Abdominal Visceral Arteries Research Articles

Laparoscopic median arcuate ligament section for median arcuate ligament compression syndrome initially detected as splenic infarction: a case report

BackgroundMedian arcuate ligament compression syndrome (MALS) causes upper abdominal pain and at times hemodynamic abnormalities in the pancreaticoduodenal region.Herein, we present a case of a 70 year-old man, initially diagnosed with splenic infarction and was successfully treated laparoscopically.Case presentationA 70-year-old man with abdominal pain admitted to our hospital. Abdominal-enhanced computed tomography revealed a poorly contrasted area in the spleen and stenosis at the root of the celiac artery. Arterial dilatation was observed around the pancreaticoduodenal arcade, however, no obvious aneurysm formation or arterial dissection was observed. Abdominal-enhanced magnetic resonance imaging indicated the disappearance of the flow void at the root of the celiac artery. The patient had no history of atrial fibrillation and was diagnosed with splenic infarction due to median arcuate ligament compression syndrome. We performed a laparoscopic median arcuate ligament section with five ports. Intraoperative ultrasonography showed a retrograde blood flow in the common hepatic artery and the celiac artery. After releasing the compression, the antegrade blood flow from the celiac artery to the splenic artery, and the common hepatic artery were visualized using intraoperative ultrasonography. The postoperative course of the patient was uneventful, and he was discharged on postoperative day 9. Postoperative computed tomography a month after surgery revealed no residual stenosis of the celiac artery or dilation of the pancreaticoduodenal arcade. Furthermore, the poorly contrasted area of the spleen improved.ConclusionsReports indicate that hemodynamic changes in the abdominal visceral arteries due to median arcuate ligament compression are related to the formation of pancreaticoduodenal aneurysms. In this case, median arcuate ligament compression syndrome caused splenic infarction by reducing blood flow to the splenic artery.

Vascular Behçet syndrome: from pathogenesis to treatment.

Behçet syndrome is a rare, chronic inflammatory disease of unknown aetiopathogenesis, most commonly presenting with mucocutaneous and ocular manifestations. Vascular involvement, most frequently superficialvein and deepvein thrombosis, can occur in up to 50% of patients with Behçet syndrome. Venous thrombosis at atypical sites (inferior and superior vena cava, suprahepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses and right atrium and/or ventricle) and arterial involvement (mostly in situ thrombosis and aneurysms of the pulmonary arteries, as well as aneurysms of the abdominal aorta, and peripheral and visceral arteries) are also unique features of Behçet syndrome. Behçet syndrome is considered a natural model of inflammation-induced thrombosis in humans, with an impaired immune-inflammatory response rather than traditional cardiovascular risk factors contributing to thrombogenesis. Specifically, neutrophil hyperactivation and neutrophil-mediated mechanisms of damage directly promote endothelial dysfunction, platelet activation and thrombogenesis in Behçet syndrome. This unusual pathogenesis directly determines the treatment approach, which relies mostly on immunosuppressants rather than anticoagulants for treatment of thrombosis and for secondary prevention. This Review discusses the main histopathological, pathogenetic and clinical aspects of vascular Behçet syndrome, addressing their implications for therapeutic management. Future perspectives in terms of pathogenetic studies, disease monitoring and treatment strategies are also discussed.

A Novel Solution for Distal Dilation of Chronic Dissection After Repair Involving Visceral Branches: The Road Block Strategy.

AimNotwithstanding that unprecedented endovascular progress has been achieved in recent years, it remains unclear what is the best strategy to preserve the blood perfusion of abdominal visceral arteries and promote positive aortic remodeling in patients with distal dilatation of chronic aortic dissection in abdominal visceral part (CADAV) after aortic repair. The present study developed a Road Block Strategy (RBS) to solve this conundrum.Methods and ResultsThis prospective single-center clinical study included patients suffering from symptomatic distal dilatation of CADAV after aortic repair treated with RBS from January 2015 to December 2019 and followed up regularly for at least 2 years. Stent grafts were implanted first to cover distal tears and expand the true lumen. Device embolization was performed to induce proximal and distal segmental false lumen thrombosis (FLT) apart from the level of the ostia of vital branches. Successful RBS was performed in 13 patients. Significant differences were found in maximum true lumen diameter (p < 0.05), blood flow area in false lumen (FL) (p < 0.001), and the ratio of blood lumen to FL area (p < 0.05) between the pre-procedure and the latest follow-up results. No aortic rupture, vital branches occlusion, thoracic and abdominal pain, or death occurred during hospitalization and follow-up.ConclusionsOur findings suggest that RBS is feasible in treating distal dilatation of chronic aortic dissection after prior proximal repair, inducing false lumen thrombosis, preventing deterioration of aortic dissection, and maintaining the patency of abdominal visceral arteries.

Surgical treatment of visceral and renal abdominal artery aneurysms of various etiology

Visceral artery aneurysms are detected when complications occur or accidentally during examination for other diseases gastrointestinal tract, aimed at determining the functional significance. Over the past 10 years, we have observed 31 patients with abdominal visceral artery aneurysms, 10 patients had renal aneurysms, 15 had splenic arteries, hepatic artery and pancreatoduodenal artery aneurysms – in one case. The combination of aneurysms – in 2 cases: in one case – the renal artery with the splenic artery, in the other – the renal artery and the abdominal trunk. The patients ranged in age from 24 to 78 years, the average age is 43±6.5. Previously, infection was considered the most common cause of splenic artery aneurysms in 10 cases, with most patients complaining of fever, abdominal pain and a pulsating mass in the abdomen. And two patients with splenic artery aneurysm noted abdominal trauma. 3 had atherosclerotic changes. The causes of renal artery aneurysms are atherosclerosis in 6 cases, fibromuscular dysplasia in 2 cases, and Nonspecific aortoarteritis in 2 cases. Also, atherosclerotic etiology is present in aneurysms of the external iliac arteries aneurysms. Reconstructions on hepatobiliary aneurysms were performed in 6 (20%) cases, in 5 cases - resection with end-to-end anastomosis and in 2 cases - resection of splenic artery aneurysm, resection of splenic artery aneurysms with autovenous patch, clipping of splenic artery aneurysms – in 2 cases (6.5%), endovascular techniques were applied in 18 cases (58%). In 2 cases, nephroectomy was performed due to the spread of the aneurysm into the kidney gate and the lack of conditions for reconstruction. In the immediate and long-term (after 6 months and 3 years) postoperative period, the preservation of organ function in reconstructive and ligating methods of surgical treatment is the same. Signs of ischemia and impairment in the immediate postoperative period were not observed with reconstructive and ligating methods of surgical treatment. The choice of treatment method depends on the severity of the patient’s condition, clinical manifestations, as well as the localization of the aneurysm and its morphology. Preference is given to endovascular techniques, which are not traumatic enough and provide a good long-term result. However, in each case, the patient’s management tactics are individual, the choice of the optimal method of treatment of visceral artery aneurysms is based on data from the entire spectrum of possible diagnostic manipulations.

Upper gastrointestinal lesions in patients with lower extremity peripheral artery disease: data from a retrospective analysis

Aim. To assess the prevalence and structure of upper gastrointestinal (GI) lesions, as well as the factors associated with their development, in patients with lower extremity peripheral artery disease (PAD).Material and methods. This retrospective analysis of medical records with lower extremity PAD includes data from 258 patients. All patients underwent multislice computed tomography angiography of abdominal aortic visceral branches and lower limb arteries, as well as esophagogastroduodenoscopy.Results. Endoscopic evidence of upper GI lesions was detected in 164 (63,6%) patients. In particular, gastroduodenal erosions and ulcers were observed in 57,8% of patients. All patients were divided into two groups: group I — 149 (57,8%) patients with gastroduodenal erosions and ulcers, group II — 109 (42,2%) patients without it. In group I patients, a higher incidence of mesenteric artery disease was recorded — 61,7 vs 43,1% (p=0,003). An independent factor associated with the development of gastroduodenal erosions and ulcers in patients with PAD is mesenteric artery disease (relative risk: 4,43; 95% confidence interval: 1,15-17,12 (p=0,031)).Conclusion. In patients with lower extremity PAD, upper GI diseases (63,6%) occur with a high frequency, while gastroduodenal erosions and ulcers was observed in 90,9% of them. The factors associated with the development of erosions and ulcers in patients with lower extremity PAD include mesenteric artery atherosclerosis.

Abdominal Aortic and Visceral Artery Aneurysms.

Abdominal aortic aneurysms account for nearly 9000 deaths annually, with ruptured abdominal aortic aneurysms being the thirteenth leading cause of death in the United States. Abdominal aortic aneurysms can be detected by screening, but a majority are detected incidentally. Visceral artery aneurysms are often discovered incidentally, and treatment is guided by symptoms, etiology, and size. A timely diagnosis and referral to a vascular specialist are essential for timely open or endovascular repair and to ensure successful patient outcomes.

A modified method of computed fluid dynamics simulation in abdominal aorta and visceral arteries

Purpose The flow velocity of visceral arteries was measured by 2D PCMRI to produce the patient-specific flow BC imposed on the outlets of visceral arteries in CFD simulation. This modified method aimed to improve the CFD accuracy in the abdominal aorta and visceral arteries. Methods A volunteer underwent non-contrast-enhanced MRA to scan the abdominal aorta and visceral arteries, and 2D PCMRI to obtain the flow velocity of the aforementioned vessels. The three-dimensional geometric model was reconstructed using the MRI scan data of the abdominal aorta and visceral arteries. The flow waveforms measured by 2D PCMRI were processed and then imposed on the aortic inlet and the outlets of all visceral arteries as the flow BC. The RCR parameters of the three elements Windkessel model were modulated and imposed on the aortic outlet. CFD simulation was run in the open-source software: svSolver. The same volunteer underwent 4D flow MRI to compare the flow field with those extracted from CFD results. Results Four specific time points in a cardiac cycle and three cross-sectional planes of aorta were selected to analyze the flow field, pressure and wall shear stress (WSS) from CFD. The flow waveforms and streamlines of CFD agreed with those of 4D flow MRI. The pressure waveforms, pressure distribution and WSS distribution from CFD conformed with the physiological condition of human body. Conclusion These results suggest this modified CFD method may yield reasonable flow field, pressure and WSS in the abdominal aorta and visceral arteries.

Evaluation of isolated abdominal visceral artery dissection with multi-scale spiral computed tomography: a retrospective case series

BackgroundTo evaluate the role of multi-slice spiral computed tomography (MSCT) angiography in the diagnosis of spontaneous isolated visceral artery dissection (SIVAD).MethodsTwenty-seven patients with abdominal SIVAD were included in the study. The MSCT scans of the patients were subjected to various post-processing techniques to visualize the visceral artery wall. Clinical features including arterial dissection, thrombosis, dissection length, true/false lumen, and complications were recorded.ResultsType I, IIa, and IIb SIVADs were observed in 11, 6, and 10 patients, respectively. Superior mesenteric artery (SMA) dissection was the most common (n = 16), followed by abdominal aortic dissection (n = 6), splenic artery dissection (n = 2), renal artery dissection (n = 2), and splenic artery dissection (n = 1). One patient with SMA dissection suffered small intestine ischemia, 1 with splenic artery dissection had splenic infarction, and 1 patient with left renal artery dissection experienced renal infarction. The false lumen was bigger than the true lumen in 20 patients, with 9 patients having thrombus. The true lumen was bigger than the false lumen in 7 patients.ConclusionsMSCT angiography is a valuable technique in the diagnosis and treatment of patients with SIVAD. Patients with abdominal pain suspected due to SIVAD should be examined with MSCT angiography for early detection of SIVAD.

Celiac trunk compression syndrome: modern ideas about the problem (review of literature)

Celiac trunk compression syndrome is a symptom complex arising from compression of the celiac trunk by the median arcuate ligament, crura of diaphragm or ganglion tissue of the celiac plexus. The main clinical manifestations of this pathological condition are abdominal pain syndrome, indigestion and weight loss. The disease is detected in 0.4–1 % of people in the population, more often in young people. The pathophysiological mechanisms of the development of celiac trunk compression syndrome remain not fully understood, but the key reason for its development is ischemia of the digestive system. The main instrumental methods for diagnosing compression of the celiac trunk are ultrasound duplex scanning of the abdominal aorta and visceral arteries and spiral computed angiography. The only effective treatment for celiac trunk compression syndrome is surgery. Currently, both classical techniques and various minimally invasive technologies are used to decompress the celiac trunk. The advantages of open access include good visual and manual control of anatomical structures, the possibility of eliminating complications, changing the tactics of surgical intervention, if necessary, performing vascular reconstruction. The advantage of minimally invasive surgical technologies is minimal trauma to the abdominal wall tissues, minor postoperative pain syndrome, early rehabilitation, a low risk of developing cusp, wound infection and hernial defects. Regardless of the methodology, surgical treatment can achieve a positive effect in 75–90 % of patients with celiac trunk compression syndrome. These are the modern concepts of epidemiology, pathogenesis, clinical manifestations, diagnosis and methods of treatment of this pathological condition.

Ultrashort echo time time-spatial labeling inversion pulse magnetic resonance angiography with denoising deep learning reconstruction for the assessment of abdominal visceral arteries.

Current contrast-enhanced magnetic resonance angiography (MRA) and non-contrast-enhanced balanced steady-state free precession (bSSFP) MRA cause susceptibility artifacts from metallic devices in assessing endovascular visceral-artery interventions. The aims of this study are to investigate and compare image quality (IQ) and susceptibility artifacts of three-dimensional (3D) ultrashort echo time (UTE) time-spatial labeling inversion pulse (Time-SLIP) with those of 3D bSSFP Time-SLIP and to assess denoising deep learning reconstruction (dDLR) for the improvement of the signal-to-noise ratio (SNR) in 3D UTE with sparse sampling in phantoms and human subjects. This is a prospective type of study. Pulsatile glycerin-water flow phantom with platinum-tungsten-alloy coil, stainless-steel, nitinol, and cobalt-alloy stents were used. Ten healthy volunteers (seven males) and three patients (two males) were included in this study. 3D UTE Time-SLIP and 3D bSSFP Time-SLIP at 3T were used. The phantom-based study compared the signal-intensity ratio of the device levels (SRdevice ) and distal segments (SRdistal ) to the proximal segments. The volunteer-based study measured SNR, contrast ratio (CR), and IQ. The patient study evaluated local artifacts from metallic devices. Statistical tests included paired t-tests, Wilcoxon-signed rank tests, and Kruskal-Wallis tests. In the phantom-based study, SRdevice was small with UTE Time-SLIP, except the stainless-steel stent. SRdistal was greater (49.1%-90.4%) on bSSFP images than UTE images (-11.1% to 9.6%). Among volunteers, dDLR in UTE images improved SNR (p < 0.05) and IQ (p < 0.05), but CR was unaffected. UTE Time-SLIP showed inferior SNR and IQ than bSSFP Time-SLIP in images with and without dDLR (p < 0.05 for each). However, among patients, UTE Time-SLIP showed reduced metal artifacts compared to bSSFP Time-SLIP. Irrespective of the lower SNR and IQ of 3D UTE Time-SLIP than those of 3D bSSFP Time-SLIP, the former appeared to better depict flow after stenting or coiling. This indicates the potential of 3D UTE Time-SLIP to provide suitable diagnostic images of target vessels. dDLR improved SNR with reducing artifacts related to radial sampling, while maintaining the contrast. LEVEL OF EVIDENCE: 2. TECHNICAL EFFICACY STAGE: 2.

Thoracic Endovascular Aortic Repair for Type B Aortic Dissection Associated with Retrograde Type A Intramural Hematoma

PurposeTo evaluate feasibility and efficacy of thoracic endovascular aortic repair (TEVAR) for type B aortic dissection (TBAD) associated with retrograde type A intramural hematoma (IMH). Materials and MethodsFrom April 2013 to January 2017, 15 consecutive patients with TBAD associated with retrograde type A IMH who underwent TEVAR were reviewed retrospectively. There was no cardiac tamponade, aortic regurgitation, involvement of coronary artery, or sign of cerebral ischemia in these patients. Enhanced CT was used in 4 patients to diagnose malperfusion of abdominal visceral arteries or lower extremity artery and underwent emergent TEVAR. For the remaining 11 patients, repeated enhanced CT after initial medical treatment within 24 hours from onset of pain showed expansion of IMH in 8 patients or presence of periaortic hematoma in 3 patients. Delayed TEVAR was scheduled for these cases. ResultsSuccessful deployment of the stent graft was achieved in all patients. There were no severe postoperative complications, such as retrograde type A aortic dissection or aortic rupture. Sudden death occurred in 1 patient 3 months after the procedure. Thrombosis of the false lumen, shrinkage of the diameter of the aorta, and complete absorption of the IMH were observed in the remaining patients at a mean follow-up of 19.8 months ± 6.57. ConclusionsTEVAR for treatment of TBAD with retrograde type A IMH is feasible and effective. It represents a treatment option for patients with TBAD associated with type A IMH with a proximal entry tear located in the descending aorta.

Clinical Features of Spontaneous Isolated Dissection of Abdominal Visceral Arteries.

BackgroundSpontaneous isolated dissection of abdominal visceral arteries without aortic dissection is rare and its pathology and prognosis are not yet clear; therefore, therapeutic strategies for this disease have not been established. The present multi-institution investigational study analyzed the clinical features of patients with spontaneous isolated dissection of abdominal visceral arteries.MethodsA total of 36 patients diagnosed as spontaneous isolated dissection of abdominal visceral arteries from January 2010 to October 2016 were enrolled. The medical data of the patients were retrospectively reviewed. Imaging characteristics were evaluated. Spontaneous isolated dissection of abdominal visceral arteries was detected on upper abdominal computed tomography examination in almost patients, and was detected on magnetic resonance imaging in one patient.ResultsOf the 36 cases, 26 cases involved the superior mesenteric artery dissection, nine involved the celiac artery, two involved the splenic artery, one involved the common hepatic artery, one involved the gastroduodenal artery and one involved the left gastric artery. Among the 36 patients, 20 had hypertension and 14 were current smokers. Additionally, only one patient had diabetes and four patients had dyslipidemia. Moreover, 32 cases complained of pain including abdominal pain and back pain, one had cough and three had no symptoms. Of the 36 patients, 34 cases (94.4%) were treated conservatively, and two (5.6%) required intravascular treatment. All patients were discharged without complications.ConclusionsOur findings indicate that hypertension and smoking might be closely involved in the pathogenesis of spontaneous isolated dissection of abdominal visceral arteries, whereas dyslipidemia and diabetes might be less involved. Additionally, few asymptomatic patients were accidentally diagnosed, indicating that the absence of symptoms cannot be used to rule out the presence of this disease. Randomized clinical trials cannot be performed because a considerable number of cases are required. Therefore, detailed descriptions of clinical features, as provided in our report, are important.

A New technical solution in custom-made aortic stent graft for complex aortic aneurysms

Background: The endovascular treatment of aortic aneurysm with complex anatomy, requires adjunctive procedures for anchoring, sealing and visceral arteries perfusion, with increase of morbidity and mortality. A new custom-made Aortic Stent Graft (ASG) platform, the Dominus (Braile Biomedica®, Sao Jose do Rio Preto, Brasil) modified on the design of Endovsmarts.r.l.™ (Bergamo, Italy), based on the principle of the radial force modulation, is available in the market, simplifying the procedure. The study focuses on device design, technical features and results. Methods: We treated 4 patients: 2 with abdominal aortic aneurysm and 2 with thoraco-abdominal aortic aneurysm, previously rejected by the others manufacturers, due to at least one of the following anatomical complexities: 78° aortic arch angle, 30° descending thoracic aorta angle, 40 mm aortic diameter at the level of renal arteries, 90° alfa angle, 60° abdominal aortic angle, and 6 mm iliac access diameter. The primary endpoints were 30-day clinical and technical success. The follow-up was made by physical examination and computed tomography angiography at 1,3,6 months and then yearly. Results: The thirty-day clinical and technical success was 100%. There was not any neurologic event. The mean fluoroscopy time, contrast medium volume and, time of procedure, were respectively 17.2 min, 300 ml, and 228 min. In 1 case occurred the postoperative laceration of the external iliac artery, treated by a covered stent. Themean time of hospitalization was4.4 days. During the 16 months median follow-up the patients were well and the computed tomography angiography confirmed the exclusion of the aneurysms and supraortic and abdominal visceral arteries patency. Conclusions: In this preliminary experience, the new Dominus ASG platform allowed the effective endovascular exclusionof complex aortic aneurysms, otherwise un-treatable with the current available devices. More cases and longer follow-up are required for validating these encouraging outcomes.

Association Between Abdominal Visceral Artery Calcification and All-Cause Mortality-A Computerized Tomography Imaging-Based Longitudinal Follow-Up Study.

Arterial calcification in different arterial beds has been observed to be an independent predictor of mortality. The association of abdominal visceral artery calcium with all-cause mortality remains unexplored. Patients who had undergone contrast-enhanced computerized tomography (CT) imaging for routine assessment of peripheral arterial disease (PAD) were considered for this study. A novel calcium score (abdominal visceral arteries calcium [AVAC]) for the abdominal visceral arteries (celiac axis, superior mesenteric, and renal arteries) was calculated using a modified Agatston score. Cumulative AVAC was defined as sum total of the calcium score of above individual arteries. The primary outcome was all-cause mortality. The association of AVAC with all-cause mortality was assessed. Of the 134 consecutive patients, 89 were included for analysis. Median follow-up duration was 72 (47-91) months since CT imaging; 35 (39%) patients died during this period. Hypertension and cumulative AVAC score had a significant association with all-cause mortality (P < .05). Cumulative visceral abdominal artery calcification is associated with all-cause mortality in patients with PAD. Future prospective studies are warranted to investigate this relationship in PAD and other patient cohorts.

Ultrasound of type 4 Takayasu's arteritis with abdominal aorta and visceral artery stenosis

Ultrasound of type 4 Takayasu's arteritis with abdominal aorta and visceral artery stenosis

Clinical Outcomes of Total Endovascular Aneurysm Repair for Aortic Aneurysms Involving the Proximal Anastomotic Aneurysm following Initial Open Repair for Infrarenal Abdominal Aortic Aneurysm

To evaluate initial and midterm clinical outcomes of aortic aneurysms involving the proximal anastomotic aneurysm (AAPAAs) following initial open repair for infrarenal abdominal aortic aneurysm. Between July 2006 and August 2015, 24 patients underwent elective endovascular repair for the treatment of AAPAAs at our institution. AAPAA classification has been categorized as 3 types. Type I AAPAA is the most extensive, extending from the descending aorta to the prior proximal anastomosis as similar to Crawford type II or III thoracoabdominal aortic aneurysm. Type II AAPAA is limited to the aortic aneurysm below the diaphragm including the abdominal visceral arteries. Finally, similar to pararenal abdominal aortic aneurysm, type III AAPAA involves the renal origins, but does not extend to the celiac and superior mesenteric arteries. Total endovascular aneurysm repair (t-EVAR) consisted of fenestrated EVAR (f-EVAR), multibranched EVAR (t-Branch), and snorkel EVAR (s-EVAR) were performed for patients with high-risk open surgical repair. We retrospectively analyzed 24 cases, which were categorized with 3 types of AAPAA. F-EVAR, t-Branch, and s-EVAR for AAPAAs were performed in 15 patients (62.5%), 5patients (20.8%), and 4 patients (16.7%), respectively. Type I and type II AAPAA were identified in 13 patients (54.2%) and 7 patients (29.2%), and type III AAPAA was identified in 4 patients (16.7%). Technical success was 95.8%, and clinical success was 79.2% with t-EVAR. Spinal cord ischemia was identified in 2 patients (8.3%) of type I AAPAA, the 30-day mortality rate was 4.2% (n=1, type I AAPAA). Type II and III endoleaks occurred in 1 (4.2%, type III AAPAA) and 3 patients (12.5%, each case of type I, II, and III AAPAA), respectively. There was no open conversion or aneurysm rupture in the late follow-up period. The estimated overall survival rates of t-EVAR after 1 and 3years were 95.6% and 76.2%, respectively. Rates of freedom from aneurysm-related death and secondary intervention of t-EVAR at 3years were 90.1% and 89.7%, respectively. Finally, rates of target vessel patency at 1 and 3years were 95.3% and 88.8%, respectively. Our initial to midterm results of t-EVAR for the treatment of AAPAA were generally good with low rates of perioperative mortality and aneurysm-related death. However, more attentions should be paid for the treatment of type I AAPAA with high incidence of major adverse events.

The Epidemiologic and Clinical Findings of Patients with Buerger Disease

Buerger disease is a nonatherosclerotic peripheral arterial disease, which is mostly observed in young male smokers. Buerger disease is characterized by the observation of peripheral arterial occlusion by angiography. The condition may be caused by microembolization in the small-sized arteries of the distal extremities. Buerger disease is diagnosed based on the Shionoya's clinical diagnostic criteria, which include: (1) a history of smoking, (2) onset before the age of 50 years, (3) the presence of infrapopliteal arterial occlusions, (4) either upper limb involvement or phlebitis migrans, and (5) the absence of atherosclerotic risk factors other than smoking. Several studies have reported that oral bacterial infections (periodontitis) could activate the onset of Buerger disease. In this study, we report the epidemiologic and clinical manifestations of patients with Buerger disease. Fifty-eight patients who were surgically treated between July 1989 and June 2014 at Tokyo Medical and Dental University Hospital were enrolled in this study. All of the patients clinically diagnosed with Buerger disease based on Shionoya's clinical diagnostic criteria. Fifty-five male and 3 female patients were treated. The mean age of the patients was 48 years (range, 21-73 years). All of the patients were either smokers or had a history of smoking. The mean Brinkman index score was 780 (range, 150-1,640). Their mean age at the onset of Buerger disease was 38 years (range, 21-50). The arterial lesions extended to the femoral arteries in 25% of cases, to the iliac arteries in 8% and to the abdominal aorta and/or visceral arteries in 6% of cases. Upper limb involvement, including cyanosis, paleness, and gangrene, was obvious in 84% of patients, and phlebitis migrans was recognized in 34%. The lower extremities symptoms involved intermittent claudication in 23% of the patients, rest pain in 13% of the patients, and ulceration or gangrene in 64% of the patients. Fifteen patients had undergone surgical arterial reconstruction, 4 patients had received endovascular therapy, 33 patients had undergone lumbar sympathectomy and 8 patients had undergone thoracic sympathectomy. Twenty percent of the patients needed minor limb amputations, and 4% required major limb amputations. In the patients who were examined for their oral conditions, periodontitis corresponding to grades B (moderate periodontitis), C (severe periodontitis), and D (edentulous patients) was revealed in 31%, 56%, and 13% of the patients, respectively. More than half of the Buerger disease patients in this study were suffering from severe periodontitis. It is possible that not only the cessation of smoking, but also the improvement of periodontal care could improve the clinical symptoms related to Buerger disease.

Imaging findings in a distinct lethal inherited arteriopathy syndrome associated with a novel mutation in the FBLN4 gene.

We present the imaging findings of a newly identified lethal arteriopathy associated with a novel mutation in the gene encoding fibulin-4, occurring in a distinct community from southern India. A total of 31 children from a distinct population subgroup who presented with characteristic arterial dilatation and tortuosity were studied. All children except one belonged to unrelated families from an ethno-religious group (Muslim) from the northern coastal belt of southern India. CT angiography was performed in 30 children and contrast MRA in one. Impressive dilatation and elongation of ascending aorta, arch, descending aorta and main pulmonary arteries with characteristic narrowing of aortic isthmus were seen in all patients. Stenosis of arch branches, abdominal visceral branches and pulmonary artery branches was observed in 21 (68%), 23 (62.5%) and 20 (65%) patients respectively. Genetic studies revealed an identical mutation in exon 7 of the FBLN4 gene. On follow-up, 27 of them had died before the age of 3 years and only two children were alive after the age of 4 years. FBLN4-associated vasculopathy is a highly lethal disease characterized by severe aneurysmal dilatation of thoracic aorta, its branches and pulmonary arteries with stenoses at typical locations. • Homozygous mutations in exon 7 of the FBLN4 gene can produce lethal vasculopathy. • Fibulin-4 is a critical determinant in human elastogenesis. • Imaging findings can give a clue to underlying connective tissue disorders.

Sandwich technique reserving four abdominal visceral arteries in treatment of thoracoabdominal aortic aneurysm: a case report

Sandwich technique reserving four abdominal visceral arteries in treatment of thoracoabdominal aortic aneurysm: a case report

Managing Incidental Findings on Abdominal and Pelvic CT and MRI, Part 2: White Paper of the ACR Incidental Findings Committee II on Vascular Findings

This white paper describes vascular incidental findings found on CT and MRI of the abdomen and pelvis. Recommendations for management are included. This represents the second of 4 such papers from the ACR Incidental Findings Committee II, which used a consensus method based on repeated reviews and revisions and a collective review and interpretation of relevant literature. Topics include definitions and recommended management for abdominal aortic, iliac, splenic, renal, and visceral artery aneurysms. Other incidentally discovered aortic conditions, systemic venous anomalies, compression syndromes, abdominal venous thrombosis, and gonadal and pelvic venous conditions are also discussed. A table is provided for reference.