Aortoiliac disease

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Editors-In-Chief: Alexandra Almonacid M.D. [1]and Jeffrey J. Popma M.D. [2]

Overview

Aortoilliac disease also known as Aorotilliac occlusive disease or Leriche Syndrome is a type of Peripheral Arterial Disease (PAD) caused by occlusion of the infrarenal aorta and beyond. It can include the Common Illiac artery and it's branches. Depending on it's underlying cause it can present acutely or chronically. Acute causes include thrombosis and embolism, while chronic causes include atherosclerotic plaque formation

Classification

Morphological Stratification of Iliac Lesions-ACC/AHA Guidelines

• TASC Type A iliac lesions
  • Single stenosis less than 3 cm of the CIA or EIA (unilateral/bilateral)
• TASC Type B iliac lesions
  • Single stenosis 3 to 10 cm in length, not extending into the CFA
  • Total of 2 stenosis less than 5 cm long in the CIA and/or EIA and not extending into the CFA
  • Unilateral CIA occlusion
• TASC Type C iliac lesions
  • Bilateral 5 to 10 cm long stenosis of the CIA and/or EIA, note extending into the CFA
  • Unilateral EIA occlusion not extending into the CFA
  • Unilateral EIA stenosis extending into the CFA
  • Bilateral CIA occlusion
• TASC Type D iliac lesions
  • Diffuse, multiple unilateral stenosis involving the CIA, EIA and CFA (usually more than 10 cm long)
  • Unilateral occlusion involving both the CIA and EIA
  • Bilateral EIA occlusions
  • Diffuse disease involving the aorta and both iliac arteries
  • Iliac stenosis in a patient with an abdominal aortic anuerysm or other lesion requiring aortic or iliac surgery

Diagnosis

• MR angiography
  • Gadofosveset-enhanced MR angiography showed significant improvement (P < .001) compared with unenhanced MR angiography for diagnosis of clinically significant aortoiliac occlusive disease ( 50% stenosis) .
  • The improvement in diagnostic efficacy compared with unenhanced MR angiography was clearly demonstrated. There was an improvement in overall accuracy, sensitivity, and specificity.
• CT Angiography
  • CT angiographic examination is less invasive and less expensive than conventional angiography
  • Improves resolution with decreased contrast load and acquisition time without increasing radiation exposure

Indications for Revascularization

• Relief of symptomatic lower extremity ischemia, including claudication, rest pain, ulceration or gangrene, or embolization causing blue toe syndrome
• Restoration y/o preservation of inflow to the lower extremity in the setting of pre-existing or anticipated distal bypass
• Procurement of access to more proximal vascular beds for anticipated invasive procedures. Occasionally revascularization is indicated to rescue flow-limiting dissection complicating access for other invasive procedures

Technical Issues

• Endovascular Access
  • Ipsilateral femoral artery
  • Contralateral femoral artery
  • Brachial artery: In patients with flush occlusions at the aortic bifurcation
• Multiple access sites may be required for successful treatment:
  • Bilateral femoral
  • Femoral/brachial

Treatment Options

Interventional Management of Iliac Lesions

PTA

• Endovascular treatment of iliac stenoses
  • High technical success rates
  • Low morbidity.
• Iliac PTA/stenting
  • High rates of patency
  • Improvement in functional outcome for the individual patient
• Stent placement
  • Balloon expandable stent: Useful in Ostial Lesions
    • Greater radial force
    • Allow greater precision for placement
  • Self-expandable stent
    • Longer lesions in which the proximal vessel maybe several millimeters larger than the distal vessel
    • Used predominantly in common iliac artery orificial occlusions

Complications

• Intraoperative complications
• Dissection
• Extravasation
• Arterial rupture
• Postoperative complications
• Pseudoaneurysm formation at the access site
• Distal embolization
• Hematoma

Prognosis

• Ideal Iliac PTA Lesions
  • Stenotic lesion
  • Non-calcified
  • Discrete (< 3cm)
  • Patent run – off vessels (> 2)
  • Non- diabetic patients
• Predictors of long-term failure
  • Clinical status: CLI vs claudicant
  • Smoking
  • Women?
  • Vessel diameter < 8mm
  • Outflow status
  • Lack of antiplatelet regimen
  • Number of stents
  • Occlusion vs. stenosis

Historical Perspective

• Aortoilliac disease/Leriche's syndrome was first described by Dr. Robert Graham, a Scottish surgeon, in 1841.
• The symptoms of Aortoillliac disease described together as a syndrome was first discovered by Dr. Rene Leriche a French physician in 1940.
• In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
• In 1940, Dr. Leriche performed the first surgery to treat Aortoilliac disease/ Leriche syndrome.

Classification

• Aortoilliac disease may be classified according to ACC/AHA guidelines into 4 subtypes/groups:

• TASC Type A iliac lesions
  • Single stenosis less than 3 cm of the CIA or EIA (unilateral/bilateral)
• TASC Type B iliac lesions
  • Single stenosis 3 to 10 cm in length, not extending into the CFA
  • Total of 2 stenosis less than 5 cm long in the CIA and/or EIA and not extending into the CFA
  • Unilateral CIA occlusion
• TASC Type C iliac lesions
  • Bilateral 5 to 10 cm long stenosis of the CIA and/or EIA, note extending into the CFA
  • Unilateral EIA occlusion not extending into the CFA
  • Unilateral EIA stenosis extending into the CFA
  • Bilateral CIA occlusion
• TASC Type D iliac lesions
  • Diffuse, multiple unilateral stenosis involving the CIA, EIA and CFA (usually more than 10 cm long)
  • Unilateral occlusion involving both the CIA and EIA
  • Bilateral EIA occlusions
  • Diffuse disease involving the aorta and both iliac arteries
  • Iliac stenosis in a patient with an abdominal aortic anuerysm or other lesion requiring aortic or iliac surgery

Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• The pathogenesis of aortoilliac disease is characterized by atherosclerotic plaque buildup, Thrombosis or an embolus.
• The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
• On gross pathology, cholesterol plaques, fatty streaks, and areas of ulceration and hemorrhage are characteristic findings of atherosclerosis and thrombus formation in aortoilliac disease.
• On microscopic histopathological analysis, foam cells, necrotic core, fibrous cap and inflammatory infiltrate are characteristic findings of atherosclerosis and thrombus formation in aortoilliac disease.

Clinical Features

Clinical features of aortoilliac disease include:

• Claudication of legs and buttocks
• Pallor of lower limbs
• Numbness of lower limbs
• Weakness and soreness of lower limbs
• Loss of femoral pulses
• Erectile dysfunction
• Atrophy of affected muscles in the lower limb

Differentiating Aortoilliac Disease from other Diseases

• Aortoilliac disease must be differentiated from other diseases that cause lower limb claudication, lower limb weakness/atrophy, and loss of femoral pulses such as:

• Peripheral artery disease of the Lower limbs
• Compartment syndrome
• Chronic venous insufficency

Epidemiology and Demographics

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Aortoilliac disease is more commonly observed among patients aged 30-60 years old.
• Aortoilliac disease is more commonly observed among middle aged and elderly patients.

Gender

• Males are more commonly affected with aortoilliac disease than Females.
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [disease name].

• [Disease name] usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors

• Common risk factors in the development of aortoilliac disease are smoking, diabetes mellitus, dyslipidemia, and hypertension.

Natural History, Complications and Prognosis

• The majority of patients with peripheral arterial disease remain asymptomatic for [duration/years].
• Early clinical features include intermittent claudication, decreased lower limb pulses, and erectile dysfunction.
• If left untreated, [#%] of patients with aortoilliac disease may progress to develop critical limb ischemia, muscle atrophy, and poor wound healing.
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of aortoilliac disease is made when 3 of the following diagnostic criteria are met:

• claudication
• weak lower limb pulses
• erectile dysfunction
• [criterion 4]

Symptoms

• Peripheral arterial disease may start out asymptomatic. Symptoms depend on the arteries affected.
• Symptoms of aortoilliac disease may include the following:

• caludication of lower limbs
• erectile dysfunction
• cold extremities
• weight loss
• pain in buttocks

Physical Examination

• Patients with aortoilliac disease can appear normal or distressed in appearance.
• Physical examination may be remarkable for:

• weak lower limb pulses
• atrophy of lower limb
• cold peripheries
• pallor
• cyanosis
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with aortoilliac disease.

• Other laboratory findings consistent with the diagnosis of Peripheral artery disease including aortoilliac disease include abnormal lipid panel and abnormal inflammatory markers.

Imaging Findings

• CT angiography is the imaging modality of choice for aortoilliac disease.
• On CT angiography, aortoilliac disease is characterized by occlusion of the Common Illiac, External Illiac, or Common Femoral Arteries.
• Doppler Ultrasound may demonstrate decreases blood flow in the Common Illiac, External Illiac, or Common Femoral Arteries.
• Abdominal ultrasound may also be used to aid in diagnosis

Other Diagnostic Studies

• Peripheral Arterial disease including aortoilliac disease may also be diagnosed using Ankle Brachial Index.
• Ankle Brachial Index < 0.9 is diagnostic of occlusive disease .

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

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