History

Fact Explanation
Introduction The condition also called as Buerger's disease and characterized by non atherosclerotic segmental inflammation of small and medium sized arteries, thrombophlebitis of the superficial or deep veins and Raynaud’s syndrome. The core aetiology of the disease is tobacco smoking though few cases are reported following chewing of tobacco and marijuana. So the disease is typically seen in smoking young males and due to higher incidence of smoking in females currently females are also suffering from the illness. It does not occur in paediatric and geriatric population. The progression of the diseases is directly related to the continued consumption of tobacco. Though the exact pathophysiology is yet to be disclosed current evidence shows inflammatory thrombi formation which could be due to an abnormalities in immunoreactivity. In summary this is an irreversible disease causing ultimate limb amputation due to the inadequate perfusion of end organs which causes ischaemia and finally gangrene. Although the disease is commonest at extremities it can affect cerebral, coronary, renal, retinal and mesenteric circulation as well[1][2].
Intermittent claudication Intermittent claudication is the ischaemic pain occurring in exerting limbs due to poor perfusion of the musculature during exertion due to arterial insufficiency and it is reproducible with same exertion and relieved by resting. So during the initial presentation they might complain pain in the foot or calf with walking and reliving with resting. If the exertion continued through the pain the pain may ascends to proximal muscle groups in extensive occlusion or might get worsen. After the resting the pain can be reproduced when walking similar distance. Similar phenomena would occur when exerting the upper limbs. Walking distance will depend on the severity of the arterial occlusion. Type of symptoms of claudication will differ according to the site of the occlusion. Eg: In aortoiliac obstruction claudication will present in both buttocks, thighs and calves and patient will suffer from impotence. In iliac obstruction there will be unilateral claudication in the thigh and calf and sometimes in buttock. In femoropopliteal there will be unilateral claudication in the calf. In a distal obstruction claudication will present in calf and foot[3][4][8].
Rest pain With the ischemia, patient will have continuous pain at rest. pain will be more at night, on lying down/ elevation( due to further reduction of blood supply with the effect of gravity). It characteristically relief with hanging down. So patient will used to sleep on chairs/ on a bed will hanging legs for a long time[3][5][6].
Coldness and numbness over the limb moderate to severe ischemia can present with these symptoms[7][8].
Ulceration and discoloration With severe ischemia ulceration can be occur. Ulcers are common on the dorsum of the feet, on the shins and around the malleoli. In gangrene (dry gangrene) there will be discoloration of the limb[7][9].
Features suggestive of arterial occlusion With this condition arteries in all systems can affect causing various symptoms. Eg: Brain strokes can occur giving features of paralysis, paresthesia and cranial nerve palsy. In spinal cord, features of infarction like limb paralysis, paresthesia, bladder/ bowel dysfunction. In bones, ischemic pain at site of the joint/back pain and fractures following long bone infarction can be seen. Myocardial infarctions can occur following hypoperfusion of the myocardium( chest pain, difficulty in breathing, dizziness). In lungs there will be shortness of breath and pleuritic type chest pain. In mesentry, acute abdominal pain will be the presentation. In digits, painful fingers and toes with small bone infarction. In kidneys infarction of medulla with papillary necrosis may lead to fail in concentrating urine causing high urine out put, dehydration and nocturnal enuresis. Chronic liver failure with micro infarction causing loss of appetite, yellowish discoloration of eyes. Splenic infarction leads to recurrent infections like upper/ lower respiratory tract infections and diarrheal illnessess[10][11].
development of severe pain and bluish discoloration over tip of fingers after exposure to cool water This Raynaud’s syndrome caused by peripheral digital ischemia[12][13].
Features suggestive of superficial thrombophlebitis Patient will have localized pain. this will gradually progress with the time. Areas will be more painful to the touch[13][14].
Features suggestive of deep thrombophlebitis (deep vein thrombosis) Patient will suggestive of sudden onset pain, swelling, redness and warmth of the limbs[15][16].
Features suggestive of venous embolism. Patient can develop feature suggestive of pulmonary embolism such as sudden on set difficulty in breathing, chest pain, dyspnoea[1][16].
History of tobacco smoking/ chewing. The exposure to tobacco is mandatory for the diagnosis as the sole risk factor is that. Rarely there will be a association with use of marijuana[4][14].
References
  1. SZUBA A, COOKE JP. Thromboangiitis obliterans. An update on Buerger's disease. West J Med [online] 1998 Apr, 168(4):255-260 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1304949
  2. MCLOUGHLIN GA, HELSBY CR, EVANS CC, CHAPMAN DM. Association of HLA-A9 and HLA-B5 with Buerger's disease. Br Med J [online] 1976 Nov 13, 2(6045):1165-1166 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1689618
  3. VIJAYAKUMAR A, TIWARI R, KUMAR PRABHUSWAMY V. Thromboangiitis Obliterans (Buerger's Disease)--Current Practices Int J Inflam [online] 2013:156905 [viewed 28 October 2014] Available from: doi:10.1155/2013/156905
  4. CASTLEDEN WM, FAULKNER K, HOUSE AK, WATT A. Haemoglobin, smoking and peripheral vascular disease J R Soc Med [online] 1981 Aug, 74(8):586-590 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1438930
  5. DE HARO J, ACIN F, BLEDA S, VARELA C, ESPARZA L. Treatment of thromboangiitis obliterans (Buerger's disease) with bosentan BMC Cardiovasc Disord [online] :5 [viewed 28 October 2014] Available from: doi:10.1186/1471-2261-12-5
  6. MATSUMOTO H, YAMAMOTO E, KAMIYA C, MIURA E, KITAOKA T, SUZUKI J, YAMAMOTO K, DEGUCHI J, HIGASHI M, TAMARU JI, SATO O. Sural Artery Bypass in Buerger's Disease: Report of a Case Ann Vasc Dis [online] 2012, 5(2):199-203 [viewed 28 October 2014] Available from: doi:10.3400/avd.cr.11.00090
  7. RYU SW, JEON HJ, CHO SS, CHOI RM, YOON JS, KO HS, LEE JD. Treatment of digit ulcers in a patient with Buerger's disease by using cervical spinal cord stimulation -a case report- Korean J Anesthesiol [online] 2013 Aug, 65(2):167-171 [viewed 28 October 2014] Available from: doi:10.4097/kjae.2013.65.2.167
  8. GASKELL P. The importance of penile blood pressure in cases of impotence Can Med Assoc J [online] 1971 Nov 20, 105(10):1047-1051 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1931242
  9. DAS S, MAITI A. Acrocyanosis: An Overview Indian J Dermatol [online] 2013, 58(6):417-420 [viewed 28 October 2014] Available from: doi:10.4103/0019-5154.119946
  10. IWAI T, UMEDA M, INOUE Y. Are There Any Objections against Our Hypothesis That Buerger Disease Is an Infectious Disease? Ann Vasc Dis [online] 2012, 5(3):300-309 [viewed 28 October 2014] Available from: doi:10.3400/avd.ra.12.00032
  11. ARKKILA PE. Thromboangiitis obliterans (Buerger's disease) Orphanet J Rare Dis [online] :14 [viewed 28 October 2014] Available from: doi:10.1186/1750-1172-1-14
  12. MADABHAVI I, REVANNASIDDAIAH S, RASTOGI M, GUPTA MK. Paraneoplastic Raynaud's phenomenon manifesting before the diagnosis of lung cancer BMJ Case Rep [online] :bcr0320125985 [viewed 28 October 2014] Available from: doi:10.1136/bcr.03.2012.5985
  13. JOVILIANO EE, DELLALIBERA-JOVILIANO R, DALIO M, ÉVORA PR, PICCINATO CE. Etiopathogenesis, clinical diagnosis and treatment of thromboangiitis obliterans - current practices Int J Angiol [online] 2009, 18(3):119-125 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2903023
  14. PIAZZA G, CREAGER MA. Thromboangiitis Obliterans Circulation [online] 2010 Apr 27, 121(16):1858-1861 [viewed 28 October 2014] Available from: doi:10.1161/CIRCULATIONAHA.110.942383
  15. IWAI T, SATO, KUME H, INOUE Y, UMEDA M, KAGAYAMA T, HIROKAWA M. Clinical Study of Phlebitis Migrans and Incompetence of the Leg's Superficial Vein in Buerger Disease Ann Vasc Dis [online] 2012, 5(1):45-51 [viewed 28 October 2014] Available from: doi:10.3400/avd.oa.11.00044
  16. TRIMBLE GX. Pulmonary embolism in Buerger's disease. West J Med [online] 1982 Jul, 137(1):78-79 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1274003

Examination

Fact Explanation
General examination Seen whether patient is in pain (with the prolonged pain), depressed, level of hydration, pallor and features of chronic smoking like nicotine stains[1][2].
Examination of a limb with claudication Inspection: Walking distance of the pain will give an idea of the severity. Palpation; Bilateral femoral and distal pulse will be absent in aortoiliac obstruction. Unilateral femoral and distal pulses will be absence in iliac obstruction. In femoropopliteal obstruction femoral pulse will be palpable with absent unilateral distal pulses. Only ankle pulses will be absent with palpable femoral and popliteal pulses in a obstruction distal to femoropopliteal region. Auscultation: In aortoiliac obstruction there will be bruit over aortoiliac region. In iliac obstruction bruit can be identify over the iliac region In femoropopliteal Unilateral claudication in the calf [3][4]
Examination of a limb with rest pain Inspection for, The position of the patient (will hang down the legs). look for presence of skin discoloration (dry gangrene) and ulcers (examine the ulcer for site, surrounding, base, edges). Tropical changes of the skin like loss of hair, sweating, So the skin will be dry. Examine for amputation, and if present examine the stump (for healing, length of the stump, movements of the proximal joint, muscle strength of the proximal part, pulses). look for any evidence of infections (fever, inflammatory changes of the surrounding area, pus collection). Palpation for, the coldness of the leg. Sensation will be impaired and pulses will be absent according to the site of the obstuction. Auscultation for, Bruit over the limb. The bruit site will depend according to the site of the obstruction. [3][5][6]
Examination for bruits and presence of andominal aortic aneurysm. look for bruits in other sites like carotid, subclavian, brachial, renal. Abdominal aortic aneurysm will present as the pulsatile abdominal lump[7][8].
Systemic signs of arterial occlusion With this condition arteries in all systems can affect causing various symptoms. Eg: Brain strokes can occur giving features of paralysis, paresthesia and cranial nerve palsy. In spinal cord, features of infarction like limb paralysis, paresthesia, bladder/ bowel dysfunction. In bones, ischemic pain at site of the joint/back pain and fractures following long bone infarction can be seen. Myocardial infarctions can occur following hypoperfusion of the myocardium( chest pain, difficulty in breathing, dizziness). In lungs there will be shortness of breath and pleuritic type chest pain. In mesentry, acute abdominal pain will be the presentation. In digits, painful fingers and toes with small bone infarction. In kidneys infarction of medulla with papillary necrosis may lead to fail in concentrating urine causing high urine out put, dehydration and nocturnal enuresis. Chronic liver failure with micro infarction giving signs of chronic liver failure like jaundice, ascites. Splenic infarction leads to pallor, easy bruising and recurrent infections like upper/ lower respiratory tract infections and diarrheal illnessess[9][10].
capillary nail-fold loops examination and observation of Raynaud’s phenomenon This can be done with an ophthalmoscope. It is helpful in distinguishing primary from secondary Raynaud's (loss of the normal loop pattern and capillary 'fallout' with haemorrhage and dots indicate underlying disease). Raynaud’s phenomenon can be observed while patient handling the cool water[11][12].
Signs of superficial thrombophlebitis There will be tender localized areas over the skin of the limb[12][13]. This condition is often migratory.
Signs of superficial thrombophlebitis Patient will develop swelling, redness and tenderness and warmth of the limbs[14][15].
Signs suggestive of pulmonary embolism Patient will be dyspnoic,coughing, wheezing and there will be fine crepts on auscultation[1][15].
References
  1. SZUBA A, COOKE JP. Thromboangiitis obliterans. An update on Buerger's disease. West J Med [online] 1998 Apr, 168(4):255-260 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1304949
  2. MCLOUGHLIN GA, HELSBY CR, EVANS CC, CHAPMAN DM. Association of HLA-A9 and HLA-B5 with Buerger's disease. Br Med J [online] 1976 Nov 13, 2(6045):1165-1166 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1689618
  3. VIJAYAKUMAR A, TIWARI R, KUMAR PRABHUSWAMY V. Thromboangiitis Obliterans (Buerger's Disease)--Current Practices Int J Inflam [online] 2013:156905 [viewed 28 October 2014] Available from: doi:10.1155/2013/156905
  4. CASTLEDEN WM, FAULKNER K, HOUSE AK, WATT A. Haemoglobin, smoking and peripheral vascular disease J R Soc Med [online] 1981 Aug, 74(8):586-590 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1438930
  5. DE HARO J, ACIN F, BLEDA S, VARELA C, ESPARZA L. Treatment of thromboangiitis obliterans (Buerger's disease) with bosentan BMC Cardiovasc Disord [online] :5 [viewed 28 October 2014] Available from: doi:10.1186/1471-2261-12-5
  6. MATSUMOTO H, YAMAMOTO E, KAMIYA C, MIURA E, KITAOKA T, SUZUKI J, YAMAMOTO K, DEGUCHI J, HIGASHI M, TAMARU JI, SATO O. Sural Artery Bypass in Buerger's Disease: Report of a Case Ann Vasc Dis [online] 2012, 5(2):199-203 [viewed 28 October 2014] Available from: doi:10.3400/avd.cr.11.00090
  7. A. FAKHREE M. B, AZHOUGH R, HAFEZ QURAN F. A Case of True Brachial Artery Aneurysm in an Elderly Male J Cardiovasc Thorac Res [online] 2012, 4(1):25-27 [viewed 28 October 2014] Available from: doi:10.5681/jcvtr.2012.006
  8. YUKIOS U, MATSUNO Y, IMAIZUMI M, MORI Y, IWATA H, TAKIYA H. Bilateral Radial Artery Aneurysms in the Anatomical Snuff Box Seen in Marfan Syndrome Patient: Case Report and Literature Review Ann Vasc Dis [online] 2009, 2(3):185-189 [viewed 28 October 2014] Available from: doi:10.3400/avd.AVDcr09010
  9. IWAI T, UMEDA M, INOUE Y. Are There Any Objections against Our Hypothesis That Buerger Disease Is an Infectious Disease? Ann Vasc Dis [online] 2012, 5(3):300-309 [viewed 28 October 2014] Available from: doi:10.3400/avd.ra.12.00032
  10. ARKKILA PE. Thromboangiitis obliterans (Buerger's disease) Orphanet J Rare Dis [online] :14 [viewed 28 October 2014] Available from: doi:10.1186/1750-1172-1-14
  11. MADABHAVI I, REVANNASIDDAIAH S, RASTOGI M, GUPTA MK. Paraneoplastic Raynaud's phenomenon manifesting before the diagnosis of lung cancer BMJ Case Rep [online] :bcr0320125985 [viewed 28 October 2014] Available from: doi:10.1136/bcr.03.2012.5985
  12. JOVILIANO EE, DELLALIBERA-JOVILIANO R, DALIO M, ÉVORA PR, PICCINATO CE. Etiopathogenesis, clinical diagnosis and treatment of thromboangiitis obliterans - current practices Int J Angiol [online] 2009, 18(3):119-125 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2903023
  13. PIAZZA G, CREAGER MA. Thromboangiitis Obliterans Circulation [online] 2010 Apr 27, 121(16):1858-1861 [viewed 28 October 2014] Available from: doi:10.1161/CIRCULATIONAHA.110.942383
  14. IWAI T, SATO, KUME H, INOUE Y, UMEDA M, KAGAYAMA T, HIROKAWA M. Clinical Study of Phlebitis Migrans and Incompetence of the Leg's Superficial Vein in Buerger Disease Ann Vasc Dis [online] 2012, 5(1):45-51 [viewed 28 October 2014] Available from: doi:10.3400/avd.oa.11.00044
  15. TRIMBLE GX. Pulmonary embolism in Buerger's disease. West J Med [online] 1982 Jul, 137(1):78-79 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1274003

Differential Diagnoses

Fact Explanation
Atherosclerotic occlusive arterial diseases In atherosclerotic occlusive arterial disease the patient would be older with underlying risk factors such as diabetes mellitus, hypertension and lipidaemia and smoking. But smoking will not be the sole risk factor as in thrombangitis obliterans. The disease want be extensive and will be athermatous occlusion with patent distal run off vessels, were as in thromangitis obliterans the distal run off also will be affected. These findings would be obvious in angiograms[1][2].
venous pain Venous pain will be aggravated in persistent standing due to poor venous return and will be relieved with moving the leg as well as by the elevation of the leg. In venous pain there would be obvious varicose veins causing venous hypertension. Duplex will differentiate the occlusion weather it is arterial or venous[3][4].
Neurogenic claudication In neurogenic claudication there would be a pain of legs on walking, due to the engorgement and occlusion of nerves at lower spinal canal and cauda equina. This pain which occurred with exertion will go off only with sitting or squatting on ground whereas in arterial claudication just resting will relieve the pain. Therefore during climbing and cycling the pain would be in patients with neurogenic claudication. Arterial duplex would be normal MRI of spine will show the pathology of the spinal canal leading to neurogenic claudication[5][6].
Peripheral neuropathy In peripheral neuropathy patient will complain the glove and stocking numbness, tingling sensation and night pain. They will have sensory impairment and nerve conduction studies will show the nerve impairment[7][8].
Other diseases causing ulcers As mentioned above patients can present with ulcers with could be arterial, venous and neuropathic. As mentioned above the co existing pain could support to differentiate the origin of ulcer. Other than that the site of ulcer; venous ulcers in bi malleolar area, neuropathic ulcers in pressure areas, arterial ulcers in distal toes. If the wound is gangrenous it would be arterial. [9][10].
Other causes of vasculitis - Autoimmune diseases: Scleroderma or the CREST syndrome (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, sclerodactyly, and telangiectasia). - Systemic lupus erythematosus, rheumatoid arthritis, mixed connective tissue disease, antiphospholipid antibody syndrome and other types of vasculitis. These could be differentiated from the history, because these are commoner in young females as well as could be associated with miscarriages and other vasculitic phenomena such as vasculitic skin lesions, oral ulcers, presence of lymph nodes and pyrexia. Inflammatory markers such as ESR and CRP will be elevated and other investigations such as ANA, ds-DNA, p-ANCA, c-ANCA might direct towards a diagnosis[11][12].
References
  1. DHALIWAL G, MUKHERJEE D. Peripheral arterial disease: Epidemiology, natural history, diagnosis and treatment Int J Angiol [online] 2007, 16(2):36-44 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2733014
  2. KASAPIS C, GURM HS. Current Approach to the Diagnosis and Treatment of Femoral-Popliteal Arterial Disease. A Systematic Review Curr Cardiol Rev [online] 2009 Nov, 5(4):296-311 [viewed 28 October 2014] Available from: doi:10.2174/157340309789317823
  3. SIMON DA, DIX FP, MCCOLLUM CN. Management of venous leg ulcers BMJ [online] 2004 Jun 5, 328(7452):1358-1362 [viewed 28 October 2014] Available from: doi:10.1136/bmj.328.7452.1358
  4. SPENTZOURIS G, LABROPOULOS N. The Evaluation of Lower-Extremity Ulcers Semin Intervent Radiol [online] 2009 Dec, 26(4):286-295 [viewed 28 October 2014] Available from: doi:10.1055/s-0029-1242204
  5. NADEAU M, ROSAS-ARELLANO MP, GURR KR, BAILEY SI, TAYLOR DC, GREWAL R, LAWLOR DK, BAILEY CS. The reliability of differentiating neurogenic claudication from vascular claudication based on symptomatic presentation Can J Surg [online] 2013 Dec, 56(6):372-377 [viewed 28 October 2014] Available from: doi:10.1503/cjs.016512
  6. COMER CM, REDMOND AC, BIRD HA, CONAGHAN PG. Assessment and management of neurogenic claudication associated with lumbar spinal stenosis in a UK primary care musculoskeletal service: a survey of current practice among physiotherapists BMC Musculoskelet Disord [online] :121 [viewed 28 October 2014] Available from: doi:10.1186/1471-2474-10-121
  7. MISRA UK, KALITA J, NAIR PP. Diagnostic approach to peripheral neuropathy Ann Indian Acad Neurol [online] 2008, 11(2):89-97 [viewed 28 October 2014] Available from: doi:10.4103/0972-2327.41875
  8. CORRAO G, ZAMBON A, BERTU L, BOTTERI E, LEONI O, CONTIERO P. Lipid lowering drugs prescription and the risk of peripheral neuropathy: an exploratory case-control study using automated databases J Epidemiol Community Health [online] 2004 Dec, 58(12):1047-1051 [viewed 28 October 2014] Available from: doi:10.1136/jech.2003.013409
  9. MINNITI CP, ECKMAN J, SEBASTIANI P, STEINBERG MH, BALLAS SK. Leg Ulcers in Sickle Cell Disease Am J Hematol [online] 2010 Oct, 85(10):831-833 [viewed 28 October 2014] Available from: doi:10.1002/ajh.21838
  10. SIMON DA, DIX FP, MCCOLLUM CN. Management of venous leg ulcers BMJ [online] 2004 Jun 5, 328(7452):1358-1362 [viewed 28 October 2014] Available from: doi:10.1136/bmj.328.7452.1358
  11. COJOCARU M, COJOCARU IM, SILOSI I, VRABIE CD. Gastrointestinal Manifestations in Systemic Autoimmune Diseases Maedica (Buchar) [online] 2011 Jan, 6(1):45-51 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3150032
  12. CASTRO C, GOURLEY M. Diagnostic Testing and Interpretation of Tests for Autoimmunity J Allergy Clin Immunol [online] 2010 Feb, 125(2 Suppl 2):S238-S247 [viewed 28 October 2014] Available from: doi:10.1016/j.jaci.2009.09.041

Investigations - for Diagnosis

Fact Explanation
Doppler ultrasound blood flow detection Hand-held Doppler is a simple non invasive test use to assess the presence occlusive arterial disease.So this is useful in measurement of ankle-brachial pressure index (ABPI)[1][2].
Duplex imaging This is a combined investigation of both doppler studies and ultrasound scanning. it assess the anatomy of the vessels and the extend of the disease condition. This also useful in excluding the associated deep vein thrombosis[1][2][3].
Colour-flow imaging In here the results of doppler studies are seen through the colour images. It is more sensitive in detecting small vascular disease[2][4].
Angiography/ Computerised tomography angiography/ Magnetic resonance angiogram/ echocardiography These will show anatomy of the vascular tree via a contrast imaging with characteristic arteriograpic findings (peripheral multiple segmental occlusion, cock-screw collaterals) and are also used to exclude a proximal embolic source [1][5][6].
D dimer level D dimer level will be elevated in deep vein thrombosis[7].
CT pulmonary angiography This is the first line imaging modality in diagnosing pulmonary embolism[8].
References
  1. OLIN JW, SEALOVE BA. Peripheral Artery Disease: Current Insight Into the Disease and Its Diagnosis and Management Mayo Clin Proc [online] 2010 Jul, 85(7):678-692 [viewed 28 October 2014] Available from: doi:10.4065/mcp.2010.0133
  2. YOSHIMUTA T, AKUTSU K, OKAJIMA T, TAMORI Y, KUBOTA Y, TAKESHITA S. Corkscrew collaterals in Buerger's disease Can J Cardiol [online] 2009 Jun, 25(6):365 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2722480
  3. BURT R, TESTORI A, OYAMA Y, RODRIGUEZ H, YAUNG K, VILLA M, BUCHA J, MILANETTI F, SHEEHAN J, RAJAMANNAN N, PEARCE W. Autologous peripheral blood CD133+ cell implantation for limb salvage in patients with critical limb ischemia Bone Marrow Transplant [online] 2010 Jan, 45(1):111-116 [viewed 28 October 2014] Available from: doi:10.1038/bmt.2009.102
  4. DE HARO J, ACIN F, BLEDA S, VARELA C, ESPARZA L. Treatment of thromboangiitis obliterans (Buerger's disease) with bosentan BMC Cardiovasc Disord [online] :5 [viewed 28 October 2014] Available from: doi:10.1186/1471-2261-12-5
  5. JUN HJ. Endovascular Revascularization for the Obstruction after Patch Angioplasty in Buerger's Disease Korean J Thorac Cardiovasc Surg [online] 2014 Apr, 47(2):174-177 [viewed 28 October 2014] Available from: doi:10.5090/kjtcs.2014.47.2.174
  6. IWAI T, UMEDA M, INOUE Y. Are There Any Objections against Our Hypothesis That Buerger Disease Is an Infectious Disease? Ann Vasc Dis [online] 2012, 5(3):300-309 [viewed 28 October 2014] Available from: doi:10.3400/avd.ra.12.00032
  7. KAMIYA C, DEGUCHI J, KITAOKA T, SUZUKI J, ABE K, SATO O. Obstruction of the Superior Mesenteric Artery Due to Emboli from the Thoracic Aorta in a Patient with Thromboangiitis Obliterans Ann Vasc Dis [online] 2014, 7(3):320-324 [viewed 28 October 2014] Available from: doi:10.3400/avd.cr.14-00035
  8. TANABE N. Diagnosis and Vasculopathy in Chronic Thromboembolic Pulmonary Hypertension Ann Vasc Dis [online] 2009, 2(3):136-143 [viewed 28 October 2014] Available from: doi:10.3400/avd.AVDctpe00209

Investigations - Fitness for Management

Fact Explanation
FBC This is useful in assessing the haemoglobin level, and the wBC and platelet counts before invasive procedures or surgeries[1][2].
ESR/CRP This will assess the presence of any ongoing inflammation and important in excluding inflammatory vasculitis associated with various autoimmune conditions[1][2][3].
Fasting blood sugar This will assess the blood sugar levels and to identify the co existing diabetes mellitus which is a common acquired condition for macro vascular diseases finally resulting amputation. [1][2].
Renal function tests like serum electrolytes, serum creatinine, blood urea Renal function need to be assess before surgical procedures[4][5].
Liver function tests like AST, ALT Liver function also need to be assess before surgical procedures[6][7].
Clotting profile with PT/ INR, APTT This will give an idea about clotting status of the patient[2][8][9].
Blood grouping and crossed matching Before surgical procedure these test will useful in preserving blood for an emergency need[10][11].
Lipid profile This will be useful in excluding any associated hyperlipidaemia and the presence of atherosclerotic occlusive arterial diseases[12][13].
ECG This will be useful in assessing the cardiac function in pre operatively as these patients are at risk of developing cardiac complications as well.[14].
Chest X ray This will assess the pulmonary suitability before a surgical procedure[15][16].
References
  1. ARKKILA PE. Thromboangiitis obliterans (Buerger's disease) Orphanet J Rare Dis [online] :14 [viewed 28 October 2014] Available from: doi:10.1186/1750-1172-1-14
  2. PIAZZA G, CREAGER MA. Thromboangiitis Obliterans Circulation [online] 2010 Apr 27, 121(16):1858-1861 [viewed 28 October 2014] Available from: doi:10.1161/CIRCULATIONAHA.110.942383
  3. VIJAYAKUMAR A, TIWARI R, KUMAR PRABHUSWAMY V. Thromboangiitis Obliterans (Buerger's Disease)--Current Practices Int J Inflam [online] 2013:156905 [viewed 28 October 2014] Available from: doi:10.1155/2013/156905
  4. LEE KS, PAIK CN, CHUNG WC, LEE KM, JUNG SH, KAWK JW, JUNG JH, BAIK JH. Colon Ischemia Associated with Buerger's Disease: Case Report and Review of the Literature Gut Liver [online] 2010 Jun, 4(2):287-291 [viewed 28 October 2014] Available from: doi:10.5009/gnl.2010.4.2.287
  5. GROVE WJ, STANSBY GP. Buerger's disease and cigarette smoking in Bangladesh. Ann R Coll Surg Engl [online] 1992 Mar, 74(2):115-118 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2497513
  6. LI QL, HE DH, HUANG YH, NIU M. Thromboangiitis obliterans in two brothers Exp Ther Med [online] 2013 Aug, 6(2):317-320 [viewed 28 October 2014] Available from: doi:10.3892/etm.2013.1160
  7. DE HARO J, ACIN F, BLEDA S, VARELA C, ESPARZA L. Treatment of thromboangiitis obliterans (Buerger's disease) with bosentan BMC Cardiovasc Disord [online] :5 [viewed 28 October 2014] Available from: doi:10.1186/1471-2261-12-5
  8. KAMIYA C, DEGUCHI J, KITAOKA T, SUZUKI J, ABE K, SATO O. Obstruction of the Superior Mesenteric Artery Due to Emboli from the Thoracic Aorta in a Patient with Thromboangiitis Obliterans Ann Vasc Dis [online] 2014, 7(3):320-324 [viewed 28 October 2014] Available from: doi:10.3400/avd.cr.14-00035
  9. IWAI T, UMEDA M, INOUE Y. Are There Any Objections against Our Hypothesis That Buerger Disease Is an Infectious Disease? Ann Vasc Dis [online] 2012, 5(3):300-309 [viewed 28 October 2014] Available from: doi:10.3400/avd.ra.12.00032
  10. MURPHY MP, WANG H, PATEL AN, KAMBHAMPATI S, ANGLE N, CHAN K, MARLEAU AM, PYSZNIAK A, CARRIER E, ICHIM TE, RIORDAN NH. Allogeneic endometrial regenerative cells: An "Off the shelf solution" for critical limb ischemia? J Transl Med [online] :45 [viewed 28 October 2014] Available from: doi:10.1186/1479-5876-6-45
  11. RIORDAN NH, CHAN K, MARLEAU AM, ICHIM TE. Cord blood in regenerative medicine: do we need immune suppression? J Transl Med [online] :8 [viewed 28 October 2014] Available from: doi:10.1186/1479-5876-5-8
  12. DORAISWAMY VA, GIRI J, MOHLER E III. Premature peripheral arterial disease - difficult diagnosis in very early presentation Int J Angiol [online] 2009, 18(1):45-47 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2726566
  13. BANDAWAR MS, ANSARI MS, BEHERA A, BHADADA SK. An unusual cause of toe necrosis Indian J Endocrinol Metab [online] 2013, 17(1):160-162 [viewed 28 October 2014] Available from: doi:10.4103/2230-8210.107872
  14. TAMURA A, ASO N, KADOTA J. Corkscrew appearance in the right coronary artery in a patient with Buerger's disease Heart [online] 2006 Jul, 92(7):944 [viewed 28 October 2014] Available from: doi:10.1136/hrt.2005.077453
  15. LI QL, HE DH, HUANG YH, NIU M. Thromboangiitis obliterans in two brothers Exp Ther Med [online] 2013 Aug, 6(2):317-320 [viewed 28 October 2014] Available from: doi:10.3892/etm.2013.1160
  16. KANEYAMA J, KAWARADA O, SAKAMOTO S, HARADA K, ISHIHARA M, YASUDA S, OGAWA H. Vasospastic Limb Ischemia Presenting Acute and Chronic Limb Ischemia Ann Vasc Dis [online] 2014, 7(2):169-172 [viewed 28 October 2014] Available from: doi:10.3400/avd.cr.13-00113

Investigations - Followup

Fact Explanation
Swab from ulcers for culture and ABST This will be useful in excluing the presence of infection in a chronic ullcer associated with limb ischemia[1][2].
Ultrasound scan This is useful during follow up to assess the presence of abdominal aortic aneurysm[3][4].
Investigations to exclude other causes of vasculitis like Antinuclear antibody, rheumatoid factor, complement level, anticentromere antibody, Scl-70 antibody, antiphospholipid antibodies These tests will be useful in excluding other causeds of systemic vasculitis like Systemic Lupus Erythematosus, antipospholipid antibody syndrome, rheumatoid arthritis[5][6].
References
  1. KI V, ROTSTEIN C. Bacterial skin and soft tissue infections in adults: A review of their epidemiology, pathogenesis, diagnosis, treatment and site of care Can J Infect Dis Med Microbiol [online] 2008 Mar, 19(2):173-184 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2605859
  2. YEBOAH-MANU D, DANSO E, AMPAH K, ASANTE-POKU A, NAKOBU Z, PLUSCHKE G. Isolation of Mycobacterium ulcerans from Swab and Fine-Needle-Aspiration Specimens J Clin Microbiol [online] 2011 May, 49(5):1997-1999 [viewed 28 October 2014] Available from: doi:10.1128/JCM.02279-10
  3. KUNISHIGE H, ISHIBASHI Y, KAWASAKI M, MORIMOTO K, INOUE N. Risk Factors Affecting Survival after Surgical Repair of Ruptured Abdominal Aortic Aneurysm Ann Vasc Dis [online] 2013, 6(3):631-636 [viewed 28 October 2014] Available from: doi:10.3400/avd.cr.13-00035
  4. PAVLIC V, VUJIC-ALEKSIC V, ZUBOVIC N, GOJKOV-VUKELIC M. Periodontitis and Buerger's Disease: Recent Advances Acta Inform Med [online] 2013 Dec, 21(4):250-252 [viewed 28 October 2014] Available from: doi:10.5455/aim.2013.21.250-252
  5. OHGI S, OHGI N. Relation between Isolated Venous Thrombi in Soleal Muscle and Positive Anti-Nuclear Antibody Ann Vasc Dis [online] 2012, 5(3):321-327 [viewed 28 October 2014] Available from: doi:10.3400/avd.oa.12.00052
  6. VIJAYAKUMAR A, TIWARI R, KUMAR PRABHUSWAMY V. Thromboangiitis Obliterans (Buerger's Disease)--Current Practices Int J Inflam [online] 2013:156905 [viewed 28 October 2014] Available from: doi:10.1155/2013/156905

Investigations - Screening/Staging

Fact Explanation
Rutherford classification(staging system for clinical symptoms) Grade 0: Category 0 - Asymptomatic Grade I: Category 1 - Mild claudication Grade I: Category 2 - Moderate claudication Grade I: Category 3 - Severe claudication Grade II: Category 4 - Rest pain Grade III: Category 5 - Ischemic ulcer not exceeding digits Grade IV: Category 6 - Severe ischemic ulcer or gangrene [1][2].
Leriche-fontaine classification (clinico-pathological classification) I; Asymptomatic or effort pain with Relative hypoxia ;Silent arteriopathy II A: Effort pain/pain-free walking distance >200 m with Relative hypoxia: Stabilized arteriopathy, noninvalidant claudication II B: Pain-free walking distance <200 m with Relative hypoxia: Instable arteriopathy, invalidant claudication III A: Rest pain, ankle arterial pressure >50 mm Hg with Cutaneous hypoxia, tissue acidosis, ischemic neuritis: Instable arteriopathy, invalidant claudication III B: Rest pain, ankle arterial pressure <50 mm Hg with Cutaneous hypoxia, tissue acidosis, ischemic neuritis: Instable arteriopathy, invalidant claudication IV: Trophic lesions, necrosis or gangrene with Cutaneous hypoxia, tissue acidosis, necrosis: Evolutive arteriopathy [1][3]
References
  1. VIJAYAKUMAR A, TIWARI R, KUMAR PRABHUSWAMY V. Thromboangiitis Obliterans (Buerger's Disease)--Current Practices Int J Inflam [online] 2013:156905 [viewed 28 October 2014] Available from: doi:10.1155/2013/156905
  2. KARAKOYUN R, KöKSOY C, ŞENER Z, GüNDüZ U, KARAKAş B, KARAKOYUN M. Comparison of quality of life in patients with peripheral arterial disease caused by atherosclerosis obliterans or Buerger's disease Cardiovasc J Afr [online] 2014 Jun, 25(3):124-129 [viewed 28 October 2014] Available from: doi:10.5830/CVJA-2014-017
  3. ORBAN F. New Trends in the Treatment of Thromboangeiosis (Buerger's Disease): Moynihan Lecture delivered at the Royal College of Surgeons of England on 9th March 1960 Ann R Coll Surg Engl [online] 1961 Feb, 28(2):69-100 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2414039

Management - General Measures

Fact Explanation
Health education Patient should be educated regarding the disease, symptoms associated with, possible complications, investigations needed, available treatment options, predisposing factors for exacerbations and prognosis[1][2][5][6].
Wound care This could classified as optimizing systemic and local factors. Regarding the systemic factors correction of haemoglobin level, glycaemic control, nutrition, high protein diet can be done. Regarding the local factors timely performed wound debridement, proper wound dressing, physio therapy and using antibiotics. The proper wound care will reduce the rate of amputation[6][9].
Prevention Prevention is only by non smoking[3][4].
Prevention of the complications Following measures would be beneficial in minimizing the complications Avoiding injuries to the extremities by wearing properly fitting footwear and identifying risk activities and avoiding those. Early treatment for the ulcers before becoming septic also important. Avoiding exposure to cold water which will further impede the circulation. Avoidance vasoconstrictive drugs[7][8].
References
  1. PAVLIC V, VUJIC-ALEKSIC V, ZUBOVIC N, GOJKOV-VUKELIC M. Periodontitis and Buerger's Disease: Recent Advances Acta Inform Med [online] 2013 Dec, 21(4):250-252 [viewed 28 October 2014] Available from: doi:10.5455/aim.2013.21.250-252
  2. VIJAYAKUMAR A, TIWARI R, KUMAR PRABHUSWAMY V. Thromboangiitis Obliterans (Buerger's Disease)--Current Practices Int J Inflam [online] 2013:156905 [viewed 28 October 2014] Available from: doi:10.1155/2013/156905
  3. GROVE WJ, STANSBY GP. Buerger's disease and cigarette smoking in Bangladesh. Ann R Coll Surg Engl [online] 1992 Mar, 74(2):115-118 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2497513
  4. JINDAL RM, PATEL SM. Buerger's disease and cigarette smoking in Bangladesh. Ann R Coll Surg Engl [online] 1992 Nov, 74(6):436-437 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2497682
  5. PATWA JJ, KRISHNAN A. Buerger's Disease (Thromboangiitis Obliterans)- Management by Ilizarov's Technique of Horizontal Distraction. A Retrospective Study of 60 Cases Indian J Surg [online] 2011 Jan, 73(1):40-47 [viewed 28 October 2014] Available from: doi:10.1007/s12262-010-0186-1
  6. RYU SW, JEON HJ, CHO SS, CHOI RM, YOON JS, KO HS, LEE JD. Treatment of digit ulcers in a patient with Buerger's disease by using cervical spinal cord stimulation -a case report- Korean J Anesthesiol [online] 2013 Aug, 65(2):167-171 [viewed 28 October 2014] Available from: doi:10.4097/kjae.2013.65.2.167
  7. MATSUMOTO H, YAMAMOTO E, KAMIYA C, MIURA E, KITAOKA T, SUZUKI J, YAMAMOTO K, DEGUCHI J, HIGASHI M, TAMARU JI, SATO O. Sural Artery Bypass in Buerger's Disease: Report of a Case Ann Vasc Dis [online] 2012, 5(2):199-203 [viewed 28 October 2014] Available from: doi:10.3400/avd.cr.11.00090
  8. ARKKILA PE. Thromboangiitis obliterans (Buerger's disease) Orphanet J Rare Dis [online] :14 [viewed 28 October 2014] Available from: doi:10.1186/1750-1172-1-14
  9. DE HARO J, ACIN F, BLEDA S, VARELA C, ESPARZA L. Treatment of thromboangiitis obliterans (Buerger's disease) with bosentan BMC Cardiovasc Disord [online] :5 [viewed 28 October 2014] Available from: doi:10.1186/1471-2261-12-5

Management - Specific Treatments

Fact Explanation
Absolute cessation of smoking Cessation of smoking is the only proven action to slow or stop the disease progression. Smoking, chewing tobacco and even nicotin patch replacement should be avoided[1][2].
Medical management A prostaglandin analogue called Intravenous iloprost not used much yet in current practice appears to be effective in improving symptoms, enhancing the resolution of distal-extremity trophic changes. It also reduces the rate of amputation in affected patients[3]. The use of thrombolytic agents seems to be effective but still no hard evidence[4][5]. Other pharmacological modalities such as antiplatelets, steroids, vasodilators, anticoagulants andcalcium channel blockers are generally being ineffective[5].
Pain management This could be pharmacological or non pharmacological. Nonsteroidal anti-Inflammatory drugs can be use to treat the pain which occurs with the limb ischemia. But usually the pain is not resolved by simple analgesics. So the most of the patients may require opioid analgesics such as tramadol and morphine. Tricyclic antidepressants such as amitriptyllin neuropathic pain drugs such as gabapentin and valporate also can be used[6][7]. In some patients the pain will be intractable and not controlled with analgesics. So in these cases invasive procedures may be needed. Non pharmacological This could be either simple invasive procedures such as nerve infiltration or could be major interventions like sympathectomy[8][9].
Amputation of non viable and high risk extremities. In Thrombangitis oblitarans the arterial grafting can be rarely performed because unlike in atherosclerotic occlusive arterial disease medium and small vessels are also affected. So it is difficult find a patent distal vessels to act as a run off for the graft. There fore other than for the pain management by invasive procedures the surgical options are towards timely amputation which prevent the occurrence of sepsis but the surgeon should try the minimum possible amputation. Indications for amputation are dead limb, gangrene, deadly limb and severe rest pain[4][10][11].
References
  1. JINDAL RM, PATEL SM. Buerger's disease and cigarette smoking in Bangladesh. Ann R Coll Surg Engl [online] 1992 Nov, 74(6):436-437 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2497682
  2. GROVE WJ, STANSBY GP. Buerger's disease and cigarette smoking in Bangladesh. Ann R Coll Surg Engl [online] 1992 Mar, 74(2):115-118 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2497513
  3. DE HARO J, ACIN F, BLEDA S, VARELA C, ESPARZA L. Treatment of thromboangiitis obliterans (Buerger's disease) with bosentan BMC Cardiovasc Disord [online] :5 [viewed 28 October 2014] Available from: doi:10.1186/1471-2261-12-5
  4. RYU SW, JEON HJ, CHO SS, CHOI RM, YOON JS, KO HS, LEE JD. Treatment of digit ulcers in a patient with Buerger's disease by using cervical spinal cord stimulation -a case report- Korean J Anesthesiol [online] 2013 Aug, 65(2):167-171 [viewed 28 October 2014] Available from: doi:10.4097/kjae.2013.65.2.167
  5. BUCCI F, REDLER A, FIENGO L. Critical Limb Ischemia in a Young Man: Saddle Embolism or Unusual Presentation of Thromboangiitis Obliterans? Case Rep Vasc Med [online] 2013:830540 [viewed 28 October 2014] Available from: doi:10.1155/2013/830540
  6. JORGE VC, ARAúJO AC, NORONHA C, PANARRA A, RISO N, VAZ RISCADO M. Buerger's disease (Thromboangiitis obliterans): a diagnostic challenge BMJ Case Rep [online] :bcr0820114621 [viewed 28 October 2014] Available from: doi:10.1136/bcr.08.2011.4621
  7. AFSHARFARD A, MOZAFFAR M, MALEKPOUR F, BEIGIBOROOJENI A, REZAEE M. The Wound Healing Effects of Iloprost in Patients with Buerger's Disease: Claudication and Prevention of Major Amputations Iran Red Crescent Med J [online] 2011 Jun, 13(6):420-423 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3371931
  8. CHANDER J, SINGH L, LAL P, JAIN A, LAL P, RAMTEKE VK. Retroperitoneoscopic Lumbar Sympathectomy for Buerger's Disease: A Novel Technique JSLS [online] 2004, 8(3):291-296 [viewed 28 October 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3016814
  9. KOTHARI R, SHARMA D, THAKUR DS, KUMAR V, SOMASHEKAR U. Thoracoscopic Dorsal Sympathectomy for Upper Limb Buerger's Disease JSLS [online] 2014, 18(2):273-276 [viewed 28 October 2014] Available from: doi:10.4293/108680813X13753907292755
  10. VIJAYAKUMAR A, TIWARI R, KUMAR PRABHUSWAMY V. Thromboangiitis Obliterans (Buerger's Disease)--Current Practices Int J Inflam [online] 2013:156905 [viewed 28 October 2014] Available from: doi:10.1155/2013/156905
  11. PATWA JJ, KRISHNAN A. Buerger's Disease (Thromboangiitis Obliterans)- Management by Ilizarov's Technique of Horizontal Distraction. A Retrospective Study of 60 Cases Indian J Surg [online] 2011 Jan, 73(1):40-47 [viewed 28 October 2014] Available from: doi:10.1007/s12262-010-0186-1