Diseases of blood vessels

Dr. Mohammed Hajhamad, MB.ChB. (Egypt) M.S (Malaysia)

Department of SurgeryInternational Medical School

Management and Science University

Contents Introduction Diagnosis and

investigations Aneurysmal diseases and

dissectionThoracic aortic aneurysmAortic dissectionAAAOthers

Acute arterial occlusion Buerger disease Inflammatory arteritis and Arterial diseases of upper

limb

Diseases of venous systemIntroductionVTESVTVaricose VeinsChronic Venous insufficiency

Lymphedema

Arterial diseases … introduction

Peripheral arterial disease occurs in 12 percent of adult population.

It affects around 8-10 million people in US.

Most common presentation is intermittent claudication.

These patients have significantly higher risk of death compared with healthy controls of similar age.

Arterial diseases … diagnosis

Vascular history: Intermittent claudication (describe) TIA or stroke symptoms Mesenteric ischemia Previous vascular intervention Up to 30 percent of vascular

patients are diabetics. Most of them are smokers.

Arterial diseases … diagnosis

Vascular examination: Pulses (2+, 1+, 0 or 3+) Foot: for pallor on elevation, rubor on

dependency, ulceration or gangrenous toes.

Upper limbs for signs of ischemia or ulcerations.

Ankle-brachial index ABI (n>1) Aortic (aneurysm), carotid (bruits) and

vascular graft (stenosis) examination.

Arterial diseases … investigations

Duplex Scan (B-mode and Doppler US)- Carotid arteries stenosis- Bypass grafts of lower limbs- Abdominal aorta- DVT

Segmental pressures (ABI) Angiography,

- invasive (arterial access)- contrast … renal, drug interaction and allergy.- hydration status of the patients

CT-Angiography MR-Angiography

Arterial diseases … medical management …

Smoking cessation (reduce 10-mortality 54% 18%)

Control blood pressure (stroke and coronary events)

Reducing blood lipid levels Correction of elevated homocysteine level

(elevated in 60% of vascular patients) Tight control of blood sugar Exercise programs (24% reduction in

cardiovascular mortality) (30 minutes, 3 times/week)

Others: cilostazol (intermittent claudication), Aspirin and Clopidogrel.

Aneurysmal disease … introduction

Aneurysm: focal dilatation of an artery greater than 1.5 times its normal diameter.

Classification:Shape: fusiform, spindle-shaped, saccularWall constituents: true (by wall of the artery) false or pseudo (partly by wall and partly by adjacent structures.Etiology: dissecting, mycotic or traumatic.

Aortic aneurysm … Thoracic Incidence is estimated to be 5.9 per 100,000 per

year. Etiology:

- nonspecific medial degeneration (fragmentation of elastic fibers and loss of smooth muscles) most common.- Aortic dissection, two channels, weak outer layer.- Genetic disorders> Marfan syndrome, 75-85% have ameurysm.> Ehlers-Danlos syndrome, esp. type IV> Familial aortic aneurysm> Bicuspid aortic valves- Post-stenotic dilatation- Infection- Aortitis (Takayasu, Giant cell arteritis, Rheumatoid aortitis)- Traumatic

Clinical manifestations Asymptomatic, incidentally discovered Signs and symptoms are related to the segment

involved.1. Local compression and erosion

pain, radiate to neck and jaw (mimicking angina) airways compression hoarseness of voice dysphagia, hematemesis

2. Symptoms related to aortic valve insufficiency widened pulse pressure diastolic murmur heart failure

3. Distal embolization, to intercostals, visceral, renal or lower limbs.

4. Rupture

Investigations Plain x-ray, CXR, AXR and spine. Echocardiography (transthoracic or

trans-esophageal) CT Angiography MR Angiography Aortography (rarely used) and

cardiac catheterization

Treatment Aim is to prevent fatal rupture. Indication for surgery:

5-6 cm sizerate of dilatation > 1 cm/yearpatients with connective tissue disorders.

Pre-operative workupCardiacPulmonaryRenal

Surgical options:Resection of aneurysmal sac, replacement by a graft, reconstruction of branches.

Aortic dissection … Thoracic It’s a progressive separation of the aortic

wall layers, usually occurs after a tear in the intema and inner media, resulting in formation of two or more channels.

5-10 patients/million/year. If not treated, 50% die within 24 hours and

60% die within a month. Risk factors:

- smoking- hypertension- atherosclerosis- hypercholesterolemia- connective tissue disorders.

Clinical manifestations Sever chest pain radiate to the back

(tearing) Acute heart failure and dysponea due to

involvement of aortic valve. Cardiac tymponade Rupture shock and death Depending on vessels involved

- coronaries ischemic pain- carotid stroke- celiac liver failure- renal renal failure - ischemic limbs

Investigations Diagnosis usually delayed, 39% diagnosed

only after 24 hours. Clinical suspicion index is the critical point. Discrepancy between extremity

pulse/pressure is classic. CTA MRA Echocardiography Coronary angiogram in selected patients

Treatment Blood pressure control (permissive

hypotension 60-70 mmHg) Analgesia. According to location:Proximal aorta urgent graft replacement of

ascending aorta.Distal aorta pharmacotherapy

stabilization surgery.Endovascular treatment can be considered.

Abdominal aortic aneurysm (AAA)

AAA usually grows at a rate of 0.4 cm/year Risk of rupture is related to diameter of the

aneurysm.5 cm 3-5%/year7 cm 19%/year Intervention is indicated for all aneurysm 5

cm or above.

Abdominal aortic aneurysm (AAA)

Male and female are equal Progressive loss of elastin with increased

metalloprotease activity. Proteolysis and inflammation are the

driving forces in AAA. Normal 12 % elastin, in aneurysm only 1 % Family history is significant, For parents with AAA, up to 29% of

siblings found to have AAA.

Abdominal aortic aneurysm (AAA)

Mostly asymptomatic, indecently discovered.

Abdominal and low back pain GI bleed Pulsatile mass in the epigastric regionDiagnosis:1. USG2. CTA Treatment:Surgery, aneurysm opened longitudinally,

thrombus evacuated, graft is inserted, end-to-end anastomosis.

EVAR

Abdominal aortic aneurysm (AAA)

Post-operative complications: Pneumonia Ileus Renal failure Lower limb ischemia Colonic ischemia Spinal cord ischemia Graft infection

Ruptured Abdominal aortic aneurysm (RAAA)

Mortality is 50% Most patients die at home before reaching

the hospital Most patients are unaware of having AAA Sudden onset of sever abdominal or back

pain. Fainting Shock Once diagnosis made operation theater

Other aneurysms Iliac artery aneurysm Popliteal aretery aneurysm Femoral artery aneurysm Hepatic artery aneurysm Splenic artery aneurysm Renal artery aneurysm

Acute arterial occlusionLower limbs

One of the most common vascular emergency of lower limbs

Causes:1. Arterial embolism2. Thrombosis3. Graft occlusion4. Dissection5. Trauma (penetrating or blunt)

Manifestations5 P(s)1. Pain2. Pallor3. Paresthesia4. Paralysis5. Pulselessness+ Poikilothermia (perishing cold)Most common at femoral bifurcationTypically at foot and calf

treatment Immediate anticoagulation,

IV Heparin 800 u/kg as loading dose 180 u/kg/h infusion

Analgesia Oxygen Hydration

Surgery:femoral emobolectomy +/- faciotomyBypass

Endovascularcatheter directed thrombolysisthrombectomy (aspiration or mechanical)

Acute arterial occlusionMesenteric artery

GI blood supply is via CA, SMA and IMA Chronic or acute occlusion

Mortality 50-75% Early recognition is vital

Acute arterial occlusionMesenteric artery

Clinically:Acutesever abdominal pain, out of proportion to the findings.Sudden onset of bowel crampsbloody diarrheaChronic:abdominal anginaweight loss as patients afraid to eat

Diagnosis CTAMesenteric arteriography

Acute arterial occlusionMesenteric artery

Treatmentfluid resuscitationsystemic anticoagulationcorrect metabolic acidosisbowel resection if gangrene

Chronic revascularization, either by endartrectomy of bypass.

Acute arterial occlusionRenal artery

70% due to atherosclerosis Fibromuscular dysplasia of renal artery Renal artery aneurysm AVM

Presentation:Usually with hypertensionDiagnosis: Renal duplex scanCTAMRA

Acute arterial occlusionRenal artery

TreatmentMedical therapy:

antihypertensiveTransluminal balloon angioplasty and stentingSurgical revascularization

Inflammatory arteritis and vasculitides

Buerger Disease Takayasu arteritis

Giant cell arteritisBehcet diseasePolyarteritis nodosaRaynaud syndromeFibromuscular dysplasia

Buerger Disease(Thromboangiitis obliterans)

Progressive nonatherosclerotic segmental inflammatory disease that affects small and medium size arteries and veins of upper and lower limbs

Typically smokers men between 25-50 years There is thrombosis of vessels Start at age of 40, at foot, leg, arms or hands

with claudicating and ulceration Treatment is by stopping smoking

no role for bypass or graftingamputation of necrotic tissue and wound care

Takayasu Arteritis More case reports from

Japan ,India, South-east Asia, Mexico

No geographic restriction No race – immune Incidence-2.6/million/year-

N.America/Europe The incidence in Asia is 1

case/1000-5000 women.

Takayasu Arteritis Age 10-40 Primarily aorta Present with constitutional symptoms Fever, anorexia, weight loss, arthralgia and

malnutrition. With progression of disease patient presents with

vascular insufficiency- renal artery stenosis, retinopathy, aortic regurgitation, cerebrovascular symptoms, angina, congestive heart failure

ESR, CRP and WCC are elevated Treatment with steroids and cytotoxic agents Surgery (bypass) for advanced cases

Raynaud Syndrome Group of symptoms associated with peripheral

spasm mainly in upper limbs. Typically intermittent spasm in responds to

stimuli, like cold, smoking, or emotional stress. Color changes, pallor cyanosis redness 70-90% in women Diagnosis is clinically, angiogram for sever cases No curative treatment Life style modification, gloves, hand warmers,

stop smoking, avoid vibrating instruments CCB Surgery for debridement and ulceration

CHRONIC VENOUS INSUFFICIENCY

(CVI)

CVI Occurs when the vein valves become dysfunctional

and impairs venous blood return.

Affects up to 20% of adults.

By age 50 ~40% of women and 20% of men have significant vein problems.

More people lose work time from vein disorders then from artery disease. 1.

RISK FACTORS Advancing age Family history of venous disease Ligamentous laxity (eg, hernia, flat fleet) Prolonged standing Increased body mass index Smoking Sedentary lifestyle Lower extremity trauma Prior venous thrombosis (superficial or deep) Arteriovenous shunt Hereditary conditions High estrogen states Pregnancy 2.

PROGRESSION OF VEIN DISEASE

ASYMPTOMATIC SUPERFICIAL VENOUS DILATATION

Telangiectasias (intradermal)

Reticular veins (subdermal)

PROGRESSION OF VEIN DISEASE

ASYMPTOMATIC VS SYMPTOMATIC VARICOSE VEINS (subcutaneous)

PROGRESSION OF VEIN DISEASE

Leg edema

PROGRESSION OF VEIN DISEASE

Skin changes Hyperpigmentation

PROGRESSION OF VEIN DISEASE

Skin changes Stasis dermatitis

PROGRESSION OF VEIN DISEASE

Skin changes Corona phlebectatica

a. venous cups (veins)

b. telangiectasias

c. reticular veins

d. stasis spots (capillaries)

PROGRESSION OF VEIN DISEASE

Lipodermatosclerosis a form of panniculitis just above the

ankles. 9.

PROGRESSION OF VEIN DISEASE

Venous stasis ulceration(s)

EVALUATION VENOUS DOPPLER ULTRASOUND Evaluate for deep and superficial

venous thrombosis. Evaluate for incompetent veins with significant reflux disease. Evaluate for incompetent perforating veins and tributaries.

MANAGEMENT OF CVI LEG ELEVATION – heart level for 30 minutes 3-4

times daily improves micro-circulation reduces edema, and promotes healing of venous ulcers.4.

EXERCISE – daily walking and simple ankle flexion exercises.

MANAGEMENT OF CVI Compression therapy - avoid contraindications such as

cellulitis or significant arterial occlusive

disease.

MANAGEMENT OF CVI – COMPRESSION THERAPY

Compression bandages – elastic or non-elastic with single or multi-layers.

MANAGEMENT OF CVI – COMPRESSION STOCKINGS

MANAGEMENT OF CVI PNEUMATIC COMPRESSION THERAPY

MANAGEMENT OF CVI – SKIN CARE

Skin cleansing – wash with a mild non-soap cleanser (e.g. Dove, Olay, Caress).

Emollients – provides a film of oil to lubricate the skin (e.g. Vaseline, Lubriderm, Aveeno).

Barrier preparations – physically block chemical irritants and moisture.(e.g. Zinc oxide, Vaseline).

Topical corticosteroids – often used to treat stasis dermatitis. 4.

MANAGEMENT OF CVI – VENOUS STASIS ULCERS

Surgical debridement – used to remove devitalized tissue.

Enzymatic agents – used to break down necrotic tissue (e.g. Santyl).

Growth factors – synthesized by many cell types such as platelets, neutrophils, and epithelial cells (e.g. Regranex).

Bioengineered tissue – used for a variety of non-healing ulcers (e.g. Apligraf, Dermagraft).

Skin grafting – an option for non-healing ulcers. 4.

MANAGEMENT OF CVI – ABLATION THERAPY

Indications – patients with persistent signs/symptoms of venous disease after a minimum of 3 months of medical therapy (e.g. compression) and documented reflux (e.g. >0.5 seconds of reflux GSV).

Absolute contraindications – acute DVT or phlebitis and pregnancy. 5,6.

Radiofrequency versus laser endovenous ablation therapy.

MANAGEMENT OF CVI - SCLEROTHERAPY

DETERGENT AGENTS - Sodium tetradecyl sulfate - Polidocanol OSMOTIC AGENTS - Hypertonic saline - Glycerin

DVT Epidemiology and Etiology

Annual incidence of venous thromboembolism (VTE) is 1/1000

DVT accounts for one half of VTE Majority of lower extremity DVT arise

from calf veins but ~20% begin in proximal veins

About 20% of calf-limited DVTs will propagate proximally

DVT – VTE Risk Factors Malignancy Surgery Trauma Pregnancy Oral

contraceptives or hormonal therapy

Immobilization Inherited

thrombophillia

Presence of venous catheter

Congestive failure Antiphospholipid

antibody syndrome

Hyperviscosity Nephrotic

syndrome Inflammatory

bowel disease

DVT – Clinical Presentation Classically = calf pain, tenderness,

swelling, redness and Homan’s sign› Overall sens/spec = 3-91%› Unreliable for diagnostic decisions

Wells developed and tested a clinical prediction model for DVT

Wells PS, Anderson DR, Bormanis J, et al. Value of assessment of pretest probability of deep-vein thrombosis in clinical management. Lancet 1997;350 (9094):1795-8.

DVT – Imaging Available imaging and ancillary

tests:› Compression US – first line

test, high sens/spec› Venography – gold standard› MRI – Lower quality evidence

only at present› Impedance

plesmythography – not in US› Complete lower extremity

US – experimental

PE Epidemiology and Etiology

100-200,000 deaths per year due to PE Most PE arise from lower extremity DVT In patients with DVT, 40-60% will have

a PE on V/Q scanning

“Pulmonary embolus is not a disease. It is a complication of DVT.” Ken Moser MD

PE Clinical Presentation

Dyspnea, pleuritic pain and cough most common symptoms

Tachypnea, rales and tachycardia most common signs

ABG limited value for diagnosis EKG and CXR often abnormal, but

usually lacking specificity to aid diagnosis

PIOPED Study. JAMA. 1990;263(20):2753-59. Stein PD, Goldhaber SZ, Henry JW. Chest 1995;107:139-43

CXR FINDINGS Hampton’s Hump: -wedge-shaped configuration at

lung periphery due to infarcted lung

Westermark sign: -pulmonary oligemia

PE – Imaging Studies PIOPED study quantified the value of

V/Q scans in diagnosing PE› Normal/near-normal scans exclude PE in

low-moderate risk patients› High probability scans confirm PE in

moderate-high risk patients› Drawbacks: more difficult test and 73%

patients had indeterminate scans LE compression US showing DVT helps

diagnostically, but a negative study insufficient to exclude VTEPIOPED Study. JAMA. 1990;263(20):2753-59

PE – Helical CT (CTA) Eng performed a systematic review (SR) of all

studies & SRs on CTA prior to 2003› Only 1/6 SRs and 3/8 primary studies found CTA >90%

sensitive for PE In a similar SR in 2005 Roy concluded

› Negative CTA could safely exclude PE in low risk patients

› Negative LE US plus negative CTA could exclude PE in moderate risk patients

At the time of those SRs no studies of faster multidetector CTA (MDCT) were available

Eng J, Krishnan JA, Segal JB, et al. AJR 2004;183(6):1819-27. Roy PM, Colombet I, Durieux P, et al. BMJ 2005;331(7511):259.

VTE – Other Therapy Issues Anticoagulation same for DVT & PE Thrombolysis - risk/benefit uncertain;

clinical outcomes generally not improved Vena cava filters

› Contraindication to anticoagulation› Rarely survivors of massive PE› Rare patients with recurrent VTE on

adequate anticoagulation› Prophylaxis in certain high risk patients

Lymphedema

Lymphedema An abnormal accumulation of protein-

rich fluid in the interstitium, causing chronic inflammation and reactive fibrosis of the affected tissues

Usually in an extremity, but can also occur in the head, neck, genitals, and abdomen

Lymphedema Affects 1% of the American population

(2.5 million people) Still poorly understood in the medical

community Largest cause of lymphedema in the

world is Filariasis (considered secondary lymphedema)

Filariasis is a parasitic infiltration into the lymphatics that is very common in third world countries (affects 90 million people)

Types of Lymphedema Primary lymphedema is a result of

lymphatic dysplasia.› May be present at birth› Can develop later in life without known

cause Secondary lymphedema is much

more common.› Result of surgery, radiation, injury, trauma,

scarring, or infection of the lymphatic system

Secondary Lymphedema

Primary Lymphedema

Lymphedema

Primary lymphedema Lymphangiodysplasia – general malformation Hypoplasia – fewer than normal # of lymph

collectors Aplasia – absences of collectors in a distinct

area Milroy's Disease is congenital lymphedema

evident at birth Meige’s Syndrome is primary lymphedema

onset at puberty (lymphedema praecox) Lymphedema Tardum is primary lymphedema

onset after age 35

Secondary lymphedema There is a known cause for the presence of

edema Surgery: breast cancer, melanoma,

prostate/bladder cancer, lymphoma, ovarian cancer, hip replacements

Radiation therapy Trauma – scarring, crush injury Infection CVI Obesity Self-induced

http://www.lymphedema-therapy.com/RED2.gif

Angiosarcoma Can develop after long-standing lymphedema “Stewart - Treves Syndrome” Angiosarcoma after mastectomy was first

described in 1948 by Stewart and Treves Signs: reddish-blue and blackish-blue lumps that

rapidly increase in size, bleed easily and ulcerate at an early stage

Very rare & poor prognosis

Lymphedema is a disease. All other edemas are symptoms.There is no cure for lymphedema.There is only management.

Diagnosis of

Lymphedema

Physical exam and history are most important.

Characteristics of Benign Lymphedema

Slow onset, progressive Pitting in early stages Cellulitis is common Rarely painful but discomfort is common Skin changes – hyperkeratosis, papillomas,

lichenification Ulcerations are unusual Starts distally

› Toes square, positive Stemmer’s sign› Dorsum of foot “buffalo hump”› Loss of ankle contour› Asymmetric if bilateral

History What is the reason for the swelling? How long has the extremity been swollen? How fast did the edema progress/develop? What are the underlying diseases? Is there pain? Other conditions? Other treatments? Medications?

Inspection Location of swelling (distal or proximal) Any skin changes Lymphatic cysts, fistulas Ulcers Scars or radiation burns Papillomas Hyperkeratosis

Palpation Temperature – indicative of infection Stemmer sign is (+) when a thickened cutaneous

fold of skin at the dorsum of the toe or finger cannot be lifted or is difficult to lift. Positive Stemmer’s sign is indicative of lymphedema.

Skin folds Pitting Fibrosis Muscular status

Diagnostic Tests

Direct lymphography: invasive, oily contrast injected into a surgically exposed lymphatic vessel. Damaging. Has been replaced by CT, MRI, US.

Lymphoscintigraphy: noninvasive, assesses dynamic process in superficial and deep lymphatics

CT MRI These tests are often not performed due to lack

of clinical importance

Differential Diagnosis

Lipedema Chronic venous insufficiency Acute deep vein thrombosis Cardiac edema Congestive heart failure Malignancy/active cancer Filariasis Myxedema Complex regional pain syndrome

Lipedema Mainly in women Bilateral, symmetrical edema

from iliac crest to ankles Dorsum of feet never involved (-) Stemmer’s sign Little or no pitting No cellulitis Painful to palpation Bruise easily

CVI Gaiter distribution Non-pitting Brawny Hemosiderin staining Fibrosis of subcutaneous

tissue Atrophic skin

Acute DVT Sudden onset Unilateral Painful Cyanosis (+) Homan’s sign Potentially lethal (PE) Diagnosis with venous doppler Not treatable with PT

Cardiac edema Right heart insufficiency Greatest edema distally Always bilateral Pitting Complete resolution with elevation No pain May treat with PT if cleared by

Cardiologist

Congestive Heart Failure Bilateral heart failure Pitting edema Orthopnea, paroxysmal noctural

dyspnea, DOE Jugular venous distension Diagnosis with physical exam, chest x-

ray, cardiac echo

Malignant lymphedema

Pain, paresthesia, paralysis Central location, proximal onset Rapid development, continuous progression Swelling and nodules in supraclavicular fossa Hematoma-like discoloration (angiosarcoma) Ulcers and non-healing open wounds Recurrent malignancy

Filariasis

Prevalent in 3rd world countries; Can still be treated successfully with CDT. Most therapists in the US will never encounter Filariasis.

Lymphedema Treatment Options

Pneumatic compression pump Surgery Complete decongestive therapy (CDT) Elastic support garments Medications

Pneumatic Compression Pumps

Advantages:1. Can be used at home by patients2. Fast application3. Financially lucrative for DME vendors ($4000 per pump)

Surgery

Microsurgical techniques Liposuction Debulking/Reduction procedures

Skin and Nail Care Eliminate bacteria and fungal growth

by using medicated powders, hydrocortisone cream where indicated.

Reduce the risk of infection by avoiding injury, cleaning all injuries immediately, calling MD at first sign of infection.

QUESTIONS

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