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Vascular Diseases, Vascular Diseases VEINS, LYMPHATICS Varicose, TP/PT-90% Inferior Vena Cava Syndrome -obstruction, compression -neoplasms, femoral/iliam thrombus -HEPATOCELLULAR, RENAL CELL CA -grow w/in vv extend to IVC ->edema of legs, distended superficial collaterals of lower abd ->massive proteinuria (kidneys), Lymphangitis -acute inflam due to bac spread -esp GABHS-Strep pyogenes -dilated lymph, filled w/ exudate ->LN enlargement w/ red streaks on skin along lymph path can->cellulitis, focal abscesses can spill into vv->bacteremia Lymphedema Lymphedema-lymphatic blockage 2ndary to:1)malig tumor spread 2)surgical LN remova (mastectomy) 3)postirradiation fibrosis 4)filariasis 5)postinflam thrombosis/scar ->Chylous ascites, chylothorax, chylopericardium -via rupture of obstructed, dilated lymphatic Primary Lymphedema less common -can be isolated congenital or familial -fam-Milroy dz(heredofamilial congenital LE) Lymphedema praecox-10-25yo FM -slow accumulation of edema in involved limb All->edema->interstitial fibrosis->peau d'orange, ulcers on skin, Vascular Diseases ANEURYSMS/DISSECTION -most common in heart/Ao TRUE-blood stays intravasc -via Atherosclerosis, CMD FALSE-communicate w/ extravasc Saccular vs. Fusiform in macro AAA -AS thin aa wall (tunica media) 2ndary to plaque -aneurysms occur in AAo or iliac and other Ao parts -Usually between renal aa and bifurcation MORPHOLOGY Can be saccular, cylindroid, or fusiform cm dia ញ cm length -Mural thrombus often w/in sac, can fill sac -Fusi/Cylin-layers of mural throm- only partial fill Inflam AA-dense periaortic fibrosis, giant cells, macs Mycotic AA-infx by circulating orgs in wall-Salmonella -suppuration further destroys media->rupture PATHOGENESIS Men ᡪyo, familial, genetic structural CT defects ->more susceptible AS, HTN dmg -matrix metalloproteinases, TPAs->degrade ECM->AAA CLINICAL - depends on location -rupture in peritoneal or retro->massive or fatal hemorr -occlusion of branch aa via pressure or thrombus -esp iliac, renal, mesenteric, vertebral(cord) brr. -embolism from atheroma, mural thrombus -impingement on nearby struction, eg ureter -palpable pulsating abd mass - simulates tumor Tx - fear rupture, esp ɱcm -replace w/ graft SOON -50% mort if wait til after rupture, Vascular Diseases TUMORS Malignant Tumors Angiosarcomas-endoth neoplasm -from diff'd (like heman) to anaplastic -in adults, skin, soft tissue, breast, liver -can come from lymphedema -esp 10yr post radical mastectomy -arises from lymphangiosarcoma -swelling->nodules->fuse->ulcerate -also via radiation after Tx for BRCA, etc. -also via foreign material MORPH-small, red (-)Sx->large, fleshy, pale -central softening, necrotic areas uscope-all degrees of diff'ation -more malignant w/ spindles Hepatic AS-arsenic, Thorotrast, PVC -long latent period POOR OUTCOME-most die ɝyr, Vascular Diseases THERAPEUTIC INTERVENTIONS Balloon Angioplasty, etc. -dilation of atheromatous stenotic vessel w/ balloon catheter -eg Percutaneous Transluminal Cor Ang ->plaque fracture->local hemorr dissection ->stretches media of dissected segment ->instability -can ->abrupt reclosure-usually due to dissection/thrombosis -BUT MOST IMPROVE PROGNOSIS -30-50%->proliferative restenosis w/in 6 mo -via cell injury, thrombosis, inflam, elastic, Vascular Diseases TUMORS Benign Tumors/Tumor-like Conditions Nevus Flammeus-MOST COMMON ECTASIA-salmon patch -birthmark-vessel dilation in dermis -Port-wine stain-grows w/ age->UGLY -in Sturge-Weber syndrome if CN V -ass'd w/ hemiplegia, seizures, MR, skull radiopacity Spider Telangiectasia-radial, pulsatile, dilated subcut aa -on face, neck, upper chest -PREGNANT WOMEN, LIVER CIRRHOSIS-hyperestrinism Hereditary Hemorrhagic Tel (Osler-Weber-Rendu syn) -auto dom-dilated caps/vv on skin, oral mucous membs, resp, GI, urinary tracts -can rupture->nosebleeds, hematuria, bleeding into gut, Vascular Diseases THERAPEUTIC INTERVENTIONS Vascular Replacement Large diameter Dacron work better than smaller, fabric -For small-AUTOLOGOUS SAPHENOUS VEIN -expanded polytetrafluorethylene (Teflon) -Failure due to thrombosis, intimal fibrosis, or atherosclerosis Healing depends on sm. mm cells anastomosis -neointima not widespread-w/ endoth cells -pseudointima w/ other cell materials CABG-Aortocoronary Bypass -use saphenous vv or internal mammary aa. -mostly successful -late recurrence due to graft occlusion, or atherosclerosis distal to graft -50% long-term patency in 10 years for saph -thrombosis, intimal thickening, plaques -90% for int. mammary, Vascular Diseases HYPERTENSIVE VASCULAR DISEASE Pathogenesis 2ndary causes understood: -renovascular HTN-renal a stenosis->renin -pheochromocytoma->catecholamines->constrict Genetic-polygenic, heterogeneous -but single gene mutations-aldost metabolism, -Na reabsorption mech (Liddle Syndrome) Environmental-stress, obesity, smoking, sedentary, high salt intake Mechanisms 1)Renal retention of Excess Na-primary cause of HTN -hgih volume->vasoconstriction (autoreg)->HTN -resets pressure natriuresis 2)Vasoconstriction/Vasc Hypertrophy -via constrictors or structural changes -due to 1)relaxation response (meditation) 2)inc. vasoconstrictor release 3)inc. sensitivity -some constrictors are also growth fx for sm mm. PATHOLOGY-acclerated atherogenesis -vasc walla changes->dissection, cerebrovasc hemorr MORPH-2 small vessel dzs 1)Hyaline Arteriosclerosis-elderly, normo- or HTN -also in DM-hyaline thickening +small lumen ->BENIGN NEPHROSCLEROSIS, small kidneys 2)Hyperplastic Art-more acute, severe elevations -esp in malignant HTN -onion-skin thickening, narrow lumen ->NECROTIZING ARTERIOLITIS, esp in kidney, Vascular Diseases VEINS, LYMPHATICS Varicose, TP/PT-90% Phlebothrombosis-esp DVT in leg (90%) Thrombophlebitis-PT w/ inflammation Predispositions-Cardiac failure, CA, preg, postop, bed rest, immobilization -hypercoag. state -also in periprostatic, pelvic vv. -large skull vv, dural sinuses, abd infx Thrombi w/ few Sx if any-edema, cyanosis, dilated sup vv, pain ->Embolic Episode-pulm, can be final blow Hypercoag w/ Panc, Colon, Lung adenoCA ->migratory TP (Trousseau Sign) Prevent with mvmt in bed, exercises, even anticoags Phlegmasia Alba Dolens-painful white leg -pregnant women peripartum (milk leg), Vascular Diseases ATHEROSCLEROSIS (AS) See Atherosclerosis Map