3NORMAL VESSELSArterial walls are thicker than veinsTo accommodate pulsatile flow and higher blood pressures.
4Structure of blood vessels Tunica intimaEndothelium and connective tissueTunica mediaSmooth muscle and elastic tissueTunica externa or tunica adventitiaConnective and elastic tissue
5ArteriesLarge arteries are elastic (conducting) arteries – pressure reservoirsMedium arteries are muscular (distributing) arteries – more smooth muscleContraction or relaxation of muscle changes the size of the lumen, and so controls the blood pressure in the vessel.
6Capillaries Only a single layer of endothelium and a basement membrane Connect arterioles and venulesFunctional part of systemTrue capillaries begin at a precapillary sphincter which controls blood flow through the capillary
7Veins Relatively thin; less elastic Larger in diameter than arteries Have valves to prevent backflow of bloodFlow to heart is assisted by contraction of skeletal muscles
8Veins have larger diameters, larger lumina, and thinner, less well-organized walls veins are more prone to dilation, compression, and easy penetration by tumors and inflammatory processes.
9Lymphatics are thin-walled, endothelium-lined channels that drain excess interstitial tissue fluid eventually returning it to blood via the thoracic duct. Lymphatic flow also contains mononuclear inflammatory cells and a host of proteins; by passing through lymph nodes,
10Functions of the lymphatics 1. lymphatics constitute an important pathway for continuous sampling of peripheral tissues for infection.2.These channels can also disseminate disease by transporting microbes or tumor cells from distant sites to lymph nodes and eventually to the systemic circulation
11The main cellular components of the walls of vessels are: 1) endothelial cells2) smooth muscle cells3) pericytes (the cells normally arranged along capillaries and venules)
12Endothelial cells:a) serve as a semipermeable membrane,b) regulate thrombosis, thrombolysis and platelet adherence,c) influate vascular tone and blood flow,d) metabolize hormones,e) regulate immune and inflammatory reactions,f) modify lipoptoteins in the artery wall,g) regulate the growth of other cell types, including smooth muscle cells.
13Endothelial injuryis critical to the formation of thrombi, to the initiation of atherosclerosis and the vascular effects of hypertension and other disorders !!
15Pathology of blood and lymphatic vessels The term endothelial dysfunction is often used to describe several types of potentially reversible changes in the functional state of endothelial cells that occur in response to environmental stimuli.
16The term endothelial activation reflects alterations in gene expression and protein synthesis. Inducers of endothelial activation include cytokines and bacterial products (which cause inflammatory injury and septic shock), hemodynamic stress and lipid products (involved in pathogenesis of atherosclerosis), advanced glycosylation of end products (involved in pathogenesis of diabetes), as well as viruses, complement components and hypoxia..
17Activated endothelial cells also elaborate adhesion molecules, other cytokines and chemokines, growth factors, molecules of the major histocompatibility complex (MHC), procoagulant or anticoagulant factors and vasoactive molecules that are involved either in vasoconstriction or in vasodilatation
182) Smooth muscle cells are capable: a)to mediate vasoconstriction, By Nitric oxideb) to mediate vasodilatation,c) to synthesize the collagen, elastin, and proteoglycans,d) to elaborate the growth factors and cytokines,e) to proliferate, andf) to migrate to the intima. 3) Pericytes have role as supportive and connective elements.
19Diseases of arteriasA) Congenital anomaliesB) AtherosclerosisC) Hypertensive vascular diseaseD) Inflammatory disease – Arteritides (Vasculitides)E) Raynaud diseaseF) Aneurysms a dissections
20Congenital anomoliesAVM Arterio Venous malformationsAbnormal communication between the high pressure arteries and low pressure veins.Usually congenital but acquried by trauma or inflammation.Most often described in Brain as AVMAsymptomatic or hemorrhage or pressure effect.
21Diseases of arterias B. Atherosclerosis It is a generic term for three patters of vascular disease that have in common thickening and loss of elasticity of arterial walls:1) Atherosclerosis – characterized by the formation of intimal fibrous plaques that often have a central core rich in lipid (fibrofatty plaques).2) Mönckeberg medial calcific sclerosis – characterized by calcific deposits in medium-sized muscular arteries in persons older than 50 years. These medial lesions forming irregular medial plates or discrete transverse rings have much less clinical importance.3) Arteriosclerosis – the hyaline and hyperplastic thickening of small arteries and arterioles which causes luminal narrowing and down stream ischemic injury.
22Monckeberg arteriosclerosis (medial calcific sclerosis) involves the media of medium sized muscular arteries, most typically the radial and ulnar arteries,persons older than 50 years of age. It does not obstruct arterial flow because the intima is not involved.a. Ring-like calcifications in the media of the arteries are characteristic.b. Stiff, calcific "pipestem" arteries result.c. This form of arteriosclerosis may coexist with atherosclerosis, but it is distinct from and unrelated to it.
23ARTERIO-SCLEROSIS GENERIC term for ANYTHING which HARDENS arteries Atherosclerosis (99%)Mönckeberg medial calcific sclerosis (1%)Arteriolosclerosis, involving small arteries and arterioles, generally regarded as NOT strictly being part of atherosclerosis, but more related to hypertension and/or diabetesAtherosclerosis and arteriosclerosis are often used so interchangeably, it is hardly worth differentiating them anymore, other than to know they are different!
24Hyaline Arteriolosclerosis Hyaline Arteriolosclerosis. This vascular lesion consists of a homogeneous pink hyaline thickening of the walls of arterioles with loss of underlying structural detail and with narrowing of the lumen.Present inElderly patients, whether normotensive or hypertensive,patients with hypertension.It is also common as part of the characteristic microangiography in diabetes .
25HISTOPATHOLOGY of ESSENTIAL HYPERTENSION Often, benign or “malignant” hypertension is described as two different types of changes in arterioles, usually renal.Benign: Hyalization of arteriole wallMalignant: Fibrinoid necrosis and “onion skinning” of arteriole wall“HYALINE” = BENIGN HTN. “HYPERPLASTIC” = MALIGNANT HTN. SYS> ) ONION SKIN 2) “FIBRINOID” NECR.
26Hyperplastic Arteriolosclerosis. Seen in Malignant hypertension (typically, diastolic pressures over 120 mm Hg associated with acute cerebral and/or renal injury).Hyperplastic arteriolosclerosis is associated with "onion-skin," concentric, laminated thickening of the walls of arterioles with luminal narrowing The laminations consist of SMCs and thickened, duplicated basement membrane.
27In malignant hypertension, these hyperplastic changes are accompanied by fibrinoid deposits and vessel wall necrosis (necrotizing arteriolitis), particularly prominent in the kidney.
30Primary hypertension Also called essential or idiopathic hypertension % of all casesNo specific cause identifiedCan happen with retention of sodium and water → increased blood volume.Also low dietary potassium, calcium and magnesium intakes
31Suspected causes Interaction of genetics and environment Overactivity of sympathetic nervous systemOveractivity of renin / angiotensin/ aldosterone systemSalt and water retention by kidneysAnd others
33Always know that hypertension can be understood best by remembering the simple BP=COxPR equation.
34ReninAngiotensinAldosterone AXIS (RAAS) If the perfusion of the juxtaglomerular apparatus in the kidneys decreases, then the juxtaglomerular cells release the enzyme renin.Renin cleaves an inactive peptide called angiotensinogen, converting it into angiotensin I.Angiotensin I is then converted to angiotensin II by angiotensin-converting enzyme (ACE), which is found mainly in lung capillaries.Angiotensin II is the major bioactive product of the renin-angiotensin system. Angiotensin II acts as an endocrine, autocrine/ paracrine, and intracrine hormone.Sometimes this is called RAASReninAngiotensin (but angiotensis does SO MUCH MORE than just activate aldosterone)AldosteroneSodium
35GENETICACQUIREDA little more thorough diagram of RAAS pathology---- genetic and acquired.
36GENETIC vs. ENVIRONMENTAL GENETIC UN-CONTROLLABLEENVIRONMENTAL CONTROLLABLESTRESSOBESITYSMOKINGPHYSICAL ACTIVITYNaCl INTAKE
38FATTY STREAK (non-palpable, but a visible YELLOW streak) 2) ATHEROMA (plaque) (palpable)3) THROMBUS (non-functional, symptomatic)Atherosclerosis is a LIFELONG, even childhood, process. This chart is worth knowing.
40MAJOR factors Hyperlipidemia Hypertension Cigarette Smoking Diabetes Milletus“Framingham” data. Please remember that these are the ONLY four MAJOR risk factors. If somebody tells you there is anything else which is a MAJOR risk factor, nominate that joker for a Nobel prize.
41PATHOGENESIS“atherosclerosis is a chronic inflammatory response of the arterial wall initiated by injury to the endothelium”
42PATHOGENESIS Chronic endothelial injury LDL, Cholesterol in arterial WALLOXIDATION of lipoproteinsMonocytes migrate endothelium*Platelet adhesion and activationMigration of SMOOTH MUSCLE from media to intima to activate macrophages (foam cells)Proliferation of SMOOTH MUSCLE and ECMAccumulation of lipids in cells and ECMLike the sequence of events described in acute inflammation, atherosclerosis is its own Cecil B. DeMille saga! Not only should you all be familiar with these processes, but their ORDER as well.* This may be the first (i.e., earliest) microscopic and gross finding.
43This is the VERY VERY best diagram of atherosclerosis you will ever see. The nice thing about diagrams is that you really do not have to memorize them, but just visually “recall” the concepts!
44A very well constructed graphic understanding of the pathogenesis of atherosclerosis. Please be expected to not only KNOW these five items, but their correct ORDER too.
45The mechanism of Hyperlipidemia contributing to Atherosclerosis Endothelial impairment by chronic hyperlipidemia by increasing o2 free radicles productionDecay in NOImpair vaso dilationImpair endothelial functiuon Oxidise the LDL
46Fatty streak is the earliest lesion in Atherosclerosis CompositionAtherosclerotic plaquesComponentsSmoothmuscle cells macrophages and t cellsECMIntracellualr and extracellular lipids.
47Fatty STREAKS can be SEEN as YELLOW, but NOT palpated, ATHEROMAS can be palpated, thrombi are symptomatic often.A rule of thumb is that stenosis is symptomatic if > 90%.
48MORPHOLOGIC CONCEPTS Macrophages (really monocytes) infiltrate Intimal ThickeningLipid AccumulationStreakAtheromaSmooth Muscle Hyperplasia and MigrationFibrosisCalcificationAneurysmThrombosisThese are also, generally, in order of severity.
49Cholerterol (really cholesterol esters) makes macrophages “foamy” and cause “clefts” extracellularly.PLAQUE
51ADVANCED FEATURES RUPTURE ULCERATION EROSION ATHEROEMBOLI HEMORRHAGE THROMBOSISANEURYSMThese should all be household words in your vocabulary.
52ANEURYSMS TRUE vs. FALSE ATHEROSCLEROTIC NON-ATHEROSCLEROTIC CONGENITALLUETIC (SYPHILITIC)TRAUMATIC“MYCOTIC” (MIS-leading term)2° to VASCULITISSACCULAR (i.e., “Berry”) vs. FUSIFORMDISSECTION vs. NON-DISSECTION
53An aneurysm is a localized abnormal dilation of a blood vessel or the heart When an aneurysm involves all three layers of the arterial wall (intima, media, and adventitia) or the attenuated wall of the heart, it is called a "true" aneurysmfalse aneurysm (also called pseudoaneurysm) is a breach in the vascular wall leading to an extravascular hematoma that freely communicates with the intravascular space ("pulsating hematoma").
54Know the difference between a TRUE (endothelial expansion) and FALSE (NO endothelial expansion) aneurysm
55True AneurysmsAtherosclerotic.Syphilitic,CongenitalVentricularFalse aneurysmsVentricular ruptuture after MI
56They are of 2 typesSaccularFusiform<ost common causes of aneurysmAtherosclerosisHypertension
57Pathogenesis1.The intrensic quality of the vascular wall conncetive tissue is poorEg Marfan syndromeLoeys Deitz syndromeEhlers Danlos syndrome
582.The balance of collagen synthesis and degradation is altered by inflammatory. Infiltration and destructive proteolytic enzymes.Increaesed production of MMP by macrophages in atherosclerosis or vasculitisAlso decreased in tissue MMP inhibitors
593.The vascular wall weakend through loss of smooth muscle cells. Hypoxia due to athermanous plaque and leads to ischemia and HT in turn leads to smooth muscle loss and scarring and decreased synthesis of ECM and increased amount of gycosaminoglycons called as cystic medial degeneration seen in Marfan syndrome and scurvy.
60Most abdominal aortic aneurysms (AAA) occur between the renal arteries and the bifurcation of the aorta
61Abdominal Aortic Aneurysm Atherosclerosis, the most common cause of aneurysms, causes thinning and weakening of the media secondary to intimal plaques.Site-. Atherosclerotic aneurysms occur most frequently in the abdominal aorta (abdominal aortic aneurysm, often abbreviated AAA), but the common iliac arteries, the arch, and descending parts of the thoracic aorta can also be involved.
62Pathogenesis AAA occurs more frequently in men and rarely develops before age 50. Atherosclerosis is a major cause of AAA, but there are clearly other contributors, since the incidence is less than 5% in men older than 60 years,
63Two AAA variantsInflammatory AAAs are characterized by dense periaortic fibrosis containing abundant lymphoplasmacytic infiltrate with many macrophages and often giant cells. Their cause is uncertain.
64Mycotic AAAs are atherosclerotic lesions infected by lodging of circulating microorganisms in the wall, particularly in the setting of bacteremia from a primary Salmonella gastroenteritis. In such cases, suppuration further destroys the media, potentiating rapid dilation and rupture.
65Syphilitic ( luetic) aneurysm a. This aneurysm is a manifestation of tertiary syphilis, which has become rare with bettertreatment and control of the disease. It is caused by syphilitic aortitis, which is characterizedby obliterative endarteritis of the vasa vasorum and necrosis of the media.Grossly, these changes result in a "tree-bark" appearance
66The narrowing of the lumina of the vasa vasorum causes ischemic injury of the aortic media, with patchy loss of the medial elastic fibers and muscle cells, followed by inflammation and scarring, the aorta loses its elastic recoil producing an aneurysm. Contraction of fibrous scars may lead to wrinkling of intervening segments of aortic intima, grossly reminiscent of "tree bark."
67Unlike atherosclerotic aneurysms, syphilitic aneurysms characteristically involve the ascending aorta. Dilation of the ascending aorta may widen the aortic commissures,leading to aortic valve insufficiency
68Clinical featuresThe clinical features depend on the consequences of the AAARupture into peritoneal cavity leads to fatal hemorrhageObstruction into the branch leads to ischemic injuryEmbolsim
69Compression of the adjucent structures like urter or vertebral erosion the risk of ruture depends upon the sizes a abdominal mass.
70THORACIC ANEURYSMS Hypertnesion is common cause Marfan syndrome and loeys Dietz syndromeEncroachmentRespiratory difficultiesDysphagiaCoughPainAortic valve dilatationRupture
71Dissection (i.e., blood or hemorrhage disrupting the wall of a large artery) can be both a cause or an effect of an aneurysm.DISSECTION
72Aorrtic DissectionBlood splays apart the laminar planes of the media to form a blood-filled channel within the aortic wall this channel often ruptures through the adventitia and into various spaces, where it causes either massive hemorrhage or cardiac tamponade
73(1) men aged 40 to 60 years, with antecedent hypertension (more than 90% of cases of dissection), and(2) younger patients with systemic or localized abnormalities of connective tissue affecting the aorta.3.Iatrogenic following catheterization or bypass surgery.During and after pregnancy.
74Hypertension is the major risk factor. There is medial hypertrophy of the vasa vasorum.Degenerative changes in the aortic mediaLoss of smoioth muscle cellsThe medial weakness trigger the intimal tear.There is cystic medial degeneration.
75Clinical features.The more common (and dangerous) proximal lesions (called type A dissections), involving either the ascending aorta only or both the ascending and descending aorta.(types I and II of the DeBakey classification)Distal lesions not involving the ascending part and usually beginning distal to the subclavian artery (called type B dissections or DeBakey type III).
76The classic clinical symptoms of aortic dissection are the sudden onset of excruciating pain, usually beginning in the anterior chest, radiating to the back between the scapulae, and moving downward as the dissection progresses; the pain can be confused with that of myocardial infarction.
77The most common cause of death is rupture of the dissection into body cavities (i.e., pericardial, pleural, or peritoneal). Retrograde dissection into the aortic root can cause disruption of the aortic valvular apparatus. cardiac tamponade,aortic insufficiency,and myocardial infarction or extension of the dissection into the great arteries of the neck or into the coronary, renal, mesenteric, or iliac arteries
78VASCULITISVasculitis, or inflammation of vessel walls1.Immune mediated2.Direct invasion by infectious agents.
79an result from infections, but it more commonly has an immunologic basis such as immune complex deposition, ANCAs, or anti-EC antibodies.Different forms of vasculitis tend to specifically affect vessels of a particular caliber and location.
82Patients with Vasculitis have circulating antibodies that react with the Neutrophil cytoplasmic antigens.
83GIANT CELL ARTERITIS.It is the most common form of Vasculitis seen in elderly patients and It is a chronic typically granulomatous inflammation of large and small sized arteries.
84“TEMPORAL” ARTERITIS Giant Cell Arteritis, GCA ADULTSMainly arteries of the head and temporal arteries are the most visibly, palpably, and surgically accessibleBLINDNESS most feared sequelaeGRANULOMATOUS WALL inflammation diagnosticOFTEN associated with marked ESR elevation to be then known as POLYMYALGIA RHEUMATICAAnti-NEUTROPHIL AB’s often POSITIVE
93KAWASAKI DISEASE CHILDREN <4 years. CORONARY ARTERIES LEADING cause of ACQUIRED heart disease in childrenUSA and JAPANFatal in only 1%
94Also it is Mucocutaneous lymphnode syndrome as it involves oral, conjuctival erythema and edema of hands and cervical lymphnode enlargement.It is associated with Mycoplasma and HIV infection in some cases.Untreated develop cardiovascular complications like pericarditis ,MI and aneurysm.Immunoglobulin and ASA is the treatment.
95MICROSCOPIC POLYANGIITIS HYPERSENSITIVITY VASCULITIS LEUKOCYTOCLASTIC VASCULITIS SMALL VESSELS OF ALL TYPES, e.g., capillaries and veins tooFRAGMENTED NEUTROPHILSCalled PAUCI IMMUNE INJURYIittle or no immunoglobulins in most lesions.Necrotising GLOMERULONEPHRITIS in 90% of the patients.Most are ALLERGIC reactions to allergens like penicillin or strepDERMATOLOGIST’s DISEASE
96WEGENER GRANULOMATOSIS M>F, often in 40’sACUTE NECROTIZING GRANULOMAS OF UPPER an LOWER respiratory tractNECROTIZING GRANULOMATOUS VASCULITIS of SMALL vessels of ALL typesOften renal involvement, “crescentic” glomerulonephritisANTI-NEUTROPHIL-CYTOPLASMIC-AB’s usually presentMortality has improved significantly, but flare-ups, i.e., recurrences, are still the main concern.
97Clinical FeaturesPatients present with Chronic Sinusitis, Pnemonitis,Hemoptysis and Hematuria,mucosal ulcerations of the nasopharynxEpistaxis,nasal crusting.Nodular infiltration and Glomerular nephritis .
98Pathologically Wegener's Granulomatosis is characterized by three features: Necrotizing granulomatous inflammation of the upper and lower respiratory tracts.Disseminated small vessel necrotizing vasculitis that affects both arteries and veins.Focal necrotizing glomerulonephritis.
99Diagnostic criteria for Wegener's Granulomatosis are, 1) Abnormal urine sediments, like red blood cells or casts, 2) An abnormal chest radiograph. 3) Oral ulcers or nasal discharge and 4) granulomatous inflammation on biopsy. Pathogenesis is believed to be a hypersensitivity reaction to infection with staphylococcus aureus and parvovirus B-19 [1
101necrosis granulomas necrosis granulomas necrosis granulomas Why is the “necrosis” of Wegener’s not called “caseating”? Ans: Because, by tradition, rather than objectivity or logic, “caseation” has been attributed to tuberculosis. Yes, we know this is not fair.
102CHURG - STRAUSS SYNDROME Called as Allergic granulomatosis and Angitis.Associated with Asthma, rhinitis,lung infiltrates peripheral Eosinophilia,and Necrotising granulomas.Present with cutaneous purpura bleeding frm GIT and Focal and glomerulo sclerosis
103Allergic granulomatosis and angiitis strong association with allergic rhinitis, bronchial asthma, and peripheral eosinophilia; p-ANCAs are present in roughly half the patients. In Churg-Strauss syndrome, and perivascular tissues by eosinophils. Unlike Wegener granulomatosis, severe renal disease is infrequent in Churg-Strauss syndrome; instead, coronary arteritis and myocarditis
104THROMBOANGIITIS OBLITERANS BUERGER(‘s) Disease 100% caused by cigarette smokingMEN>>>F, 30’s, 40’sOften arteries are 100% obliterated, hence the name “obliterans”EXTREMITIES most often involved.
105acute and chronic inflammation of medium-sized and small arteries, principally the tibial and radial arteries, with occasional secondary extension into extremity veins and nerves.
106intravenous prostaglandin analogue. Clinical Features The early manifestations are a superficial nodular phlebitis,Resting pain on the forefoot ischaracteristic, with possible ischemiculcers or gangrene of foot/ toes;upper limb ischemia [40% to 50%of patients) with ulceration andgangrene; Raynaud's phenomenon.Treatment: smoking cessation essential;intravenous prostaglandin analogue.
108RAYNAUD’S PHENOMENON.It is a vasospasm in the peripheral arteries due to cold and emotional stimuli.Pallor due to vasospasmCynosis due toRubor due to reactive hyperemia.
109Raynaud “Phenomenon” PRIMARY: (formerly Raynaud “DISEASE”) Digital PALLORCYANOSISHYPEREMIA(WHITE) (BLUE) (RED)Vasoconstriction usually triggered by COLD, emotionCan be tip of nose, not only digitsSelf Limited, Gangrene UN-commonArteries often do NOT show diagnostic pathologySECONDARY: (formerly Raynaud “Phenomen.”)AtherosclerososSLEBuerger Disease
110RAYNAUD PHENOMENONThere are two types of Raynaud phenomenon.Primary and SecondaryPrimary Raynaud phenomenon (previously called Raynaud disease) reflects an exaggeration of central and local vasomotor responses to cold or emotion,
111Structural changes in the arterial walls are absent except late in the course, when intimal thickening can appear. The course of primary Raynaud phenomenon is usually benign, but long-standing, chronic cases can result in atrophy of the skin, subcutaneous tissues, and muscles. Ulceration and ischemic gangrene rare.
113“Varicose” Veins 20% of population, Female 30% male 20% Related to increased venous pressure, age, valve dysfunctionSuperficial veins of lower extremities most commonPATH: 1) DILATED, 2) TORTUOUS, ) ELONGATED, 4) SCARRED (phlebosclerosis), 5) CALCIFICATIONS, 6) NON-UNIFORM SMOOTH MUSCLEConceptually like varices or hemorrhoids
114.B. Varicose veins1. Abnormally distended, lengthened, and tortuous veins2. Locationsa. Superficial saphenous veins (most common site)b. Distal esophagus (due to portal hypertension)c. Anorectal region (e.g.. hemorrhoids)d. Left scrotal sac (e.g., varicocele)
115Superficial varicosities; causes:; a. Valve incompetence of perforator branches with reversal of blood flow from high-pressure deep venousSystem into superficial system.• Exacerbated by pregnancy, prolonged standing, obesity, oral contraceptives,advanced ageFamilial tendencyc. Secondary to deep venous thrombosis• Retrograde blood How through peribrating branches into the superficial system
116(1) Nonpharmacologic•Graded compression stockings(2) Chronic treatment(a) Compression sclerotherapy(b) Ligation and stripping(e) Endovenous obliteration using radiofreqency (diathermy) or laser.
118Phlebothrombosis1. Thrombosis of vein with out inflammation. Causes Phlebothrombosis:a. Stasis of blood flowb. Hypercoagulability-(e.g.. antithrombin III deficiency)3. Locationa. Most often occurs in the deep vein of the calfb. Less common sites includes portal vein, hepatic vein, dural sinuses,
119(1) Swelling(2) Pain on dorsiflexion of foot (Homans' sign) and compression of the calf(3) Pitting edema distal to the thrombosis (increased hydrostatic pressure)Unilateral sometime bilateral either impaired venous or lymphatic return.
120SVC SYNDROME Usually from bronchogenic CA or mediastinal lymphoma “DUSKY CYANOSIS” of:HeadNeckArms
121Pathogenesisa. Extrinsic compression of the superior vena cavab. Due to a primary lung cancer (90% of cases)• Usually a small cell carcinoma of the lung2. Clinical findingsa, "Puffiness" and blue to purple discoloration of the face, arms, and shouldersb. Retinal hemorrhage, stroke3. Treatment• Radiation; stent to bypass obstruction.
122IVC SYNDROME Secondary to: Bilateral leg edema NEOPLASMS (external compression)ASCENDING THROMBOSIS from FEMORALS, ILIACSAAA, Gravid uterusBilateral leg edemaMassive proteinuria if renal veins involved (like nephrotic syndrome)
123LYMPHANGITIS From regional infections Group-A beta-hemolytic strep most commonLymphatics dilated, filled with WBCsCellulitis usually present tooLymphadenitis also usually followsIf lymph nodes cannot filter (process) antigens enough septicemia
124Diseases of veins and lymphatics E) Lymphangitis and LymphedemaPrimary diseases – extremely uncommon.Secondary processes – develop in association with inflammation or cancer.Lymphangitis: bacterial infections spreading into and through the lymphatics.Obstructive lymphedema: Occlusions of lymphatic drainage by tumor, by postradiation fibrosis, by filariasis, trauma or by inflammatory thrombosis which is accompanied by abnormal accumulation of interstitial fluid in the affected part.
125LYMPHANGIOMA Small 1-2 mm Simple or capillary type and Cavernous 90% Head and neck region in kids <2Generally……RAREWhen large size and/or spaces present often called “CYSTIC HYGROMA”
126Vascular TUMORSBENIGN (NEVER metastasize, in fact some are not even TRUE neoplasms, but hamartomas)INTERMEDIATE (rarely metastasize)MALIGNANT (FREQUENT and EARLY metastases, like any other sarcoma lung)Vascular tumors generally follow the same diagnostic patterns of other mesenchymal (i.e., “soft” tissue) tumors. Often the KEY difference is that “endothelium” lined blood filled spaces, or identification of endothelial cells by antigenic markers, such as factor VIII, is usually present.
127Vascular tumors can be endothelium derived or Arise from the cells support and or surrounds the vessel.Benign tumors produce obvious vascular channels filled with blood cells or lymph lined by normal endothelial layerMalignant are more cellular and show atypia.
128HEMANGIOMA CAPILLARY (small vascular spaces) Often a generic term for ANY benign blood vessel tumor very common tumor.7% of infants and childhood tumor.CAPILLARY (small vascular spaces)Also called “juvenile”, often called “birth marks”Usually regress with ageCAVERNOUS (LARGE vascular spaces)Also called “adult”Usually do NOT regressSmall spaces = often small people, i.e., kidsLarge spaces = often large people, i.e., adults
129A Hemangioma and D Pyogenic granuloma B Juvenile capillarty hemangioma C Cavernous hemangioma
130Capillary hemangioma occurs on skin mucous membrane and oral cavity and lips Strawberry or juvenile hemangioma of the skin is common.
131Cavernous hemangiomas are a component of von Hippel-Lindau disease occurring within the cerebellum or brain stem and eye grounds, along with similar angiomatous lesions or cystic neoplasms in the pancreas and liver; von Hippel-Lindau disease is also associated with renal neoplasms.
132Pyogenic granulomaIt is a form of capillary hemangioma.Seen after a trauma usually reach in size about 2 to 3 cm after 6 weeks.There is also pregnancy tumor( granuloma gravidarum)They undergo fibrosis. Or need excision.
133Cystic HygromaThese lesions are typically found in the neck or axilla of children and, rarely, in the retroperitoneum; cavernous lymphangiomas of the neck are common in Turner syndrome These lesions can occasionally be enormous (≤15 cm in diameter) and may fill the axilla or produce gross deformities about the neck. Tumors are composed of massively dilated lymphatic spaces lined by ECs and separated by intervening connective tissue stroma containing lymphoid aggregates. The tumor margins are not discrete and the lesions are not encapsulated, making resection difficult.
135Vascular Ectasiasare common lesions characterized by local dilation of preexisting vessels; they are not true neoplasms. Telangiectasia is a term used for a congenital anomaly or acquired exaggeration of preformed vessels-usually in the skin or mucous membrane
139MISC. “BENIGN” TUMORS -ectasias, telangiectasias Nevus Flammeus,, port wine stain----Spiders (spider telangiectasias), ass. W. pregnancy, cirrhosis Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Bacillary Angiomatosis, in HIV patients, caused by bacilli of Bartinella species“-ectasia” is a generic term meaning dilation and is primarily used with regard to veins rather than arteries.
140The so-called port wine stain is a special form of nevus flammeus; these lesions tend to grow with a child, thicken the skin surface, and demonstrate no tendency to fade.
141Spider Telangiectasia non-neoplastic vascular lesion grossly resembles a spider; there is a radial, often pulsatile,dilated subcutaneous arteries or arterioles (resembling legs) about a central core (resembling a body) that blanches when pressure is applied to its center.
142It is commonly seen on the face, neck, or upper chest Causes.hyperestrogenic statessuch as pregnancy orcirrhosis.
143Bacillary Angiomatosis Bacillary angiomatosis is an opportunistic infection in immunocompromised persons that manifests as vascular proliferations involvingskin, bone, brain, and other organs.First described in patients with acquired immunodeficiency syndrome,Causative organismgram-negative bacilli of the Bartonella hensle
144Skin lesions are red papules or nodules rounded subcutaneous masses. Capillary proliferation due to VEGF production by HIF 1 alpha.Nuclear atypia and mitosis also lesions contain neutrophils and bacteria
145Kaposi SarcomaKaposi sarcoma (KS) common in patients with acquired immunodeficiency syndrome (AIDS) prior to the advent of effective antiretroviral therapy; indeed, its presence is used as a criterion for diagnosing AIDS.
1471.Chronic KS also called clasiic European kS They usually present red to purple nodules on the skin on the lower extremities.Due to malignancy or altered immunity.Not seen with AIDS pateients
1482.Lymphedenopathic KS called African or endemic KS Skin lesions are less.Visceral involvement is more and aggressive.
149Pathogenesis of KSKS herpes virusKSHV proteins disrupt the cell cycle and viral genes produce P53 inhibitors and prevent apoptosis
150Malignant tumrosAngiosarcoma it is a endothelial tumor.Hepatic angio sarcoma following environmental exposurePlastic factory workers(Vinayl chloride)Arsenic pesticidesRadio contrast( Thorotrast)Induced by radiation
151They present as red nodules Locally invasive and metastasize readily.Aggressive tumors.
153PATHOLOGY OF VASCULAR INTERVENTION morphologic changes that occur in vessels following therapeutic intervention (i.e., balloon angioplasty, stenting, or bypass surgery) typically recapitulate many of the changes that occur in the setting of any vascular insult. Local EC trauma (e.g., due to a stent), vascular thrombosis (after angioplasty), and abnormal mechanical forces (e.g., a saphenous vein inserted into the arterial circulation as a coronary artery bypass graft) all elicit similar responses characteristic of vessel wall healing. As with atherosclerosis, the traumas of vascular intervention tend to induce a concentric intimal thickening composed of recruited SMCs and their associated matrix deposition
154STENTS Metallic mesh Permanently placed Stays patent longer than angioplastyOFTEN DRUG COATEDGoals:Prevent thrombosisPrevent spasmDelay RE-stenosis
155What is angioplasty?PT CA Percutaneous transluminal Coronary AGPPremedicationsAntithroboticPrevent Vasospasm
156GRAFTS 400,000 CABG grafts per year in USA Saphenous v. vs. Internal mammary a. (internal thoracic a.)50% patent after 10 years, for saphenous v.90% patent after 10 years, for mammary a.
157Vascular Replacement Synthetic or autologous vascular grafts are increasingly used to replace damaged vessels or bypass diseased arteries. Of the synthetic grafts, large-bore (12- to 18-mm) conduits function well in high-flow locations such as the aorta, while small-diameter artificial grafts (≤8 mm in diameter) generally fail because of acute thrombosis