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 Non Atherosclerotic Vascular Diseases

William L. Smead, M.D., R. Eugene Zierler, M.D.


  • Systemic Vasculitis
  • Giant Cell Arteritis
  • Takayasu's Disease
  • Radiation Induced Arterial Disease
  • Arterial Infections
  • Adventitial Cystic Disease
  • Popliteal Entrapment Syndrome
  • Buerger's Disease
  • Congenital Problems
  • Coarctation
  • Persistent Sciatic Artery
  • Aberrant Subclavian Artery
  • Arteriopathies
  • Marfan's Syndrome
  • Ehlers- Danlos Syndrome
  • Arterial Magna Syndrome
  • Cystic Medical Necrosis
  • Behcet's Disease
  • Homocystinuria
  • Intra-Arterial Drug Induced Injury

I. Immune Arteritis

1. To understand the basic pathologic mechanisms of vascular injury in the immune arteritis syndromes.

2. To define a classification system for the vasculitides on the basis of clinical findings, pathology, and prognosis in a way that assists in the diagnosis and management of these patients, recognizing the considerable overlap that exists among these diseases. This classification system would include the systemic necrotizing vasculitides , the hypersensitivity vasculitides , giant cell arteritis , and a diverse miscellaneous group.

3. To recognize the clinical problems which should alert the physician to consider a systemic vasculitis , particularly ischemic symptoms involving multiple organ systems in patients under 55 years of age, involving organs and limbs in distributions not typical for atherosclerosis, and occurring at an unusually accelerated pace.

4. To understand the basic clinical laboratory tests useful in the diagnosis and management of vasculitis . Diagnostic tests range from routine evaluations to more specific tests for defining the immunophysiology . Management requires testing to define the current inflammatory status as well as specific measurements of organ function.

5. To understand the role of arteriography and the characteristic findings in the arteritis syndromes of surgical significance.

6. To recognize the role of tissue biopsy in establishing a firm diagnosis.

7. To recognize the clinical presentation of polyarteritis nodosa , the laboratory and pathologic features of the disease which establish the diagnosis, and the complications of the disease with surgical significance.

8. To be familiar with the small vessel complications of hypersensitivity angiitis , a large category of vasculitides with a wide variety of etiologies including infection, drug and chemical allergies, connective tissue diseases, neoplasm, Henoch-Schönlein purpura , serum sickness, cryoglobulinemia , and a large miscellaneous category represented by chronic active hepatitis, primary biliary cirrhosis, inflammatory bowel disease, and intestinal bypass surgery. To understand the medical and surgical management of ischemic complications of these disorders.

9. To recognize the giant cell arteritis group of diseases which includes temporal arteritis and Takayasu-Onishi disease. To understand the distinctive arteriographic patterns of these disorders and the medical and surgical treatment strategies.

10. To be familiar with the miscellaneous vasculitis syndromes of surgical significance, including Kawasaki 's syndrome, Behcet's disease, Cogan's syndrome, and Buerger's disease.

II. Fibromuscular Dysplasia

1. To understand the pathologic classification of fibromuscular dysplasia : intimal fibroplasia , medial fibroplasia , medial hyperplasia, and perimedial dysplasia .

2. To recognize the vascular beds most frequently affected by this disorder (renal, cerebrovascular, mesenteric, and aortoiliac arteries) and the symptoms with which patients most frequently present.

3. To recognize the arteriographic patterns distinguishing each of the types of fibromuscular disease from each other and atherosclerosis.

4. To understand the natural history of fibromuscular disease in its various locations and its impact on clinical decision making.

5. To understand the various treatment options available including endovascular techniques and surgical bypass.

III. Adventitial Cystic Disease

1. To understand this rare condition producing arterial stenosis or occlusion in young patients, its clinical presentation, arteriographic features, operative findings, and management options.

IV. Popliteal Intrapment Syndromes

1. To understand the clinical presentation of this congenital anomaly predominantly affecting young men, its characteristic noninvasive vascular laboratory and arteriographic findings (provocative testing), and the available treatment alternatives.

2. To be familiar with the various anatomic variants which produce the abnormal relationship between the popliteal artery and the medial head of the gastrocnemius muscle.

3. To recognize the characteristic findings suggesting the adductor canal syndrome in which the junction of the superficial femoral and popliteal arteries is compressed by the tendinous insertion of the adductor magnus muscle at Hunter's canal.

V. Compartment Syndromes

1. To understand the multiple etiologies of compartment syndromes which have in common the production of sufficient compartmental pressure to compromise blood flow to the tissues within it, conditions which decrease compartmental volume or increase compartmental content or provide excessive external pressure.

2. To recognize those clinical situations in which compartment syndrome is more likely to develop complicating vascular injury or disease: prolonged ischemia, coexistent shock, preoperative neurologic deficits, pre-or intraoperative edema, combined arterial and venous injury, or concomitant crush injury.

3. To recognize the symptoms and signs of elevated compartment pressure and the tests available to confirm the diagnosis.

4. To understand the indications for fasciotomy and the surgical techniques available.

5. To understand the medical management of established rhabdomyolysis .

VI. Congenital Arterial Conditions

1. To be familiar with the various types of abdominal coarctations and their clinical presentations and natural history.

2. To understand the role of arteriography in the diagnosis of the problem and the planning of surgical treatment of abdominal coarctation .

3. To be familiar with the surgical options for repair and renal revascularization in patients with abdominal coarctation .

4. To recognize the arteriographic findings in patients with a persistent sciatic artery and the potential surgical implications.

VII. Diseases of the Arterial Media

1. To understand the pathologic changes of cystic medial necrosis which result in the clinical problems of aortic dissection, spontaneous rupture, and aneurysm formation.

2. To recognize the classic abnormalities associated with Marfan's syndrome and the typical cardiovascular complications.

3. To understand the natural history of Marfan's syndrome and the management options available to treat these cardiovascular problems.

4. To recognize the characteristic abnormalities in patients with Ehlers- Danlos syndrome and the issues of surgical significance including aneurysm formation, dissection, and spontaneous rupture.

5. To be familiar with the vascular changes in patients with pseudoxanthoma elasticum , arterial stenosis /occlusion and hypertension.

6. To recognize the changes associated with arteria magna syndrome and the role of arteriography in diagnosis, treatment , and patient follow-up.

VIII. Errors in Homocysteine Metabolism

1. To understand the inborn error of metabolism that produces homocysteinuria and the associated multiple abnormalities including mental retardation, lens ectopia , rapidly progressive premature atherosclerosis, and thromboembolic disorders.

2. To be familiar with the heterozygous trait which results in homocysteinemia and premature atherosclerosis, potentially ameliorated by treatment with folic acid, pyridoxine, and vitamin B 12 .

IX. Hyperviscosity Syndromes

1. To understand the myeloproliferative disorders and serum protein abnormalities that result in arterial or venous thromboembolism .

X. Arterial Infections

1. To recognize the symptoms and signs of arterial infections and the most common responsible pathogens.

2. To understand the etiologies of arterial infection including bacterial endocarditis , mycotic or infected aneurysms, drug abuse, iatrogenic contamination, and contiguity to adjacent infection.

3. To recognize the most effective techniques for obtaining positive cultures on which to base antibiotic treatment in patients with arterial infections.

4. To be familiar with the principles and treatment strategies for the management of arterial infection.

XI. Vasospastic Disoders

1. To understand the classification of cold sensitivity of the Raynaud type ( Raynaud's disease and Raynaud's phenomenon).

2. To recognize the common clinical presentations of vasospasm due to cold sensitivity.

3. To be familiar with the noninvasive diagnostic evaluation of digital ischemia and vasospasm.

4. To understand the features of uncommon vasospastic disorders, including livedo reticularis , acrocyanosis , and erythromelalgia .

5. To be familiar with the various treatment approaches to primary and secondary vasospasm.


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Posted June 2010