Small bowel gastrointestinal stromal tumor presenting with gastrointestinal bleeding in patient with type 1 Neurofibromatosis: Management and laparoscopic treatment. Case report and review of the literature.

INTRODUCTION AND IMPORTANCE: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. It may be asymptomatic; nevertheless, gastrointestinal bleeding is the most frequent symptom, due to mucosal erosion. Its poor lymph node metastatic spread makes GIST often suitable of minimally invasive surgical approach. The importance of this study is to increase the awareness among physicians about this condition in particular scenarios as in our case and to stress the role of laparoscopic surgery. CASE PRESENTATION: A 74-year-old female patient presented to the emergency department with hematemesis, followed by haematochezia and melena. The patient had a medical history of type 1 Neurofibromatosis (NF1). She underwent, after CT scan, esophagogastroduodenoscopy, and endoscopic haemostasis. Finally, we performed a laparoscopic resection of a mass of the first jejunal loop. The postoperative period was predominantly uneventful. Pathological examination confirmed a low-risk GIST. CLINICAL DISCUSSION: Proximal jejunal GIST may cause an upper and lower gastrointestinal bleeding. A multidisciplinary team approach is mandatory for the correct management of this disease and its complications (bleeding). GISTs are indicated as the most commonly gastrointestinal NF1 associated tumours. In case of localised and resectable GIST surgical treatment is the mainstay and laparoscopic surgery is a valid alternative. CONCLUSION: In case of abdominal bleeding mass in a NF1 patient, it is important to keep in mind the well-known association between NF1 and GIST to facilitate the diagnosis and to quickly perform the appropriate treatment. Laparoscopic approach is safe and effective if the oncological radicality is respected.

Does the cerebellar sequential theory explain spoken language impairments? A literature review.

During the past decades, converging evidence from clinical, neuroimaging and neuroanatomical studies has demonstrated the key role of the cerebellum in the processing of non-motor aspects of language. Although more is known about the way in which the cerebellum participates in the mechanisms involved in written language, there is ambiguous information on its role in other aspects of language, such as in non-motor aspects of spoken language. Thus, to contribute additional insight into this important issue, in the present work, we review several original scientific papers focusing on the most frequent non-motor spoken language impairments evidenced in patients affected by cerebellar pathology, namely, verbal working memory, grammar processing and verbal fluency impairments. Starting from the collected data, we provide a common interpretation of the spoken language disorders in cerebellar patients, suggesting that sequential processing could be the main mechanism by which the cerebellum participates in these abilities. Indeed, according to the cerebellar sequential theory, spoken language impairments could be due to altered cerebellar function to supervise, synchronize and coordinate the activity of different functional modules, affecting the correct optimization of linguistic processing.

Functional Changes of Mentalizing Network in SCA2 Patients: Novel Insights into Understanding the Social Cerebellum.

In recent years, increasing evidence of the cerebellar role in social cognition has emerged. The cerebellum has been shown to modulate cortical activity of social brain regions serving as a regulator of function-specific mentalizing and mirroring processes. In particular, a mentalizing area in the posterior cerebellum, specifically Crus II, is preferentially recruited for more complex and abstract forms of social processing, together with mentalizing cerebral areas including the dorsal medial prefrontal cortex (dmPFC), the temporo-parietal junction (TPJ), and the precuneus. In the present study, the network-based statistics approach was used to assess functional connectivity (FC) differences within this mentalizing cerebello-cerebral network associated with a specific cerebellar damage. To this aim, patients affected by spinocerebellar ataxia type 2 (SCA2), a neurodegenerative disease specifically affecting regions of the cerebellar cortex, and age-matched healthy subjects have been enrolled. The dmPFC, left and right TPJ, the precuneus, and the cerebellar Crus II were used as regions of interest to construct the mentalizing network to be analyzed and evaluate pairwise functional relations between them. When compared with controls, SCA2 patients showed altered internodal connectivity between dmPFC, left (L-) and right (R-) TPJ, and right posterior cerebellar Crus II.The present results indicate that FC changes affect a function-specific mentalizing network in patients affected by cerebellar damage. In particular, they allow to better clarify functional alteration mechanisms driven by the cerebellar damage associated with SCA2 suggesting that selective cortico-cerebellar functional disconnections may underlie patients' social impairment in domain-specific complex and abstract forms of social functioning.

16p11.2 microdeletion syndrome: a case report.

BACKGROUND: The recurrent ∼ 600 kb 16p11.2 microdeletion is among the most commonly known genetic etiologies of autism spectrum disorder, overweightness, and related neurodevelopmental disorders. CASE PRESENTATION: Our patient is a 2-year-old white girl from the first pregnancy of a non-consanguineous healthy young white couple (father 33-years old and mother 29-years old). Our patient and her parents' DNA were analyzed by comparative genomic hybridization-array platform. Comparative genomic hybridization-array analysis highlighted a ∼ 600 kb deletion in 16p11.2 region. It has a segregant nature, since it was found in the mother and in her 2-year-old daughter. The microdeletion was confirmed by fluorescence in situ hybridization analysis. CONCLUSIONS: The presented clinical case is worthy of note since the observed microdeletion is often associated with a clinical phenotype tending to overweightness, but the proband (female) was hospitalized due to poor height and weight development, and anorexia. Moreover, the segregant nature of the observed genomic abnormality has to be noted, as well as the phenotypic variability between the mother and daughter. The case described here enriches the phenotypical spectrum linked to the 16p11.2 microdeletion. For these reasons, in the presence of a suspected genetic pathology it is fundamental to study the proband from the clinical point of view, to extend the clinical observation to the parents, and to provide a good family anamnesis. In this way, it is possible to reveal the presence of a familial genetic pathology whose phenotypical outcomes can be highly variable among the members of a family.

Structural cerebellar correlates of cognitive functions in spinocerebellar ataxia type 2.

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum and characterized by a typical motor syndrome. In addition, the presence of cognitive impairment is now widely acknowledged as a feature of SCA2. Given the extensive connections between the cerebellum and associative cerebral areas, it is reasonable to hypothesize that cerebellar neurodegeneration associated with SCA2 may impact on the cerebellar modulation of the cerebral cortex, thus resulting in functional impairment. The aim of the present study was to investigate and quantitatively map the pattern of cerebellar gray matter (GM) atrophy due to SCA2 neurodegeneration and to correlate that with patients' cognitive performances. Cerebellar GM maps were extracted and compared between SCA2 patients (n = 9) and controls (n = 33) by using voxel-based morphometry. Furthermore, the relationship between cerebellar GM atrophy and neuropsychological scores of the patients was assessed. Specific cerebellar GM regions were found to be affected in patients. Additionally, GM loss in cognitive posterior lobules (VI, Crus I, Crus II, VIIB, IX) correlated with visuospatial, verbal memory and executive tasks, while additional correlations with motor anterior (V) and posterior (VIIIA, VIIIB) lobules were found for the tasks engaging motor and planning components. Our results provide evidence that the SCA2 neurodegenerative process affects the cerebellar cortex and that MRI indices of atrophy in different cerebellar subregions may account for the specificity of cognitive symptomatology observed in patients, as result of a cerebello-cerebral dysregulation.

Microstructural MRI Basis of the Cognitive Functions in Patients with Spinocerebellar Ataxia Type 2.

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum. The particular atrophy pattern results in some typical clinical features mainly including motor deficits. In addition, the presence of cognitive impairments, involving language, visuospatial and executive functions, has been also shown in SCA2 patients and it is now widely accepted as a feature of the disease. The aim of the study is to investigate the microstructural patterns and the anatomo-functional substrate that could account for the cognitive symptomatology observed in SCA2 patients. In the present study, diffusion tensor imaging (DTI) based-tractography was performed to map the main cerebellar white matter (WM) bundles, such as Middle and Superior Cerebellar Peduncles, connecting cerebellum with higher order cerebral regions. Damage-related diffusivity measures were used to determine the pattern of pathological changes of cerebellar WM microstructure in patients affected by SCA2 and correlated with the patients' cognitive scores. Our results provide the first evidence that WM diffusivity is altered in the presence of the cerebellar cortical degeneration associated with SCA2 thus resulting in a cerebello-cerebral dysregulation that may account for the specificity of cognitive symptomatology observed in patients.

Prevention of neural tube defects and maternal gestational diabetes through the inositol supplementation: preliminary results.

OBJECTIVE: Our study aims to demonstrate that the use in the preconceptional period until the 24th week of pregnancy of inositol and folic acid, first of all, preserves the product of conception from neural tube defects (NTDs) and then, thanks to inositol supplementation, it possibly counteracts and prevents the onset of maternal gestational diabetes (GDM). PATIENTS AND METHODS: We have collected data derived from pregnant women arrived at our laboratory, from January 2014 to January 2016, with no family history of type 2 diabetes and hypertension. The first group (n = 68 women) was treated from the preconceptional period until the 24th week of pregnancy with 1.75 g/day myo-inositol, 250 mg/day D-chiro-inositol, 12.5 mg/day Zinc pidolate, 100 mg/day methylsulfonylmethane, 120 mg/day Vitamin C and 400 mcg/day (6S)-5-methyltetrahydrofolic acid. The control group (n = 72) was only treated with 400 mcg/day folic acid. The main outcome measure was the prevalence of maternal GDM. Secondary outcome measures were the prevalence of NTDs and fetal macrosomia. RESULTS: A significant difference was found regarding body mass index (BMI), fasting oral glucose tolerance test (OGTT), after 1-h-glucose OGTT, 2-h-glucose OGTT, glycated hemoglobin (HbA1c) and serum folate, between the two groups. Five infants, in the control group, weighted greater than 4 kg. Moreover, we found a positive correlation between HbA1c and OGTT at the 24th week of pregnancy. CONCLUSIONS: This study shows the efficacy of preconceptional supplementation of inositol to reduce the risk of the onset of GDM and to confirm the importance of folic acid supplementation to avoid NTDs development. Moreover, the positive correlation between HbA1c and OGTT may be useful to consider the use of HbA1c as a single tool for GDM prevention and diagnosis in selected woman in pregnancy.

Neural substrates of motor and cognitive dysfunctions in SCA2 patients: A network based statistics analysis.

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease characterized by a progressive cerebellar syndrome, which can be isolated or associated with extracerebellar signs. It has been shown that patients affected by SCA2 present also cognitive impairments and psychiatric symptoms. The cerebellum is known to modulate cortical activity and to contribute to distinct functional networks related to higher-level functions beyond motor control. It is therefore conceivable that one or more networks, rather than isolated regions, may be dysfunctional in cerebellar degenerative diseases and that an abnormal connectivity within specific cerebello-cortical regions might explain the widespread deficits typically observed in patients. In the present study, the network-based statistics (NBS) approach was used to assess differences in functional connectivity between specific cerebellar and cerebral "nodes" in SCA2 patients. Altered inter-nodal connectivity was found between more posterior regions in the cerebellum and regions in the cerebral cortex clearly related to cognition and emotion. Furthermore, more anterior cerebellar lobules showed altered inter-nodal connectivity with motor and somatosensory cerebral regions. The present data suggest that in SCA2 a cerebellar dysfunction affects long-distance cerebral regions and that the clinical symptoms may be specifically related with connectivity changes between motor and non-motor cerebello-cortical nodes.

Intestinal permeability in non-alcoholic fatty liver disease: the gut-liver axis.

The gut-liver axis model has helped to explain the liver steatosis (NAFLD) and steatohepatitis (NASH) etiopathogenesis. The discovery of a key role for an altered intestinal permeability (IP) in this pathophysioligcal framework has closed the link between gut lumen antigenic/toxic substances and systemic and liver inflammation in NAFLD and obesity, metabolic syndrome. Recent evidence from the literature show how IP can be modulated by several non-pharmacological and pharmacological agents and be the target for future preventive and curative treatment of NAFLD and NASH. In this review we describe the concept of IP, its ultrastructural basis, its role in the NALFD pathophysiology and emerging evidence on non-pharmacological and pharmacological agents able to favourably modulate it.

Sequential treatment with monofluorophosphate and zoledronic acid in osteoporotic rats.

OBJECTIVE: Osteoporosis is the consequence of an imbalance in bone remodeling caused by excessive resorption or inappropriate bone formation. This paper proposes a sequential treatment with monofluorophosphate (MFP) and zoledronic acid (Z), together with changes in the calcium content in the diet. METHOD: Seven-week-old female Sprague Dawley rats were divided into five groups (n = 21 per group): (1) sham-operated rats (Sham); (2) ovariectomized (OVX) rats fed with a normal calcium diet (OVX); (3) OVX rats fed with a normal calcium diet and treated sequentially with monofluorophosphate and zoledronic acid (OVX.G1); (4) OVX rats sequentially fed with a low calcium diet and then a high calcium diet, without treatment (OVX.G2); (5): OVX rats fed with a low calcium diet and then a high calcium diet, treated sequentially with monofluorophosphate and zoledronic acid (OVX.G3). RESULTS: After 150 days, the OVX.G3 group showed a similar bone volume to that of the Sham group due to an increase in trabecular number. Dual X-ray absorptiometry bone analysis showed an increase of 9.8% compared with OVX rats. Additionally, an increase in the fracture load at the cortical bone and higher fracture load, ultimate load and stiffness in the compression test were found. CONCLUSION: The sequential treatment with monofluorophosphate and zoledronic acid increases trabecular bone mass, bone mineral density and bone strength.

Inhaled nitric oxide versus sodium nitroprusside for preoperative evaluation of pulmonary hypertension in heart transplant candidates.

OBJECTIVE: The development of pulmonary hypertension before heart transplantation increases the risk for postoperative right ventricular failure. Reversibility of pulmonary vascular resistance (PVR), which indicates the feasibility of heart transplantation, can be tested with the use of intravenous vasodilators, such as sodium nitroprusside (NaNTP) or prostacyclin. However, the drawback of these drugs is the development of systemic hypotension. The aim of this study was to evaluate the safely and feasibility of inhaled nitric oxide (iNO) compared with sodium nitroprusside to test PVR reversibility, while avoiding systemic hypotension. MATERIALS AND METHODS: We included all patients who were affected by end stage heart failure undergoing evaluation for heart transplantation if they showed elevated PVR > 2.5 Wood units and mean pulmonary arterial pressure (mPAP) >25 mm Hg. The hemodynamic parameters measured by right heart catheterization were: systolic blood pressure (SBP), mPAP, pulmonary capillary wedge pressure, and cardiac index (CI). The following variables were derived: transpulmonary gradient (TPG) and PVR. All patients were tested by both iNO (20-40 ppm) and intravenous NaNTP, at increasing dosages which were titrated based on systemic pressure. We randomly assigned the order of administration of iNO and NaNTP. RESULTS: The 9 male candidates has an average age of 56 ± 4 years. Seven of the 9 (71%) had postischemic cardiomyopathy, and 2 had idiopathic cardiomyopathy. We observed a reduction of mPAP (32% and 14%), PVR (41% and 32%), TPG (20% and 26%), and SBP (17% and 5%) and an increase of CI with administration of NaNTP and iNO, respectively. CONCLUSIONS: We observed a reduction in PVR and mPAP with administration of either iNO and NaNTP. A better effect of NaNTP was attributed to reducted post-load of the left ventricle. However, the main advantage of iNO was the absence of systemic hypotension and its selectivity for pulmonary vascular system, as underscored by TPG reduction.

Mid-term prognostic value of coronary artery disease in patients undergoing transcatheter aortic valve implantation: a meta-analysis of adjusted observational results.

AIMS: Coronary artery disease (CAD) negatively affects prognosis in patients undergoing surgical aortic valve replacement, being currently evaluated in the most common used risk score. Our meta-analysis aims to clarify the prognostic role of CAD on mid-term survival in patients undergoing TAVI. METHODS AND RESULTS: Studies reporting multivariate predictors of adverse outcomes in patients undergoing TAVI were systematically searched for and pooled, when appropriate, using a random-effect method. 960 citations were first screened and finally 7 studies (2472 patients) were included. Diagnosis of CAD was reported in 52%(42-65) of patients and 1169 Edwards SAPIEN and 1303 CoreValve prostheses were implanted. After a median follow up of 452 days (357-585) 24% of patients (19-33) died, and 23 (14-32) for cardiovascular death. At pooled analysis of multivariate approach, diagnosis of coronary artery disease did not increase risk of death (OR 1.0, 95% CI, confidence interval, 0.67-1.50 I(2) 0%). CONCLUSION: CAD does not affect mid-term TAVI outcome: this finding should be weighted to accurately evaluate risk and strategies for patients with severe aortic stenosis.

Interval training in men at risk for insulin resistance.

We compared 3 months of eucaloric (12 kcal/kg/wk) steady state aerobic training (AER) to interval training (INT) in men at risk for insulin resistance. Primary outcomes included oral glucose tolerance testing (OGTT) and HOMA-IR 24 h and 72 h after each participants last exercise session. Secondary outcomes were VO2max, anthropometry, and metabolic syndrome expressed as a summed z-score (zMS). We also performed a sub-analysis for participants entering the trial above and below the HOMA-IR study median. Mean (95% CI) AER ( - 12.81 mg/dl; - 24.7, - 1.0) and INT ( - 14.26 mg/dl; - 24.9, - 3.6) significantly improved 24 h OGTT. HOMA-IR did not improve for AER, but did for INT 24 h and 72 h post-exercise. VO2max improved similarly for both groups. Changes in body mass for INT ( - 2.29 kg; - 3.51, - 1.14), AER, ( - 1.32 kg; - 2.62, 0.58)] and percent body fat [INT, - 0.83%; - 1.62, - 0.03), AER ( - 0.17%; - 1.07, 0.06)] were only significant for INT. When examined as a full cohort, zMS improved for both groups. Upon HOMA-IR stratification, only high HOMA-IR AER showed significant improvements, while both low and high INT HOMA-IR participants demonstrated significant reductions (P<0.05). Eucaloric AER and INT appear to affect fasting glucose, OGTT and VO2max similarly, while INT may have a greater impact on HOMA-IR and zMS.

Effect of astaxanthin on cycling time trial performance.

We examined the effect of Astaxanthin (AST) on substrate metabolism and cycling time trial (TT) performance by randomly assigning 21 competitive cyclists to 28 d of encapsulated AST (4 mg/d) or placebo (PLA) supplementation. Testing included a VO2max test and on a separate day a 2 h constant intensity pre-exhaustion ride, after a 10 h fast, at 5% below VO2max stimulated onset of 4 mmol/L lactic acid followed 5 min later by a 20 km TT. Analysis included ANOVA and post-hoc testing. Data are Mean (SD) and (95% CI) when expressed as change (pre vs. post). Fourteen participants successfully completed the trial. Overall, we observed significant improvements in 20 km TT performance in the AST group (n=7; -121 s; 95% CI, -185, -53), but not the PLA (n=7; -19 s; 95% CI, -84, 45). The AST group was significantly different vs. PLA (P<0.05). The AST group significantly increased power output (20 W; 95% CI, 1, 38), while the PLA group did not (1.6 W; 95% CI, -17, 20). The mechanism of action for these improvements remains unclear, as we observed no treatment effects for carbohydrate and fat oxidation, or blood indices indicative of fuel mobilization. While AST significantly improved TT performance the mechanism of action explaining this effect remains obscure.

Mathematical model for the homeostasis of alpha-macroglobulins in the rat.

Alpha-macroglobulins (AM) are proteins that inactivate proteinases. Sodium monofluorophosphate (MFP) binds to AM and transiently changes AM plasma levels. As a consequence MFP is useful to modify AM homeostasis. A mathematical model to study the homeostasis of AM is proposed in this paper. The model describes changes in plasma concentration of AM, MFP concentration in the gastrointestinal tract, MFP plasma concentration, plasma concentration of AMMFP and includes rate constants of the processes involved in AM homeostasis. Estimation of the rate constants values was achieved using experimental and mathematical resources. The homeostasis of AM after an oral dose of 80 µmol of MFP was analyzed with a simulation tool. Experimental conditions that modify the homeostasis of AM had been simulated and validated using specific drugs that change some parameter of the system. The mathematical model describes accurately the behavior of the biological model. The results allow concluding that the simplifications made did not underestimate the main processes involved in the homeostasis and, also that the assumptions made were correct.

Epidural plus general anesthesia vs general anesthesia alone for elective aortic surgery: effects on gastric electrical activity and serum gastrin secretion.

BACKGROUND: The aim of this study was to evaluate differences in electrogastrographic activity and serum gastrin secretion in patients subjected to general anesthesia (GA) vs blended anesthesia (BA = GA plus epidural analgesia) for abdominal aortic surgery. METHODS: Thirty-four patients (all males: 28 with abdominal aorta aneurysm, 6 with obstructive aorto-iliac disease; mean age: 68+/-7 years) were randomly assigned either to GA (N.=17) or to BA (N.=17) for abdominal aortic surgery. Each patient was evaluated for serum gastrin secretion at the time of electrogastrography (EGG) 24 h before and after surgery, using ambulatory equipment. Gastrin levels were tested under fasting conditions and after a standard meal. EGG shows gastric electrical activity that parallels gastric motor activity. RESULTS: Before surgery, no significant difference was found for any of the EGG parameters or the serum gastrin integrated value (area under the curve [AUC]) between the two groups of patients. After surgery, an increased frequency of electrical waves (tachygastria) was observed in 22% of those undergoing GA and in 5% of patients undergoing BA. The power ratio (postprandial/fasting total power) was exceedingly high (>4) in 53% of the GA patients and in 11% of the BA patients (P<0.05). The gastrin AUC was 263+/-58 pg/mL in the GA group and 179+/-92 pg/mL in the BA group (P<0.01). CONCLUSIONS: An excess of EGG activity and serum gastrin secretion was observed in patients undergoing GA vs those submitted to BA. Thus, the latter procedure seems to affect gastric function less than GA alone.

Fluid responsiveness and right ventricular function in cardiac surgical patients. A multicenter study.

INTRODUCTION: We investigated fluid responsiveness in a population of patients undergoing coronary artery revascularization, with respect to their right ventricular ejection fraction. MATERIALS AND METHODS: This was a multicenter trial involving 11 cardiac surgical Institutions and 65 patients undergoing elective coronary artery revascularization. Hemodynamic parameters were measured before and after volume expansion using a modified pulmonary artery catheter and transesophageal echocardiographic monitoring. Patients demonstrating an increase of stroke volume >20% after volume expansion were considered as responders. Volume expansion with 7 ml/kg of plasma expander was performed when required on a clinical basis. RESULTS: In the overall population, only the change in aortic blood velocity (cut-off 13%) was a predictor of fluid responsiveness. In patients with a reduced (<0.3) right ventricular ejection fraction only the value of mean pulmonary arterial pressure was predictive of fluid responsiveness (cut-off 18 mmHg). Patients with right ventricular ejection fraction ≥0.3 demonstrated three predictors: changes in aortic blood velocity (cut-off 15%), right ventricular end diastolic volume index (cut-off 80 ml/m(2)), and left ventricular end diastolic area index (cut-off 9 cm(2)/m(2)). CONCLUSIONS: When right ventricular systolic function is depressed, the right ventricle inability to fill the left chambers results in a lack of the left-sided responsiveness predictors. When the right ventricular systolic function is preserved, all the classical fluid responsiveness predictors are confirmed. Right ventricular function is therefore to be always considered when addressing the problem of fluid responsiveness.

A novel Notch3 gene mutation not involving a cysteine residue in an Italian family with CADASIL.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary cerebrovascular disease leading to accumulating neurologic deficits and dementia. CADASIL has been linked to nucleotide substitutions and deletions in the Notch3 gene. All the mutations described until now lead to unpaired cysteine residue in the epidermal growth factor-like repeats. The authors report a family with CADASIL carrying a deletion in the Notch3 gene that did not involve a cysteine residue.

Early experience with the minimal extracorporeal circulation system (MECC) during thoracoabdominal aortic aneurysm repair.

AIM: The purpose of this report is to describe our early experience with a minimal extracorporeal circulation system (MECC), a compact closed heparin coated system consisting of a centrifugal pump and a membrane oxygenator, during thoracoabdominal aortic aneurysm (TAAA) repair. METHODS: Between January and December 2002 the MECC system was employed in seven consecutive patients (four TAAA type II, two TAAA type I and one TAAA type III according to the Crawford classification). In all patients distal aortic, selective renal and visceral perfusion was performed with this compact closed heparin coated system consisting of a centrifugal pump and a membrane oxygenator. RESULTS: The MECC system was used in all cases with no technical malfunctions. Six out seven patients were discharged from the unit. One patient developed paraplegia after TAAA repair and died on the third post-operative day from multi-organ failure. In this case the total spinal ischaemic time was 120 min and the distal aortic perfusion pressure was <50 mmHg. No cardiac, cerebral, renal, hepatic or bleeding complications were recorded in the remaining six patients. CONCLUSION: Our early experience with MECC during TAAA repair showed that it is feasible for distal aortic spinal and visceral selective perfusion. Further large clinical trials are required to determine the efficacy of this technique.

Cerebral MR venography of transverse sinuses in subjects with normal CSF pressure.

Flow artifacts or anatomic variants of venous sinuses often make MR venography (MRV) interpretation difficult. The authors investigated cerebral MRV in 111 subjects with normal CSF pressure to identify the most common flow abnormalities of transverse sinuses (TS). Disturbance of venous outflow in one transverse sinus was commonly observed in 30% of subjects whereas flow abnormalities of both TS occurred in 2 of 111 individuals. Subjects with flow gaps in both TS should undergo lumbar puncture to exclude increased CSF pressure.