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Segmental Arterial Mediolysis

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Abstract

Segmental arterial mediolysis (SAM) is an uncommon, nonatherosclerotic, noninflammatory, large- to medium-sized arteriopathy first described in 1976. It is characterized histologically by vacuolization and lysis of the outer arterial media leading to dissecting aneurysms and vessel rupture presenting clinically with self-limiting abdominal pain or catastrophic hemorrhages in the abdomen. Patients of all ages are affected with a greater incidence at the fifth and sixth decades. There is a slight male predominance. Imaging findings overlap with inflammatory vasculitis, collagen vascular disease, and fibromuscular dysplasia. The presence of segmental dissections involving the celiac, mesenteric, and/or renal arteries is the key distinguishing features of SAM. Inflammatory markers, genetic tests for collagen vascular disorders, and hypercoagulable studies are negative. Anti-inflammatory agents and immunosuppressants are not effective. A mortality rate of 50 % has been attributed to the acute presentation with aneurysmal rupture necessitating urgent surgical or endovascular treatments; in the absence of the acute presentation, SAM is a self-limiting disease and is treated conservatively. There are no established guidelines on medical therapy, although optimal control of blood pressure is considered the main cornerstone of medical therapy. The long-term prognosis is not known.

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References

  1. Slavin RE, Gonzalez-Vitale JC (1976) Segmental mediolytic arteritis: a clinical pathologic study. Lab Invest 35(1):23–29

    CAS  PubMed  Google Scholar 

  2. Slavin RE, Saeki K, Bhagavan B, Maas AE (1995) Segmental arterial mediolysis: a precursor to fibromuscular dysplasia? Mod Pathol 8(3):287–294

    CAS  PubMed  Google Scholar 

  3. Slavin RE (2009) Segmental arterial mediolysis: course, sequelae, prognosis, and pathologic-radiologic correlation. Cardiovasc Pathol 18(6):352–360. doi:10.1016/j.carpath.2008.09.001

    Article  PubMed  Google Scholar 

  4. Armas OA, Donovan DC (1992) Segmental mediolytic arteritis involving hepatic arteries. Arch Pathol Lab Med 116(5):531–534

    CAS  PubMed  Google Scholar 

  5. Lie JT (1992) Segmental mediolytic arteritis. Not an arteritis but a variant of arterial fibromuscular dysplasia. Arch Pathol Lab Med 116(3):238–241

    CAS  PubMed  Google Scholar 

  6. Slavin RE, Yaeger MJ (2012) Segmental arterial mediolysis—an iatrogenic vascular disorder induced by ractopamine. Cardiovasc Pathol 21(4):334–338. doi:10.1016/j.carpath.2011.09.003

    Article  CAS  PubMed  Google Scholar 

  7. Slavin RE, Cafferty L, Cartwright J Jr (1989) Segmental mediolytic arteritis. A clinicopathologic and ultrastructural study of two cases. Am J Surg Pathol 13(7):558–568

    Article  CAS  PubMed  Google Scholar 

  8. Inayama Y, Kitamura H, Kitamura H, Tobe M, Kanisawa M (1992) Segmental mediolytic arteritis. Clinicopathologic study and three-dimensional analysis. Acta Pathol Jpn 42(3):201–209

    CAS  PubMed  Google Scholar 

  9. Shenouda M, Riga C, Naji Y, Renton S (2014) Segmental arterial mediolysis: a systematic review of 85 cases. Ann Vasc Surg 28(1):269–277. doi:10.1016/j.avsg.2013.03.003

    Article  PubMed  Google Scholar 

  10. Tameo MN, Dougherty MJ, Calligaro KD (2011) Spontaneous dissection with rupture of the superior mesenteric artery from segmental arterial mediolysis. J Vasc Surg 53(4):1107–1112. doi:10.1016/j.jvs.2010.11.034

    Article  PubMed  Google Scholar 

  11. Leu HJ (1994) Cerebrovascular accidents resulting from segmental mediolytic arteriopathy of the cerebral arteries in young adults. Cardiovasc Surg 2(3):350–353

    CAS  PubMed  Google Scholar 

  12. Gruenwald P (1949) Necrosis in the coronary arteries of newborn infants. Am Heart J 38(6):889–997

    Article  CAS  PubMed  Google Scholar 

  13. Michael M, Widmer U, Wildermuth S, Barghorn A, Duewell S, Pfammatter T (2006) Segmental arterial mediolysis: CTA findings at presentation and follow-up. AJR Am J Roentgenol 187(6):1463–1469

    Article  PubMed  Google Scholar 

  14. Inada K, Maeda M, Ikeda T (2007) Segmental arterial mediolysis: unrecognized cases culled from cases of ruptured aneurysm of abdominal visceral arteries reported in the Japanese literature. Pathol Res Pract 203(11):771–778 [Erratum in Pathol Res Pract 2008;204(2):147]

    Article  PubMed  Google Scholar 

  15. Kalva SP, Somarouthu B, Jaff MR, Wicky S (2011) Segmental arterial mediolysis: clinical and imaging features at presentation and during follow-up. J Vasc Interv Radiol 22(10):1380–1387. doi:10.1016/j.jvir.2011.07.001

    Article  PubMed  Google Scholar 

  16. Sakano T, Morita K, Imaki M, Ueno H (1997) Segmental arterial mediolysis studied by repeated angiography. Br J Radiol 70(834):656–658

    CAS  PubMed  Google Scholar 

  17. Toriello HV, Glover TW, Takahara K, Byers PH, Miller DE, Higgins JV, Greenspan DS (1996) A translocation interrupts the COL5A1 gene in a patient with Ehlers–Danlos syndrome and hypomelanosis of Ito. Nat Genet 13(3):361–365

    Article  CAS  PubMed  Google Scholar 

  18. Rommel K, Karck M, Haverich A, Schmidtke J, Arslan-Kirchner M (2002) Mutation screening of the fibrillin-1 (FBN1) gene in 76 unrelated patients with Marfan syndrome or Marfanoid features leads to the identification of 11 novel and three previously reported mutations. Hum Mutat 20(5):406–407

    Article  PubMed  Google Scholar 

  19. van der Linde D, Verhagen HJ, Moelker A, van de Laar IM, Van Herzeele I, De Backer J, Dietz HC, Roos-Hesselink JW (2013) Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms. J Vasc Surg 57(1):96–102. doi:10.1016/j.jvs.2012.06.107

    Article  PubMed  Google Scholar 

  20. Ryan JM, Suhocki PV, Smith TP (2000) Coil embolization of segmental arterial mediolysis of the hepatic artery. J Vasc Interv Radiol 11(7):865–868

    Article  CAS  PubMed  Google Scholar 

  21. Shimohira M, Ogino H, Sasaki S, Ishikawa K, Koyama M, Watanabe K, Shibamoto Y (2008) Transcatheter arterial embolization for segmental arterial mediolysis. J Endovasc Ther 15(4):493–497. doi:10.1583/08-2384.1

    Article  PubMed  Google Scholar 

  22. Hashimoto T, Deguchi J, Endo H, Miyata T (2008) Successful treatment tailored to each splanchnic arterial lesion due to segmental arterial mediolysis (SAM): report of a case. J Vasc Surg 48(5):1338–1341. doi:10.1016/j.jvs.2008.05.056

    Article  PubMed  Google Scholar 

  23. Davran R, Cinar C, Parildar M, Oran I (2010) Radiological findings and endovascular management of three cases with segmental arterial mediolysis. Cardiovasc Intervent Radiol 33(3):601–606. doi:10.1007/s00270-009-9651-2

    Article  PubMed  Google Scholar 

  24. Soulen MC, Cohen DL, Itkin M, Townsend RR, Roberts DA (2004) Segmental arterial mediolysis: Angioplasty of bilateral renal artery stenoses with 2-year imaging follow-up. J Vasc Interv Radiol 15(7):763–767

    Article  PubMed  Google Scholar 

  25. Chan RJ, Goodman TA, Aretz TH, Lie JT (1998) Segmental mediolytic arteriopathy of the splenic and hepatic arteries mimicking systemic necrotizing vasculitis. Arthritis Rheumatol 41(5):935–938

    Article  CAS  Google Scholar 

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Correspondence to Anil Kumar Pillai.

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Pillai, A.K., Iqbal, S.I., Liu, R.W. et al. Segmental Arterial Mediolysis. Cardiovasc Intervent Radiol 37, 604–612 (2014). https://doi.org/10.1007/s00270-014-0859-4

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  • DOI: https://doi.org/10.1007/s00270-014-0859-4

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