Buergers-Disease-Understanding-Thromboangiitis-Obliterans.pptx

Medical content

1. Buerger's Disease: Understanding Thromboangiitis Obliterans

2. What is Buerger's Disease? Buerger's disease, also known as Thromboangiitis Obliterans, is a distinct vascular disorder with severe implications for affected individuals. Non-atherosclerotic Inflammation It's a nonatherosclerotic, segmental inflammatory disease that affects small and medium arteries and veins, predominantly in the limbs. This distinction from atherosclerosis is crucial for diagnosis and understanding. Affected Demographics Typically, it strikes young adults aged 20-45. Historically, it has shown a strong predominance in males, although this trend is shifting with changing tobacco use patterns. Affected areas It always starts in the lower limbs. It may start on one side and later on the other side. Upper limb involvement occurs only after the lower limbs are diseased.

3. Risk Factors and Epidemiology The overwhelming risk factor for Buerger's disease is tobacco use, with specific demographics showing higher prevalence. Tobacco Use: Strongly linked to all forms of tobacco, including cigarette smoking, chewing tobacco, and possibly marijuana. This is the single most critical factor. Age and Gender: Predominantly affects patients under 45, rarely seen in the elderly or children. Historically, a male predominance (approx. ratio 3:1) has been observed, but this is shifting due to increased smoking rates in women. Geographical and Ethnic Predisposition: More common in the Middle East, Far East, and among Ashkenazi Jewish populations, suggesting genetic or environmental influences alongside tobacco use. No Other Major Risk Factors: Unlike atherosclerosis, Buerger's disease is not associated with traditional cardiovascular risk factors like hypertension, hyperlipidemia, or diabetes.

4. Pathophysiology in Brief Understanding the underlying mechanisms of Buerger's disease is crucial for differentiating it from other vascular conditions. 1 Inflammation and Thrombosis The disease is characterised by acute inflammation and thrombosis of small and medium arteries and veins. This process involves a distinct polymorphonuclear leukocyte infiltration. 2 Vessel Wall Integrity Crucially, the vessel wall inflammation spares the internal elastic lamina, a feature that distinguishes it from atherosclerotic changes. 3 Segmental Occlusions It leads to segmental occlusions of the vessels, with characteristic "skip areas" where segments of healthy vessel exist between diseased sections. 4 Corkscrew Collaterals A hallmark angiographic finding is the formation of "corkscrew" collateral vessels, which develop in an attempt to bypass the blocked arteries. 5 Immunologic Hypotheses Immunologic mechanisms are suspected to play a role, including hypersensitivity to tobacco components and the presence of anti-endothelial antibodies.

5. Key Symptoms and Clinical Presentation The symptoms of Buerger's disease often begin subtly but progress to debilitating pain and tissue damage, indicating severe vascular compromise. Ischemic Rest Pain Often the earliest and most distressing symptom, this severe pain occurs in the hands and feet even at rest, starting distally in the toes and fingers. Intermittent Claudication Pain in the limbs induced by exercise, which is relieved by rest, progresses to pain at rest as the disease advances and blood flow is further compromised. Raynaud's Phenomenon Characterised by episodes where fingers or toes turn white or blue upon exposure to cold or stress, indicating reduced blood flow. Migratory Superficial Thrombophlebitis Approximately 16% of patients experience this condition, involving recurrent inflammation and thrombosis of superficial veins, which often migrates to different locations. Ischemic Ulcers and Digital Gangrene In advanced stages, the lack of blood supply leads to painful, non-healing ulcers and tissue death (gangrene) in the digits, often requiring amputation.

6. Diagnostic Tools and Investigations A thorough diagnostic process is essential to confirm Buerger's disease and rule out other conditions. Clinical Assessment History: Detailed inquiry into tobacco use, symptom onset, and progression. Physical Exam: Careful examination for distal ischemic signs, ulcers, gangrene, and pulses. Non-Invasive Vascular Studies Doppler Ultrasound: Assesses blood flow and identifies occlusions. Ankle-Brachial Index (ABI): Compares blood pressure in ankles and arms to detect peripheral artery disease. Angiography Definitive Imaging: Provides visual evidence of segmental occlusions, absence of proximal atherosclerosis, and the characteristic corkscrew collateral vessels. Laboratory Tests Exclusionary Tests: Blood tests to rule out other vasculitides, autoimmune disorders (e.g., lupus, scleroderma), and hypercoagulable states (e.g., thrombophilia). Diabetes Screening: Crucial to exclude diabetes as a cause of peripheral vascular disease.

7. Treatment Options

8. Revascularisation and Perfusion Strategies Omentoplasty Omentoplasty involves surgically mobilising a pedicled omental flap and wrapping it around the affected limb. The richly vascularised omentum provides a new blood supply to ischaemic tissues, promoting angiogenesis and revascularisation. Profundaplasty Profundaplasty addresses blockages in the profunda femoris artery. By improving flow through this vessel, collateral circulation around the knee joint is enhanced, leading to better perfusion for the knee joint and improving the viability of below-knee amputation flaps. These revascularisation techniques are crucial in mitigating the severe effects of Buerger's disease, often delaying or preventing the need for limb amputation.

9. Adjunctive Therapies and Amputation Considerations Lumbar Sympathectomy Lumbar sympathectomy aims to increase cutaneous perfusion and promote ulcer healing by disrupting sympathetic vasoconstrictor tone. However, this procedure may divert blood flow from muscles to the skin, potentially exacerbating muscle ischaemia. Amputations Amputations are performed at various levels—typically below-knee or above-knee—based on the site, severity, and extent of vessel occlusion. The goal is to remove non-viable tissue and prevent the spread of infection. Ilizarov Method The Ilizarov method, primarily for bone lengthening, has shown promise in alleviating rest pain and claudication by inducing neo-osteogenesis and improving overall limb blood supply, thereby potentially reducing the need for amputation. While some surgical approaches carry inherent risks, careful patient selection and a multidisciplinary approach are paramount for optimising outcomes in patients with Buerger's disease.

10. Complications: Ulcers and Gangrene The advanced stages of Buerger's disease are marked by severe and painful complications that can lead to permanent disability and limb loss. Chronic Ischemic Ulcers: Persistent lack of blood flow leads to excruciatingly painful, non-healing ulcers, typically on the fingers and toes. These are prone to infection. Digital Gangrene: Progressive tissue necrosis, resulting in digital gangrene. This tissue death is irreversible and often requires amputation of the affected digits or even parts of the limb. Secondary Infections and Sepsis: Untreated ulcers and gangrene can become infected, leading to local cellulitis, osteomyelitis, and potentially life-threatening systemic sepsis. Limb Amputation: Without strict smoking cessation and early intervention, the progression of ulcers and gangrene frequently necessitates limb amputation, causing significant morbidity.