. CONCLUSIONS: From the surprisingly limited evidence available in the literature, it must be concluded that all non-calcified echogenic renal lesions detected with ultrasound need a CT to rule out an RCC.