Raynaud Phenomenon and Other Vasospastic Disorders.

Vasospastic disorders are prevalent in the general population and can affect individuals of any age. Primary (or idiopathic) vasospastic disorders often have a benign course; treatment focuses on the control of symptoms. Secondary vasospastic disorders occur owing to an underlying condition and have an increased risk of complications, including tissue loss and digital ulcerations; treatment should focus on the underlying condition. In this review, we discuss the pathophysiology, clinical presentation, diagnosis, and management of vasospastic disorders, including Raynaud syndrome, acrocyanosis, livedo reticularis, and pernio.

Secondary Raynaud's phenomenon is associated with microvascular peripheral endothelial dysfunction.

Previous studies in patients with Raynaud's phenomenon (RP) have found an association between microvascular abnormalities assessed by nail fold capillaroscopy and macrovascular peripheral endothelial dysfunction (PED), but the association between RP and nitric oxide related (NO) microvascular PED is not yet established. We performed a retrospective cross-sectional analysis of patients who were referred to Mayo Clinic between 2006 and 2014 for routine cardiovascular evaluation and who underwent evaluation of Reactive Hyperemia Peripheral Arterial Tonometry (index <2 consistent with PED). Identification of the presence of RP was determined by retrospective chart review. Six hundred sixty six individuals were included in this study (mean age 51.9 ± 13.5 years, 411 (61.3%) women), 637 (95.1%) individuals did not have RP (control group), and 29 (4.3%) had secondary RP. Only 4 patients had primary RP and were thus excluded from the final analyses. In a multivariate analysis adjusting for age, sex, smoking status, and use of statins we found a significant association between secondary RP and microvascular PED in all patients (Odds ratio: 2.45; 95% confidence interval 1.13-5.34; P = 0.0236) that remained significant in women after stratifying by sex. Secondary RP is associated with microvascular PED, detected using a non-invasive NO-dependent method. Early detection of microvascular PED could help in identifying individuals with secondary RP who are at risk for developing connective tissue disease as well as CVD.

Diagnosis of hypothenar hammer syndrome in a patient with acute ulnar artery occlusion.

A 56-year-old truck driver with a history of tobacco use presented with acute onset digital ischaemia in the ulnar distribution of his dominant hand, associated with severe pain. Occupational exposures included extensive manual labour and prolonged vibratory stimuli. Workup with Doppler and angiography confirmed the diagnosis of hypothenar hammer syndrome (HHS). After the failure of medical management, he underwent ulnar artery thrombectomy with reconstruction and arterial bypass grafting. His pain improved significantly postsurgically, and he was able to return to a normal routine. This case illustrates the classic presentation, examination, imaging findings and management options of HHS. HHS should be considered in patients with digital ischaemia and associated occupational exposures. Diagnosing the condition appropriately allows for optimal management, aiming at minimising symptoms and maximising quality of life.

Digital obstructive arterial disease can be detected by laser Doppler measurements with high sensitivity and specificity.

OBJECTIVE: This study was conducted to determine the sensitivity and specificity of laser Doppler flowmetry (LDF) measurements for digital obstructive arterial disease (DOAD) using angiography as the reference standard and to compare the accuracy of different classical tests used to assess DOAD. Diagnosis of vascular abnormalities at the digital level is challenging. Angiography is the gold standard for assessment of DOAD but is invasive and expensive to perform. METHODS: We performed a retrospective analysis of consecutive patients referred at Mayo Clinic (Rochester, Minn) for upper extremity arterial assessment during a 27-month period. Finger-brachial index, skin blood flow (in arbitrary units [a.u.]), and skin temperature (in degrees Celsius) were recorded in each digit on the pulp at baseline and after a thermal challenge test (hand placed in a thermal box at 47.0°C for 15 minutes). Angiogram analysis was blinded and performed by a radiologist using a vascularization scale ranging from 0 (no vessel) to 4 (normal). The receiver operating characteristic curve was used to define a specific cutoff point to detect DOAD. Twenty-two patients had LDF measurements and complete angiograms. RESULTS: A total of 185 digits were analyzed because some patients had only analysis of one hand. The best area under the curve (AUC) was 0.98 (range, 0.94-0.99) for postwarming skin blood flow, with a cutoff point of ≤206 a.u. This AUC was statistically different from AUCs of all the other tests (P < .01). Sensitivity and specificity were 93% (95% confidence interval, 85%-97%) and 96% (95% confidence interval, 90%-99%), respectively. CONCLUSIONS: LDF combined with a thermal challenge is highly accurate, safe, and noninvasive means to detect DOAD.

May-Thurner syndrome associated with Klippel-Trenaunay syndrome.

We present an unusual case of a 23-year-old man who had symptomatic lower extremity varicosities that have been present since birth. He was complaining of pain and swelling of several years duration. Evaluation revealed Klippel-Trenaunay syndrome with associated compression of the left common iliac vein by the overriding left common iliac artery (May-Thurner syndrome). The patient was treated with left common iliac vein stenting followed by high ligation and inversion stripping of his grossly incompetent left small saphenous vein, stripping of his aberrant left lateral embryonic veins, and also by multiple stab phlebectomies, with excellent early result.

Lumbar artery pseudoaneurysm caused by a Gunther Tulip inferior vena cava filter.

Inferior vena cava (IVC) filters are widely used to decrease the risk of pulmonary embolism in patients with contraindications to anticoagulation. Complications include local hematoma, access site deep venous thrombosis (DVT), filter migration and embolization, leg penetration through the IVC wall, IVC occlusion, and filter fracture with embolization. Other rare complications include leg penetration into adjacent organs including duodenum and ureter. Lumbar artery pseudoaneurysms are rare and may be spontaneous, iatrogenic, or traumatic. To date, there have been 3 case reports of lumbar artery pseudoaneurysms caused by IVC filters. We present an additional case of a lumbar artery pseudoaneurysm caused by a Gunther Tulip IVC filter treated successfully with selective embolization.

External iliac vein thrombosis in an athletic cyclist with a history of external iliac artery endofibrosis and thrombosis.

External iliac artery endofibrosis describes an intimal subendothelial fibrosis leading to wall thickening and stenosis that has been described in high-performance athletes. There are anatomical, mechanical, and probably metabolic factors that may contribute to this pathology. Ankle-brachial index (ABI) measurement with exercise testing, duplex ultrasound, computed tomography (CT) or magnetic resonance (MR) angiogram, and ultimately arteriography help to make the diagnosis. Management can be conservative, but most cases require surgical intervention. External iliac vein stenosis and thrombosis in cyclists has rarely been described in the literature. We report a case of extensive left lower limb deep venous thrombosis (DVT) including the external iliac vein diagnosed in a 57-year-old athletic cyclist with a history of external iliac artery thrombosis.

Índice de resistividade renal como preditor da revascularização renal para hipertensão renovascular / Renal resistance index predicting outcome of renal revascularization for renovascular hypertension

FUNDAMENTO: A estenose arterial renal (EAR) é uma causa potencialmente reversível de hipertensão arterial sistêmica (HAS) e nefropatia isquêmica. Apesar da revascularização bem sucedida, nem todos os pacientes (pt) apresentam melhora clínica e alguns podem piorar. OBJETIVO: O presente estudo se destina a avaliar o valor do índice de resistividade renal (IR) como preditor dos efeitos da revascularização renal. MÉTODOS: Entre janeiro de 1998 e fevereiro de 2001, 2.933 pacientes foram submetidos ao duplex ultrassom renal. 106 desses pacientes apresentaram EAR significativa e foram submetidos a angiografia e revascularização renal. A pressão arterial (PA) foi medida antes e depois da intervenção, em intervalos de até 2 anos e as medicações prescritas foram registradas. Antes da revascularização, o IR foi medido em 3 locais do rim, sendo obtida uma média dessas medições. RESULTADOS: Dos 106 pacientes, 81 tiveram IR<80 e 25 RI>80. A EAR foi corrigida somente por angioplastia (PTA) em 25 pts, PTA + stent em 56 pts e cirurgicamente em 25 pts. Dos pacientes que se beneficiaram da revascularização renal; 57 dos 81 pacientes com IR <80 apresentaram melhora em comparação a 5 de 25 com IR > 80. Usando um modelo de regressão logística múltipla, o IR esteve significativamente associado à evolução da PA (p = 0,001), ajustado de acordo com os efeitos da idade, sexo, PAS, PAD, duração da hipertensão, o tipo de revascularização, número de fármacos em uso, nível de creatinina, presença de diabete melito, hipercolesterolemia, volume sistólico, doença arterial periférica e coronariana e tamanho renal (OR 99,6-95 por centoCI para OR 6,1-1.621,2). CONCLUSÃO: A resistividade intrarrenal arterial, medida por duplex ultrassom, desempenha um papel importante na predição dos efeitos pós revascularização renal para EAR.

BACKGROUND: Renal artery stenosis (RAS) is a potentially correctable cause of hypertension and ischemic nephropathy. Despite successful renal revascularization, not all patients (pt) overcome it and some get worse. OBJECTIVE: This study was designed to assess the value of renal resistance index (RI) in predicting the outcome of renal revascularization. METHODS: Between Jan 1998 and Feb 2001, 2,933 pts were referred to renal duplex ultrasound. 106 out of these had significant RAS and underwent angiography and renal revascularization. Arterial blood pressure (BP) was measured before and after the intervention, at intervals of up to 2 years and medications recorded. Prior to revascularization, RI was measured at 3 sites of each kidney and averaged. RESULTS: Out of the 106 patients, 81 had RI<80 and 25 RI>80. RAS was corrected with angioplasty (PTA) alone in 25 pts, PTA + stent in 56 pts and corrected by surgery in 25 pts. Of patients who benefited from renal revascularization; 57 of the 81 patients with RI <80 improved as compared to 5 of 25 with RI>80. Using a multiple logistic regression model, RI was significantly associated with BP outcome (p=0.001), adjusted for the effects of age, sex, SBP, DBP, duration of hypertension, type of revascularization, number of medication in use, creatinine level, presence of diabetes mellitus, hypercholesterolemia, stroke, peripheral and coronary artery disease and kidney size (OR 99.6 - 95 percentCI for OR 6.1 to 1,621.2). CONCLUSION: Intrarenal arterial resistance measured by duplex ultrasound plays an important role in predicting BP outcome after renal revascularization for RAS.

[Renal resistance index predicting outcome of renal revascularization for renovascular hypertension]. / Indice de resistividade renal como preditor da revascularização renal para hipertensão renovascular.

BACKGROUND: Renal artery stenosis (RAS) is a potentially correctable cause of hypertension and ischemic nephropathy. Despite successful renal revascularization, not all patients (pt) overcome it and some get worse. OBJECTIVE: This study was designed to assess the value of renal resistance index (RI) in predicting the outcome of renal revascularization. METHODS: Between Jan 1998 and Feb 2001, 2,933 pts were referred to renal duplex ultrasound. 106 out of these had significant RAS and underwent angiography and renal revascularization. Arterial blood pressure (BP) was measured before and after the intervention, at intervals of up to 2 years and medications recorded. Prior to revascularization, RI was measured at 3 sites of each kidney and averaged. RESULTS: Out of the 106 patients, 81 had RI<80 and 25 RI>80. RAS was corrected with angioplasty (PTA) alone in 25 pts, PTA + stent in 56 pts and corrected by surgery in 25 pts. Of patients who benefited from renal revascularization; 57 of the 81 patients with RI <80 improved as compared to 5 of 25 with RI>80. Using a multiple logistic regression model, RI was significantly associated with BP outcome (p=0.001), adjusted for the effects of age, sex, SBP, DBP, duration of hypertension, type of revascularization, number of medication in use, creatinine level, presence of diabetes mellitus, hypercholesterolemia, stroke, peripheral and coronary artery disease and kidney size (OR 99.6 - 95%CI for OR 6.1 to 1,621.2). CONCLUSION: Intrarenal arterial resistance measured by duplex ultrasound plays an important role in predicting BP outcome after renal revascularization for RAS.

Patient with both Wilkie syndrome and nutcracker syndrome.

Wilkie syndrome or superior mesenteric artery syndrome is a rare cause of duodenal obstruction in pediatric and adult age groups. Its manifestations are due to compression of the third portion of the duodenum between the aorta and the superior mesenteric artery. We present one case of a 29-year-old female with a recent history of epigastric pain and severe weight loss due to duodenal obstruction from superior mesenteric syndrome. She was treated conservatively with jejunostomy tube feeding to regain loss of weight. Our case is unique in that the patient has also nutcracker syndrome.

Uncommon arteriopathies: what the vascular surgeon needs to know.

Uncommon arteriopathies encompass a diverse range of diseases, including inherited collagen vascular disorders such as Marfan syndrome, Ehlers-Danlos Type IV, and pseudoxanthoma elasticum; vasculitides, including Takayasu's arteritis, extracranial giant cell arteritis, and Behçet's disease; neurofibromatosis type 1; intimal fibromuscular dysplasia; unusual bacterial and viral infections; and drug-induced arteriopathies. Patients with uncommon arterial disorders may present to the vascular surgeon with common surgical problems, including intermittent claudication, renovascular hypertension, Raynaud's phenomenon, and aneurysmal disease. However, the disease manifestations, expected course, and outcomes may be much different than more common arterial disorders and this can have important surgical implications. This review centers around several interesting cases and the differential diagnoses that should be considered when encountering an unusual clinical presentation. Reference is made to the literature for diagnostic criteria, clinical pearls, and how to avoid pitfalls in the evaluation and management of patients with unusual arteriopathies.