Venous angioplasty in patients with multiple sclerosis: results of a pilot study.

OBJECTIVES: Chronic cerebrospinal venous insufficiency (CCSVI) is associated with multiple sclerosis (MS). The objective of the study was to see if percutaneous transluminal angioplasty (PTA) of duplex-detected lesions, of the internal jugular and/or azygous veins, was safe, burdened by a significant restenosis rate, and whether there was any evidence that treatment reduced MS disease activity. DESIGN: This was a case-control study. MATERIALS: We studied 15 patients with relapsing-remitting MS and duplex-detected CCSVI. METHODS: Eight patients had PTA in addition to medical therapy (immediate treatment group (ITG)), whereas seven had treatment with PTA after 6 months of medical therapy alone (delayed treatment group (DTG)). RESULTS: No adverse events occurred. At 1 year, there was a restenosis rate of 27%. Overall, PTA was followed by a significant improvement in functional score compared with baseline (p < 0.02). The annualised relapse rate was 0.12% in the ITG compared with 0.66% in the DTG (p = NS). Magnetic resonance imaging (MRI) blindly demonstrates a trend for fewer T2 lesions in the ITG (p = 0.081), corresponding to a 10% decrease in the ITG compared with a 23% increase in the DTG over the first 6 months of the study. CONCLUSIONS: This study further confirms the safety of PTA treatment in patients with CCSVI associated with MS. The results, despite the significant rate of restenosis, are encouraging and warrant a larger multicentre double-blinded, randomised study.

Value of MR venography for detection of internal jugular vein anomalies in multiple sclerosis: a pilot longitudinal study.

BACKGROUND AND PURPOSE: CCSVI was recently described in patients with MS. CCSVI is diagnosed noninvasively by Doppler sonography and invasively by catheter venography. We assessed the role of conventional MRV for the detection of IJV anomalies in patients with MS diagnosed with CCSVI and in healthy controls who underwent MRV and Doppler sonography examinations during 6 months. MATERIALS AND METHODS: Ten patients with MS underwent TOF, TRICKS, Doppler sonography, and catheter venography at baseline. They were treated at baseline with percutaneous angioplasty and re-evaluated 6 months' posttreatment with MRV and Doppler sonography. In addition, 6 healthy controls underwent a baseline and a 6-month follow-up evaluation by Doppler sonography and MRV. RESULTS: At baseline, the sensitivity, specificity, PPV, and NPV of Doppler sonography for detecting IJV abnormalities relative to catheter venography in patients with MS were calculated, respectively, at 82%, 100%, 99%, and 95%. The figures were 99%, 33%, 33%, 99% for TOF and 99%, 39%, 35%, and 99% for TRICKS. Venous anomalies included the annulus, septum, membrane, and malformed valve. No agreement was found between TOF and catheter venography in 70% of patients with MS and between TRICKS and catheter venography in 60% of patients with MS. At follow-up, 50% of the patients with MS presented with abnormalities on Doppler sonography but only 30% were diagnosed with restenosis. CONCLUSIONS: Conventional MRV has limited value for assessing IJV anomalies for both diagnostic and posttreatment purposes.

The chronic cerebrospinal venous insufficiency syndrome.

Chronic cerebrospinal venous insufficiency (CCSVI) is a syndrome characterized by stenosies of the internal jugular and/or azygous veins (IJVs-AZ) with opening of collaterals and insufficient drainage proved by reduced cerebral blood flow and increased mean transit time in cerebral MRI perfusional study. The present review is aimed to give a comprehensive overview of the actual status of the art of the diagnosis and treatment of this condition. As far as the origin of venous narrowing is concerned, phlebographic studies of the IJVs and AZ systems demonstrated that venous stenoses were likely to be truncular venous malformations; mostly, they are intraluminal defects such as malformed valve, septa webs. CCSVI condition has been found to be strongly associated with multiple sclerosis (MS), a disabling neurodegenerative and demyelinating disease considered autoimmune in nature. In several epidemiological observations performed at different latitudes on patients with different genetic backgrounds, the prevalence of CCSVI in MS ranges from 56% to 100%. To the contrary, by using venous MR and/or different Doppler protocols, CCSVI was not detected with the same prevalence. Two pilot studies demonstrated the safety and feasibility in Day Surgery of the endovascular treatment of CCSVI by means of balloon angioplasty (PTA). It determines a significant reduction of postoperative venous pressure. Restenosis rate was found out elevated in the IJVs, but negligible in the AZ. However, PTA seems to positively influence clinical and QoL parameters of the associated MS and warrants further randomized control trials.

The reproducibility of colour Doppler in chronic cerebrospinal venous insufficiency associated with multiple sclerosis.

AIM: Chronic cerebrospinal venous insufficiency (CCSVI) is a syndrome described in multiple sclerosis (MS) patients, characterized by stenosis of the main extracranial veins with hampered cerebral venous outflow. In the original description echo-colour Doppler demonstrated to be an ideal non invasive tool for screening CCSVI patients, but the reproducibility was not assessed. Aim of this study is to assess the variability coefficient between trained and in not trained echo-colour Doppler operators. METHODS: Thirty-six (36) subjects, matched for age and gender, were subset in 3 groups (group A, 12 healthy controls, HC; group B, 12 multiple sclerosis patients, MS; group C, 12 patients with other neurological disease, OND) underwent echo-colour Doppler screening for CCSVI according to an original protocol previously described. The inter observer variability rate was assessed by comparing respectively trained vs not trained operators, and trained vs trained operators, by using the same echo-colour Doppler equipment. In addition, by scanning 15 subjects after one month from the first session, intra observer coefficient was also assessed in trained operator. RESULTS: The inter observer variability rate between trained and not trained echo-colour Doppler operators, were not completely satisfactory (K coefficient 0.47 95% CI 0.27-0.68). To the contrary the inter observer agreement between trained operators was much more reliable (K coefficient 0.80 95% CI 0.59-1.01). Finally, the intra observer variability rate in trained operators was 0.93, (95% CI 0.80-1.06) confirming a highly satisfactory agreement. CONCLUSION: Echo-colour Doppler is a powerful, non-invasive and reproducible tool for screening CCSVI-MS but it needs special training.

CSF dynamics and brain volume in multiple sclerosis are associated with extracranial venous flow anomalies: a pilot study.

AIM: We previously reported unexpectedly robust associations between vascular haemodynamic (VH) anomalies in the principal extracranial cerebral veins, causing chronic cerebrospinal venous insufficiency (CCSVI), and multiple sclerosis (MS). Aim of this study was to investigate the relationship between the VH changes and MRI measures of MS disease severity in a cross sectional survey. METHODS: The number of anomalous VH criteria were measured using an echo-color Doppler, whereas CSF flow, atrophy and lesion measures were obtained from quantitative magnetic resonance imaging (MRI) analysis in sixteen consecutive relapsing-remitting MS patients, (mean age: 36.1+/-SD 7.3 years, disease duration: 7.5+/-1.9 years and median EDSS: 2.5) and in 8 healthy controls (HC) with similar age and sex distributions. RESULTS: All 16 MS patients investigated and none of the HCs met the VH criteria for CCSVI (P<0.0001). MS patients showed significantly lower net CSF flow compared to the HC (P=0.038) that was associated with number of anomalous VH criteria present (r=0.79, P<0.001). Moreover, increases in the number of anomalous VH criteria present were negatively associated with lower whole brain volume (Spearman R=-0.5, P=0.05). CONCLUSION: VH changes occur more frequently in MS patients than controls. Altered VH is associated with abnormal CSF flow dynamics and decreased brain volume.

Is chronic fatigue the symptom of venous insufficiency associated with multiple sclerosis? A longitudinal pilot study.

AIM: Chronic fatigue (CF) severely affects patients with multiple sclerosis (MS), but its pathogenesis remains elusive and the effectiveness of available treatments is modest. We aimed to evaluate the effect on CF of the balloon dilatation of stenosing lesions affecting the main extracranial veins configuring the chronic cerebrospinal venous insufficiency (CCSVI), a condition strongly associated with MS. METHODS: Thirty-one MS consecutive patients (16 males, age 46.2+/-9.4 years) with associated CCSVI and CF underwent the endovascular procedure. Fatigue was assessed using the Fatigue Severity Scale (FSS) and Modified Fatigue Impact Scale (MFIS) at baseline (T0) and one (T1), six (T6) and twelve (T12) months after the procedure. In ambulatory patients (N.=28), mobility was evaluated using the 6-min walking test at T0 and T1. RESULTS: and MFIS scores significantly improved from preoperative values, and the positive trend was maintained at one year (FSS: T0=5.1+/-1.0 to T12=3.5+/-1.8, P<0.001; MFIS-total score: T0=34.9+/-14.8 to T12=22.5+/-13.7, P<0.001; MFIS-Physical subscale: T0=21.2+/-8.0 to T12=13.5+/-9.7 P<0.001; MFIS-Cognitive subscale: T0=9.2+/-9.5 to T12=6.0+/-6.3, P=0.03; MFIS-Psychosocial subscale: T0=4.5+/-2.1 to T12=2.5+/-2.1, P<0.001). Six-min walking distance (6MWD) at T1 improved significantly (332+/-190m to 378+/-200m, P=0.0002). In addition, an inverted correlation between 6MWD and MFIS-physical subscale variations was found in the subgroup of patients (N.=8) with no lower limb motor impairment (r=-0.74, P=0.035). CONCLUSION: The reestablishment of cerebral venous return dramatically reduced CF perception in a group of MS patients with associated CCSVI, suggesting that CF is likely the symptom of CCSVI.

Hemodynamic patterns of chronic cerebrospinal venous insufficiency in multiple sclerosis. Correlation with symptoms at onset and clinical course.

AIM: Chronic cerebrospinal venous insufficiency (CCSVI) is associated with multiple sclerosis (MS). CCSVI is detected by transcranial and extracranial color-Doppler high-resolution examination (TCCS-ECD) and venography that permit to identify five types of venous malformations and four major (A-D) hemodynamic patterns of anomalous extracranial-extravertebral venous outflow. We investigated possible correlation between such hemodynamic patterns and both the symptoms at onset and clinical course in patients with MS and CCSVI. METHODS: TCCS-ECD, selective venography and clinical records of 65 patients affected by definite MS and CCSVI were reviewed. RESULTS: The four hemodynamic patterns of CCSVI were unevenly (P<0.0001) distributed with respect to the types of clinical presentation and course. In particular the Type A or B patterns were common in patients with onset of optic neuritis, but rare in patients presenting with spinal cord symptoms who typically showed a type D pattern. As well, the type A or type B hemodynamic were more common in patients with relapsing remitting course than in patients with secondary progressive course and rare in patients with primary progressive course. The C hemodynamic pattern was not observed in patients with primary progressive course who showed a remarkable prevalence of the type D pattern. CONCLUSION: The distribution of venous malformations and the resulting hemodynamic pattern show correlation with symptoms at onset and clinical course in patients with MS and CCSVI.

Chronic cerebro-spinal venous insufficiency: report of transcranial magnetic stimulation follow-up study in a patient with multiple sclerosis.

The pyramidal pathway is frequently affected early on in multiple sclerosis (MS) and impaired motor performance is a major cause of disability. Pyramidal tract function can be assessed using transcranial magnetic stimulation (TMS). TMS supports the diagnosis of MS, detecting corticospinal tract involvement and monitoring its course with or without treatment. It has been never investigated whether any relationship exists between the TMS outcome measure and minimally invasive treatment of multiple severe extracranial stenosis, affecting the principal ce rebrospinal venous segments in MS patients. We report the clinical and transcranial magnetic stimulation follow-up of a patient during a relapse in relapsing-remitting MS. She underwent percutaneous balloon angioplasty of the associated chronic cerebrospinal venous insufficiency (CCSVI), due to membranous obstruction of the proximal azygous vein, with severe stenosis of the left internal jugular vein. Treatment of the associated CCSVI made a parallel improvement in both clinical and neurophysiological parameters, allowing us to avoid high dose steroid therapy. The relationship between the clinical and neurophysiological course on the one hand, and haemodynamic correction of the associated CCSVI on the other, calls for further exploration on a wider number of patients. The impact of CCSVI on the different neuro-physiological parameters has not been fully estimated, but the intriguing case here reported suggests that it may be greater than previously assumed. The demonstration of a modification of the cerebrovenous function with both clinical manifestation and via TMS suggests that the hampered cerebral venous return may contribute to the clinical course of MS.

The severity of chronic cerebrospinal venous insufficiency in patients with multiple sclerosis is related to altered cerebrospinal fluid dynamics.

Chronic cerebrospinal venous insufficiency (CCSVI) is a vascular picture that shows a strong association with multiple sclerosis (MS). The aim of this study was to investigate the relationship between a Doppler cerebral venous hemodynamic insufficiency severity score (VHISS) and cerebrospinal fluid (CSF) flow dynamics in 16 patients presenting with CCSVI and relapsing-remitting MS (CCSVI-MS) and in eight healthy controls (HCs). The two groups (patients and controls) were evaluated using validated echo-Doppler and advanced 3T-MRI CSF flow measures. Compared with the HCs, the CCSVI-MS patients showed a significantly lower net CSF flow (p=0.027) which was highly associated with the VHISS (r=0.8280, r2=0.6855; p=0.0001). This study demonstrates that venous outflow disturbances in the form of CCSVI significantly impact on CSF pathophysiology in patients with MS.

Chronic cerebrospinal venous insufficiency in patients with multiple sclerosis.

BACKGROUND: The extracranial venous outflow routes in clinically defined multiple sclerosis (CDMS) have not previously been investigated. METHODS: Sixty-five patients affected by CDMS, and 235 controls composed, respectively, of healthy subjects, healthy subjects older than CDMS patients, patients affected by other neurological diseases and older controls not affected by neurological diseases but scheduled for venography (HAV-C) blindly underwent a combined transcranial and extracranial colour-Doppler high-resolution examination (TCCS-ECD) aimed at detecting at least two of five parameters of anomalous venous outflow. According to the TCCS-ECD screening, patients and HAV-C further underwent selective venography of the azygous and jugular venous system with venous pressure measurement. RESULTS: CDMS and TCCS-ECD venous outflow anomalies were dramatically associated (OR 43, 95% CI 29 to 65, p<0.0001). Subsequently, venography demonstrated in CDMS, and not in controls, the presence of multiple severe extracranial stenosis, affecting the principal cerebrospinal venous segments; this provides a picture of chronic cerebrospinal venous insufficiency (CCSVI) with four different patterns of distribution of stenosis and substitute circle. Moreover, relapsing-remitting and secondary progressive courses were associated with CCSVI patterns significantly different from those of primary progressive (p<0.0001). Finally, the pressure gradient measured across the venous stenosies was slightly but significantly higher. CONCLUSION: CDMS is strongly associated with CCSVI, a scenario that has not previously been described, characterised by abnormal venous haemodynamics determined by extracranial multiple venous strictures of unknown origin. The location of venous obstructions plays a key role in determining the clinical course of the disease.

Clinical features of sarcomatoid renal cell carcinoma causing bilateral urinary tract obstruction.

We report on a 52-year-old female patient hospitalized because of uremia due to bilateral urinary tract obstruction caused by bilateral sarcomatoid renal cell carcinoma (SRCC). Abdominal computed tomography with contrast showed a large mass on the left side, infiltrating the left kidney, while the right kidney was described as enlarged. The latter was investigated with sonographic angiography using contrast and selective arteriography of the renal arteries, demonstrating a pseudonodular area at the inferior pole of the right kidney. The patient underwent bilateral nephrectomy and chronic hemodialysis treatment; unfortunately, after one month she died from cachexia. To the best of our knowledge this is the first case to be reported on bilateral SRCC causing bilateral urinary tract obstruction.

Pseudostenosis of axillary vein due to adduction of the upper limb.

Axillary vein stenosis is a well-documented complication in hemodialysis (HD) patients, but in adipose or athletic subjects, pseudostenosis of the axillary vein can be detected. We report a case of pseudostenosis in a fit dialysis patient who underwent phlebography of the right upper limb, performed to create a new vascular access (VA). The investigation demonstrated that the axillary vein appeared sharpened with a reduced lumen. Based on this exam, angioplasty of the right axillary vein was planned. The new phlebographic study, performed abducting the arm, demonstrated that the previously marked stenosis of the axillary vein disappeared. Our case illustrates how the reduction of the axillary vein lumen was secondary to ""muscular print"" and was present only if the arm was adducted. In uremic patients, the squeezing of the vascular nervous bundle of the upper limb is possible in fit patients at the time of phlebography, an examination that should be performed with the arm both in adduction and abduction.

High output heart failure due to an iatrogenic arterio-venous fistula after lumbar disc surgery.

Vascular injury is a rare but not uncommon complication of spinal surgery and associated with dramatic consequences. Congestive heart failure secondary to a hyperkinetic circulation can occur in systemic diseases and in arterio-venous fistulae. A 57-year-old man was admitted to hospital complaining of dyspnoea and oedema of the left leg. Eight days previously he had undergone a discectomy at L4-L5. On auscultation a systolic-diastolic murmur was noted over the entire abdomen. An echocardiogram demonstrated an enlarged right atrium, severe mitral and tricuspid regurgitation and increased pulmonary artery pressure. An abdominal CT demonstrated irregular dilatation of the left common iliac vein and through a fistula and simultaneous opacification of the right common iliac artery; subsequently, this was also confirmed by angiography. The patient underwent an emergency endovascular stent-graft of the right common iliac artery with normalization of the venous return pressure and quick resolution of the heart failure. It is important for the physician involved in clinical work to keep in mind all the possible post-surgical complications that can occur in symptomatic patients who have recently undergone an intervention.

Double right bronchial artery aneurysm treated with combined procedures.

PURPOSE: Bronchial artery aneurysms occur rarely. We present an unusual case. CASE REPORT: We present a patient with double right bronchial artery aneurysms that were treated with a combination of endovascular and surgical procedures. CONCLUSION: This case report illustrates the treatment options for this unusual problem.

Displacement of the inferior vena cava as a factor limiting catheter performance in long-term hemodialysis patients.

We report a case of a lady affected by autosomal dominant polycystic kidney disease who had been on hemodialyis for 24 years. She has exhausted all options for arterious-venous fistula. The presence of an acquired anatomical abnormality was an obstacle in order to get appropriate blood flow from standard tunnelled femoral catheters. The enlarged right kidney was pushing the inferior vena cava to the left side of the abdomen, and the abnormality was demonstrated by phlebography. Only after placing a cuffed catheter 53 cm long in her left femoral vein we could dialyze efficiently. Venography is mandatory before placing a cuffed catheter especially in uremic patients with long history of access failure, because it saves costs.

The crowned dens syndrome. Evaluation with CT imaging.

PURPOSE: The purpose of this study was to verify the value of computed tomography (CT) in the diagnosis of the "crowned dens" syndrome, not only in crystal deposition diseases, but also in other rheumatic or nonrheumatic conditions. MATERIALS AND METHODS: Thirty-eight patients (15 men and 23 women; mean age 55 years; age range 35-79) with neck pain were examined and divided into two groups: (1) patients already identified as rheumatic and referred for further investigation of the atlantoaxial region; (2) patients with symptoms confined to the cervical spine, with inconclusive radiographic findings. Unenhanced CT of the cervical spine (Tomoscan SR 7000 Philips, Eindhoven, Netherlands) was performed in all patients. There were 11 cases of rheumatoid arthritis (ten women and one man), two calcium pyrophosphate dihydrate crystal deposition disease (both women), one of systemic sclerosis (a woman), one of osteoarthritis (a man), one of seronegative arthritis (a man), four of neoplasm (one woman and three men) with suspected cervical involvement, one (a man) of haematological disease (lymphoma), one (a woman) of menopausal osteoporosis, ten (five men and five women) of recent or previous trauma with suspected involvement of the skull base and first cervical vertebrae and six of unknown painful cervical dysfunction (three men and three women). RESULTS: CT demonstrated calcific deposits around the dens in 12 patients (three men and nine women), in the transverse and alar ligaments, and in the anterior atlantooccipital membrane. CT revealed horseshoe- or crown-like calcification surrounding the odontoid process. In our series, other rheumatic diseases, especially rheumatoid arthritis, showed similar irregular calcifications of the atlantoaxial joint. Discussion. In calcium pyrophosphate dihydrate (CPPD) crystal deposition disease, the spine may be the only site of involvement, generally asymptomatic. Crystals located in the transverse ligament of the atlas give rise to the crowned dens syndrome, usually in patients affected by severe degenerative lesions of the atlantoaxial joint and peripheral chondrocalcinosis. Symptoms may be absent, or a neurological compressive syndrome may develop. Symptoms tend to worsen with age. The diagnosis is not always easy, as the symptoms are similar to those of other diseases, such as meningitis, cervicobrachial pain, occipitotemporal headache, calcific tendinitis of the longus colli muscle, spondylodiscitis and retropharyngeal abscess. CONCLUSION: CT is the gold standard in identifying crowned dens syndrome, as it is able to depict the shape and site of calcification and any bone erosions. Radiography of other joints (wrist, knee, pubic symphysis) may help to ascertain whether the disease is due to calcium pyrophosphate dihydrate or hydroxyapatite crystals, and is therefore recommended for routine patient management. Magnetic resonance imaging (MRI) is indicated for the study of neurological complications.

A case of disseminated Langerhans' cell histiocytosis treated with thalidomide.

Tumor necrosis factor alpha (TNF-alpha) seems to play a key role in the pathogenesis of Langerhans' cell histiocytosis (LCH). Thalidomide is an immunomodulator agent of inflammatory cytokines including TNF-alpha. To our knowledge this is the first case of disseminated LCH successfully treated with thalidomide.

[Metastatic bone disease. Strategies for imaging]. / La malattia ossea metastatica. Strategia della diagnostica per immagini.

Skeletal metastases represent the most common malignant bone tumor. They occur mainly in adults and even more frequently in the elderly. The most common metastases in men are from prostate cancer (60%) and in women from breast cancer (70%). Other primitive tumors responsible for bone metastases are: lung, kidney, thyroid, alimentary tract, bladder, and skin. The spine and pelvis are the most common metastatic sites, due to the presence of red (haematopoietic active) bone marrow in a high amount. As a general rule, the radiographic pattern was lytic type; other aspects were osteosclerotic, mixed, lytic vs mixed and osteosclerotic vs lytic patterns. The main symptom is pain, although many bone metastases are asymptomatic. The most severe consequences are pathologic fractures and cord compression. Clinical evaluation of patients with skeletal metastases needs multimodal diagnostic imaging, able to detect lesions, to assess their extension and localization, and eventually drive the biopsy (for histo-morphological diagnosis). These techniques give different performances in terms of sensitivity and specificity; but none of the modalities alone seems to be adequate to yield a reliable diagnostic outcome. Therefore multidisciplinary cooperation is required to optimize the screening, clinical management and follow-up of the patients. In other terms, what is the efficacy of these new diagnostic tests compared to the "older" diagnostic tests? Frequently the new procedures do not replace the older one, but it is added to the diagnostic workup, thereby increasing costs without impacting the "patient's condition". The aim of the present work is to propose an "algorithm" for the detection and diagnosis of skeletal metastases, which may be applied differently in symptomatic and asymptomatic oncologic patients. Bone scintigraphy remains the first choice technique in the evaluation of asymptomatic patients, in whom skeletal metastases are supposed. Although it has a high sensitivity, scintigraphy is unspecific. So that a negative scan response has to be re-evaluated with other methods: if clinical status remains "negative", the diagnostic route can stop. On the contrary, in patients with "positive" scan or with local symptoms and pain, the screening of metastatic lesions must be accomplished by a combination of radiography and CT: the result may be negative (for low sensitivity of conventional radiology), not conclusive (in this case bone biopsy is necessary) or symptoms are not due to metastatic lesions (i.e., osteoarthritis). CT represents an excellent mean of defining the extent of any metastatic lesions, especially those located at sites difficult to evaluate (vertebral column and pelvis). Before bone biopsy is carried out, MRI must be performed, because it is the only technique that makes it possible to distinguish between bone marrow components. It has been used most extensively in the evaluation of spine metastases. The limitation of MRI is the unspecificity of its findings, which may lead to an equivocal diagnosis, and because only part of the skeleton can be studied.