Abstract
Vascular disease usually involves arterial stenosis, aneurysms, and dissections caused by atherosclerosis. The high prevalence of atherosclerosis ensures that most general surgeons have experience managing patients with traditional vascular disease. In contrast, non-atheroslcerotic disorders affecting the arteries and veins occur less frequently and may not be familiar to most clinicians. Left untreated, non-atherosclerotic vascular conditions can have serious and potentially fatal consequences. This chapter reviews clinically relevant non-atherosclerotic vascular diseases including hypercoagulable states, popliteal entrapment syndrome, Buerger’s disease, vasculitis, and fibromuscular dysplasia. Patients with these disorders differ from traditional vascular patients by age, comorbidities, presentation, and treatment. A basic understanding of non-atherosclerotic vascular disease can help general surgeons recognize and appropriately manage patients with these rare conditions.
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References
Kearon C, Crowther M, Hirsh J. Management of patients with hereditary hypercoagulable disorders. Annu Rev Med. 2000; 51:169–85.
Rao AK, Sheth S, Kaplan R. Inherited hypercoagulable states. Vasc Med. 1997;2:313–20.
Ray SA, Rowley MR, Loh A, et al. Hypercoagulable states in patients with leg ischemia. Br J Surg. 1994;81:811–4.
Eldrup-Jorgensen J, Flanigan DP, Brace L, et al. Hypercoagulable states and lower limb ischemia in young adults. J Vasc Surg. 1989;9:334–41.
Donaldson MC, Weinberg DS, Belkin M, et al. Screening for hypercoagulable states in vascular surgical practice: a preliminary study. J Vasc Surg. 1990;11:825–31.
Crowther MA, Kelton JG. Congenital thrombophilic states associated with venous thrombosis: a qualitative overview and proposed classification system. Ann Intern Med. 2003;138(2):128–34.
Egeberg O. Inherited antithrombin deficiency causing thrombophilia. Thromb Diath Haemorrh. 1965;13:516–30.
Mateo J, Oliver A, Borrell M, et al. Laboratory evaluation and clinical characteristics of 2,132 consecutive unselected patients with venous thromboembolism. Results of the Spanish Multicentric Study on Thrombophilia (EMET-study). Thromb Haemost. 1997;77:444.
Van Den Belt AGM, Huisman MV, Hirsh J. Familial thrombophilia: a review analysis. Clin Appl Thromb Hemost. 1996; 2(4):227–36.
Weiss P, Soff GA, Halkin H, et al. Decline of protein C and S and factors II, VII, IX and X during the initiation of warfarin therapy. Thromb Res. 1987;45(6):783–90.
Borgel D, Gandrille S, Aiach M. Protein S deficiency. Thromb Haemost. 1997;78:351.
Clark DA, Williams WL, Marlar RA. Mesenteric vein thrombosis associated with familial deficiency of free protein S. Arch Pathol Lab Med. 1991;115:617.
Baglin T, Gray E, Greaves M, et al. British Committee for Standards in Haematology. Clinical guidelines for testing for heritable thrombophilia. Br J Haematol. 2010;149:209–20.
Rodeghiero F, Tosetto A. Activated protein C resistance and factor V Leiden mutation are independent risk factors for venous thromboembolism. Ann Intern Med. 1999;130:643–50.
Rees DC, Cox M, Clegg JB. World distribution of factor V Leiden. Lancet. 1995;346:1133–4.
Koster T, Rosendaal FR, de Ronde H, et al. Venous thrombosis due to poor anticoagulant response to activated protein C: Leiden thrombophilia study. Lancet. 1993;342:1503–6.
Leroyer C, Mercier B, Escoffe M, et al. Factor V Leiden prevalence in venous thromboembolism patients. Chest. 1997;111:1603–6.
Springer J, Villa-Forte A. Thrombosis in vasculitis. Curr Opin Rheumatol. 2012;24:1–7.
Hellgren M, Svensson PI, Dahlback B. Resistance to activated protein C as a basis for venous thromboembolism associated with pregnancy and oral contraceptives. Am J Obstet Gynecol. 1995;173:210.
Olivieri O, Friso S, Manzato F, Guella A, Bernardi F, Lunghi B, Girelli D, Azzini M, Brocco G, Russo C. Resistance to activated protein C in healthy women taking oral contraceptives. Br J Haematol. 1995;91:465–70.
Henkens CMA, van der Bom JG, van der Meer FJ. Lowered activated protein C-sensitivity ratio related to increased factor VIII-clotting activity. Thromb Haemost. 1995;74:1198–9.
Cumming AM, Tait RC, Fildes S, Young A, Keeney S, Hay CR. Development of resistance to activated protein C during pregnancy. Br J Haematol. 1995;90:725–7.
Vandenbroucke JP, Koster T, Briet E, et al. Increased risk of venous thrombosis in oral-contraceptive users who are carriers of factor V Leiden mutation. Lancet. 1994;344:1453–7.
McColl MD, Ramsay JE, Tait RC, et al. Risk factors for pregnancy associated venous thromboembolism. Thromb Haemost. 1997;78:1183–8.
Ridker PM, Hennekens CH, Selhub J, et al. Interrelation of hyperhomocyst(e)inemia, factor V Leiden, and risk of future venous thromboembolism. Circulation. 1997;95:1777–82.
Gandrille S, Greengard JS, Alhenc-Gelas M, et al. Incidence of activated protein C resistance caused by the ARG 506 GLN mutation in factor V in 113 unrelated symptomatic protein C-deficient patients. Blood. 1995;86:219–24.
Zoller B, Holm J, Svensson P, Dahlback B. Elevated levels of prothrombin activation fragment 1 + 2 in plasma from patients with heterozygous Arg506 to Gln mutation in the factor V gene (APC resistance) and/or inherited protein S deficiency. Thromb Haemost. 1996;75:270–4.
Lane DA, Mannucci PM, Bauer KA, et al. Inherited thrombophilia: part 2. Thromb Haemost. 1996;76(6):824–34.
Ridker PM, Hennekens CH, Miletich JP. G20210A mutation in prothrombin gene and risk of myocardial infarction, stroke and venous thrombosis in a large cohort of US men. Circulation. 1999; 99:999–1004.
Ridker PM, Miletich JP, Stampfer MJ, et al. Factor V Leiden and risks of recurrent idiopathic venous thromboembolism. Circulation. 1995;92:2800–2.
Simioni P, Prandoni P, Lensing AWA, et al. The risk of recurrent venous thromboembolism in patients with an Arg506Gln mutation in the gene for factor V (factor V Leiden). N Engl J Med. 1997; 336:399–403.
Kearon C, Gent M, Hirsh J, et al. A comparison of three months of anticoagulation with extended anticoagulation for a first episode of idiopathic venous thromboembolism. N Engl J Med. 1999; 340:901–7.
Eichinger S, Pabinger I, Stumpflen A, et al. The risk of recurrent venous thromboembolism in patients with and without factor V Leiden. Thromb Haemost. 1997;77(4):624–8.
Doggen CJ, Cats VM, Bertina RM, Rosendaal FR. Interaction of coagulation defects and cardiovascular risk factors: increased risk of myocardial infarction associated with factor V Leiden or prothrombin 20210A. Circulation. 1998;97(11):1037–41.
Rosendaal FR, Doggen CJM, Zivelin A, et al. Geographic distribution of the 20210 G to a prothrombin variant. Thromb Haemost. 1998;79:706–8.
Koster T, Blann AD, Briet E, et al. Role of clotting factor VIII in effect of von Willebrand factor on occurrence of deep-vein thrombosis. Lancet. 1995;345:152.
O’Donnell JO, Tuddenham EGD, Manning R, et al. High prevalence of elevated factor VIII levels in patients referred for thrombophilia screening: role of increased synthesis and relationship to acute phase reaction. Thromb Haemost. 1997;77:825.
Kamphusien PW, Eikenboom JCJ, Bertina RM. Elevated factor VIII levels and the risk of thrombosis. Arterioscler Thromb Vasc Biol. 2001;21:731.
Stampfer MJ, Malinow MR, Willett WC, et al. A prospective study of plasma homocyst(e)ine and risk of myocardial infarction in US physicians. JAMA. 1992;268:877–81.
Clarke R, Daly L, Robinson K, et al. Hyperhomocysteinemia: an independent risk factor for vascular disease. N Engl J Med. 1991;324(17):1149–55.
den Heijer M, Koster T, Bloom HJ, et al. Hyperhomocysteinemia as a risk factor for deep vein thrombosis. N Engl J Med. 1996;334:759–62.
Simioni P, Prandoni P, Burlina A, et al. Hyperhomocysteinemia and deep-vein thrombosis: a case controlled study. Thromb Haemost. 1996;76:883.
Arnesen E, Refsum H, Bonaa KH, et al. Serum total homocysteine and coronary heart disease. Int J Epidemiol. 1995;24:704–9.
Nygard O, Nordrehaug JE, Refusum H, et al. Plasma homocysteine levels and mortality in patients with coronary artery disease. N Engl J Med. 1997;337:230.
Lim W, Crowther MA. Antiphospholipid antibodies: a critical review of the literature. Curr Opin Hematol. 2007;14:494.
Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006; 4:295.
Turiel M, Sarzi-Puttini P, Peretti R, et al. Thrombotic risk factors in primary antiphospholipid syndrome. A 5 year prospective study. Stroke. 2005;36:1490.
Rosove MH, Brewer PMC. Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients. Ann Intern Med. 1992;117:303.
Khamashta MA, Cuadrado MD, Mukic F, et al. The management of thrombosis in the antiphospholipid antibody syndrome. N Engl J Med. 1995;332:993.
de Groot PG, Lutters B, Derksen RH, et al. Lupus anticoagulants and the risk of a first episode of deep venous thrombosis. J Thromb Haemost. 2005;3:1993.
Trousseau A. Phlegmasia Alba Dolen. In: Clinique Medicale De L’Hotel-Dieu de Paris. Paris: JB Balliere; 1865. p. 654.
Blom JW, Doggen CJ, Osanto S, Roendaal FR. Malignancies, prothrombotic mutations and the risk of venous thrombosis. JAMA. 2005;293:715–22.
Lee AY, Levine MN, Baker RI, et al. Low-molecular-weight heparin versus a coumarin for the prevention of recurrent venous thromboembolism in patients with cancer. N Engl J Med. 2003; 349(2):146–53.
Hull RD, Pineo GF, Brant RF, et al. LITE Trial Investigators. Long-term low-molecular-weight heparin versus usual care in proximal-vein thrombosis patients with cancer. Am J Med. 2006; 119:1062.
Prandoni P, Lensing AW, Piccioli A, et al. Recurrent venous thromboembolism and bleeding complications during anticoagulant treatment in patients with cancer and venous thrombosis. Blood. 2002;100:3484.
Heit JA, Kobervig CE, James AH, et al. Trends in the incidence of venous thromboembolism during pregnancy or postpartum: a 30 year population-based study. Ann Intern Med. 2005;143:697.
Andres RL, Miles A. Venous thromboembolism and pregnancy. Obstet Gynecol Clin North Am. 2001;28:613.
James AH, Tapson VF, Goldhaber SZ. Thrombosis during pregnancy and the postpartum period. Am J Obstet Gynecol. 2005; 193:216.
Faught W, Garner P, Jones G, et al. Changes in protein C and protein S levels in normal pregnancy. Am J Obstet Gynecol. 1995;172:147.
Comp PC, Thurnau GR, Welsh J, et al. Functional and immunologic protein S levels are decreased during pregnancy. Blood. 1986;68:881.
Johnson CM, Mureebe L, Silver D. Hypercoagulable states: a review. Vasc Endovascular Surg. 2005;39(2):123–33.
Ginsberg J, Brill-Edwards P, Burrows RF, et al. DVT during pregnancy: leg and trimester presentation. Thromb Haemost. 1992; 67:519.
Martinelli I, DeStafano V, Taioli E, et al. Inherited thrombophilias and first venous thromboembolism during pregnancy and puerperium. Thromb Haemost. 2002;87:791–5.
Warkentin TE. Clinical presentation of heparin-induced thrombocytopenia. Semin Hematol. 1998;35(4 Suppl 5):9.
Warkentin TE, Elavathil LJ, Hayward CP, et al. The pathogenesis of venous limb gangrene associated with heparin-induced thrombocytopenia. Ann Intern Med. 1997;127:804.
Simioni P, Sanson BJ, Prandoni P, et al. Incidence of venous thromboembolism in families with inherited thrombophilia. Thromb Haemost. 1999;81:198–202.
Antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines. Chest. 2012;141(2 suppl):7S–47S.
Caprini JA. Risk assessment as a guide for the prevention of the many faces of venous thromboembolism. Am J Surg. 1991; 1S:S3–10.
Geerts WH, Bergqvist D, Pineo GF, Heit JA, Samama CM, Lassen MR, Colwell CW, American College of Chest Physicians. Prevention of venous thromboembolism: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest. 2008;133:381S–453.
Anderson JA, Weitz J. Hypercoagulable states. Crit Care Clin. 2011;27:933–52.
Landefeld CS, Beyth RJ. Anticoagulant-related bleeding: clinical epidemiology, prediction, and prevention. Am J Med. 1993;95: 315–28.
Palareti G, Leali N, Coccheri S, et al. Bleeding complications of oral anticoagulant treatment: an inception-cohort, prospective collaborative study (ISCOAT). Lancet. 1996;348:423–8.
Schafer AI, Levine MN, Konkle BA, et al. Thrombotic disorders: diagnosis and treatment. Hematology. 2003;520–39.
Stuart TP. Note on a variation in the course of the popliteal artery. J Anat Physiol. 1879;13:162.
Hamming JJ. Intermittent claudication at an early age, due to an anomalous course of the popliteal artery. Angiology. 1959;10: 369–70.
Bouhoutsos J, Daskalakis E. Muscular abnormalities affecting the popliteal vessels. Br J Surg. 1981;68:501–6.
Gibson MHL, Mills JG, Johnson GE, et al. Popliteal entrapment syndrome. Ann Surg. 1977;185:341–8.
Turnipseed WD. Popliteal entrapment syndrome. J Vasc Surg. 2002;35:910–5.
Turnipseed WD. Clinical review of patients treated for atypical claudication: a 28-year experience. J Vasc Surg. 2004;40:79–85.
Rignault DP, Pailler JL, Lunel F. The “functional” popliteal entrapment syndrome. Int Angiol. 1985;4:341–3.
Pillai J. A current interpretation of popliteal vascular entrapment. J Vasc Surg. 2008;48:61S–5.
Chernoff DM, Walker AT, Khorasani R, et al. Asymptomatic functional popliteal artery entrapment: demonstration at MR imaging. Radiology. 1995;195:176–80.
Turnipseed WD, Pozniak M. Popliteal entrapment as a result of neurovascular compression by the soleus and plantaris muscles. J Vasc Surg. 1992;15:285–94.
Sinha S, Houghton J, Holt P, et al. Popliteal entrapment syndrome. J Vasc Surg. 2012;55(1):252–62.
Roche-Nagle G, Wong KT, Oreopolulos G. Vascular claudication in a young patient: popliteal entrapment syndrome. Hong Kong Med J. 2009;15(5):388–90.
Ehsan O, Darwish A, Edmundson C, et al. Non-traumatic lower limb vascular complications in endurance athletes. Review of literature. Eur J Vasc Endovasc Surg. 2004;28:1–8.
Levien L. Popliteal artery thrombosis caused by popliteal entrapment syndrome. In: Greenhalgh RM, Powell JT, editors. Inflammatory and thrombotic problems in vascular surgery. London: WB Saunders Co Ltd; 1997. p. 159–68.
Gibson MHL, Mills JG, Johnson GE, et al. Popliteal entrapment syndrome. Ann Surg. 1997;185:341–8.
Murray A, Halliday M, Croft RJ. Popliteal artery entrapment syndrome. Br J Surg. 1991;78:1414–9.
Di Cesare J, Marsili L, Marino G, Masciocchi C, et al. Stress MR imaging for evaluation of popliteal artery entrapment. J Magn Reson Imaging. 1994;4:617–22.
Collins PS, McDonald PT, Lim RC. Popliteal artery entrapment: an evolving syndrome. J Vasc Surg. 1989;10:484–90.
Zund G, Brunner U. Surgical aspects of popliteal artery entrapment syndrome: 26 years of experience with 26 legs. Vasa. 1995; 24:29–33.
DiMarzo L, Cavallaro A, Mingoli A, et al. Popliteal artery entrapment syndrome: the role of early diagnosis and treatment. Surg. 1997;122:26–31.
DiMarzo L, Cavallaro A, Sciacca V, et al. Natural history of entrapment of the popliteal artery. J Am Coll Surg. 1994;178: 553–6.
Akkersdijk WL, de Ruyter JW, Lapham R, et al. Colour duplex ultrasonographic imaging and provocation of popliteal artery compression. Eur J Vasc Endovasc Surg. 1995;10:342–5.
Deshpande A, Denton M. Functional popliteal entrapment syndrome. Aust N Z J Surg. 1998;68:660–3.
Kim SY, Min SK, Ahn S, et al. Long-term outcomes after revascularization for advanced popliteal artery entrapment syndrome with segmental arterial occlusion. J Vasc Surg. 2012;55(1): 90–7.
Papaioannou S, Tsitouridis K, Giataganas G, et al. Evaluation of popliteal arteries with CT angiography in popliteal artery entrapment syndrome. Hippokratia. 2009;13:32–7.
Rich NM, Collins Jr GJ, McDonald PT, et al. Popliteal vascular entrapment. Its increasing interest. Arch Surg. 1979;114: 1377–84.
Espinoza LR. Buerger’s disease: thromboangitis obliterans 100 years after the initial description. Am J Med Sci. 2009;337(4): 285–6.
Piazza G, Creager M. Thromboangitis obliterans. Circulation. 2010;121:1858–61.
Puechal X, Fiessinger JN, Kahan A, et al. Rheumatologic manifestations in patients with thromboangitis obliterans. J Rheumatol. 1999;26:1764–8.
Olin JW. Thromboangitis obliterans. N Engl J Med. 2000;343: 864–9.
Ohta T, Ishioashi H, Hosaka M, et al. Clinical and social consequences of Buerger disease. J Vasc Surg. 2001;39:176–80.
Jayne D. Update on the European Vasculitis Study Group trials. Curr Opin Rheumatol. 2001;13:48–55.
Luqmani R. Large vessel vasculitides. Update for the cardiologist. Curr Opin Cardiol. 2012;27(6):578–84.
Devauchell-Pensec V, Jousee S, Destombe C, et al. Epidemiology, imaging and treatment of GCA. Joint Bone Spine. 2008;75(3): 267–72.
Weyand C, Liao JY, Goronzy JJ, et al. The immunopathology of GCA: diagnostic and therapeutic implications. J Neuroophthalmol. 2012;32(3):259–65.
Wlavick MD, Walvick MP. GCA: laboratory predictors of a positive temporal artery biopsy. Ophthalmology. 2011;118:1201–4.
Danesh-Meyer H. Temporal artery biopsy: skip it at your patient’s peril. Am J Ophthalmol. 2012;154(4):617–9.
Quinn EM, Kearney DE, Keohane C. Temporal artery biopsy is not required in all cases of suspected GCA. Ann Vasc Surg. 2012;26(5):649–54.
Cowey S, Reynolds C, Joglekar S, et al. Does temporal artery biopsy influence management of GCA? Int J Surg. 2010;8:501.
Achkar AA, Lie JT, Hunder GG, O’Fallon WM, Gabriel SE. How does previous glucocorticosteroid treatment affect the biopsy findings in giant cell (temporal) arteritis? Ann Intern Med. 1994; 120:987–92.
Dastgir G, Gutman J, Shinder R. Facial nerve injury: a complication of superficial temporal artery biopsy. Am J Ophthalmol. 2011;153(1):187.
Ypilantis E, Courtney ED, Chopra N, et al. Importance of specimen length during temporal artery biopsy. Br J Surgery. 2011; 98:1556–60.
Scafer V, Zwerina J. Biologic treatment of large vessel vasculitides. Curr Opin Rheumatol. 2012;24(1):31–7.
Talwar KD, Kumar K, Copra P, et al. Cardiac involvement in non-specific arteritis. Am Heart J. 1991;122:1666–70.
Curry TK, Messina LM. Fibromuscular dysplasia: when is intervention warranted? Semin Vasc Surg. 2003;16(3):190–9.
Bornak A, Milner R. Diagnosing and treating atypical arterial pathologies of aortic arch vessels: dissection and fibromuscular dysplasia. Semin Vasc Surg. 2011;24:36–43.
Olin JW, Froehlich J, Xiaokui G, et al. The United States registry for fibromuscular dysplasia results in the first 447 patients. Circulation. 2012;125:3182–90.
Weinberg I, Jaff MR. Nonatherosclerotic disorders of the lower extremities. Circulation. 2012;126:213–22.
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Harris, L.M., Lukan, J. (2015). Non-atherosclerotic Vascular Disease: Vasculitis, Popliteal Entrapment, Hypercoagulable. In: Gahtan, V., Costanza, M. (eds) Essentials of Vascular Surgery for the General Surgeon. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1326-8_22
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