Digestive Disease Interventions 2018; 02(03): 231-239
DOI: 10.1055/s-0038-1668568
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Middle Aortic Syndrome: Pathogenesis, Visceral Manifestations, and Management

Eric J. Monroe
1   Department of Radiology, Seattle Children's Hospital, Seattle, Washington
2   Department of Radiology, University of Washington, Seattle, Washington
,
Brian H. Morray
3   Division of Pediatric Cardiology, Seattle Children's Hospital, Seattle, Washington
,
Yongdong Zhao
4   Department of Pediatrics, Seattle Children's Hospital, Seattle, Washington
5   Department of Pediatrics, University of Washington, Seattle, Washington
,
Caitlin A. Smith
6   Department of Surgery, Seattle Children's Hospital, Seattle, Washington
› Author Affiliations
Further Information

Publication History

02 July 2018

09 July 2018

Publication Date:
20 August 2018 (online)

Abstract

Middle aortic syndrome (MAS) is characterized by severe narrowing of the abdominal aorta, frequently involving the great abdominal vessels. Although increasingly implicated in primary hypertension, up to one-third of cases affect the mesenteric vasculature, and the resultant manifestations may be underrecognized. Early intervention with interdisciplinary medical management and invasive arterial reconstruction affords preservation of end-organ function and improvement in patient survival. This article reviews the embryology of the thoracoabdominal aorta and major abdominal arteries, as well as the pathogenesis, clinical manifestations, and management of the nonatherosclerotic abdominal coarctation otherwise known as MAS.

 
  • References

  • 1 Abbott ME. Coarctation of the aorta of the adult type: II. A statistical study and historical retrospect of 200 recorded cases with autopsy, of stenosis or obliteration of the descending arch in subjects above the age of two years. Am Heart J 1928; 3 (05) 574-618
  • 2 Maycock W. Congenital stenosis of the abdominal aorta. Am Heart J 1937; 13 (06) 633-646
  • 3 Graham LM, Zelenock GB, Erlandson EE, Coran AG, Lindenauer SM, Stanley JC. Abdominal aortic coarctation and segmental hypoplasia. Surgery 1979; 86 (04) 519-529
  • 4 Connolly JE, Wilson SE, Lawrence PL, Fujitani RM. Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies. J Am Coll Surg 2002; 194 (06) 774-781
  • 5 Stanley JC, Graham LM. . L. M. (2000). Abdominal aortic coarctation and hypoplasia. In: Gewertz B, Schwartz LB. , eds. Surgery of the Aorta and Its Branches. Philadelphia, PA: WB Saunders; 2000: 11-16
  • 6 Coleman DM, Eliason JL, Ohye RG, Stanley JC. Long-segment thoracoabdominal aortic occlusions in childhood. J Vasc Surg 2012; 56 (02) 482-485
  • 7 Rumman RK, Nickel C, Matsuda-Abedini M. , et al. Disease beyond the arch: a systematic review of middle aortic syndrome in childhood. Am J Hypertens 2015; 28 (07) 833-846
  • 8 Delis KT, Gloviczki P. Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Perspect Vasc Surg Endovasc Ther 2005; 17 (03) 187-203
  • 9 Tummolo A, Marks SD, Stadermann M. , et al. Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol 2009; 24 (11) 2225-2232
  • 10 Keibel F, Mall FP. , eds. Manual of Human Embryology. Vol. 2. Philadelphia, PA: J. B. Lippincott Company; 1912
  • 11 Lin PH, Chaikof EL. Embryology, anatomy, and surgical exposure of the great abdominal vessels. Surg Clin North Am 2000; 80 (01) 417-433 , xiv
  • 12 Mills B, Gest T. Persistent double dorsal aorta: a systematic review of the literature. Clin Anat 2017; 30 (04) 517-524
  • 13 Arnot RS, Louw JH. The anatomy of the posterior wall of the abdominal aorta. Its significance with regard to hypoplasia of the distal aorta. S Afr Med J 1973; 47 (21) 899-902
  • 14 Panayiotopoulos YP, Tyrrell MR, Koffman G, Reidy JF, Haycock GB, Taylor PR. Mid-aortic syndrome presenting in childhood. Br J Surg 1996; 83 (02) 235-240
  • 15 Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KEC. Idiopathic mid-aortic syndrome in children. Pediatr Nephrol 2008; 23 (07) 1135-1142
  • 16 Singer DB, Rudolph AJ, Rosenberg HS, Rawls WE, Boniuk M. Pathology of the congenital rubella syndrome. J Pediatr 1967; 71 (05) 665-675
  • 17 Siassi B, Klyman G, Emmanouilides GC. Hypoplasia of the abdominal aorta associated with the rubella syndrome. Am J Dis Child 1970; 120 (05) 476-479
  • 18 Morris CA. Williams syndrome. In: Adam MP, Ardinger HH, Pagon RA. , et al., eds. GeneReviews® [Internet]. Seattle, WA: University of Washington; 1999. . Apr 9 [Updated 2017 Mar 23]
  • 19 Kelleher CM, McLean SE, Mecham RP. Vascular extracellular matrix and aortic development. Curr Top Dev Biol 2004; 62: 153-188
  • 20 Pober BR, Johnson M, Urban Z. Mechanisms and treatment of cardiovascular disease in Williams-Beuren syndrome. J Clin Invest 2008; 118 (05) 1606-1615 radfo
  • 21 Radford DJ, Pohlner PG. The middle aortic syndrome: an important feature of Williams' syndrome. Cardiol Young 2000; 10 (06) 597-602
  • 22 Ledbetter DH, Rich DC, O'Connell P, Leppert M, Carey JC. Precise localization of NF1 to 17q11.2 by balanced translocation. Am J Hum Genet 1989; 44 (01) 20-24
  • 23 Feldkamp MM, Gutmann DH, Guha A. Neurofibromatosis type 1: piecing the puzzle together. Le J Canad Des Sci Neurol 1998; 25 (03) 181-191
  • 24 Friedman JM, Arbiser J, Epstein JA. , et al. Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force. Genet Med 2002; 4 (03) 105-111
  • 25 Riccardi VM. Type 1 neurofibromatosis and the pediatric patient. Curr Probl Pediatr 1992; 22 (02) 66-106 , discussion 107
  • 26 Huffman JL, Gahtan V, Bowers VD, Mills JL. Neurofibromatosis and arterial aneurysms. Am Surg 1996; 62 (04) 311-314
  • 27 Gomez MR. Neurocutaneous diseases. In: Bradley WC. , ed. Neurology in Clinical Practice. Vol. 2. Boston, MA: Butterworth-Heinemann; 1991: 1327
  • 28 Schürch W, Messerli FH, Genest J. , et al. Arterial hypertension and neurofibromatosis: renal artery stenosis and coarctation of abdominal aorta. Can Med Assoc J 1975; 113 (09) 879-885
  • 29 Oderich GS, Sullivan TM, Bower TC. , et al. Vascular abnormalities in patients with neurofibromatosis syndrome type I: clinical spectrum, management, and results. J Vasc Surg 2007; 46 (03) 475-484
  • 30 Gulati A, Bagga A. Large vessel vasculitis. Pediatr Nephrol 2010; 25 (06) 1037-1048
  • 31 Stanley JC, Graham LM, Whitehouse Jr WM. , et al. Developmental occlusive disease of the abdominal aorta and the splanchnic and renal arteries. Am J Surg 1981; 142 (02) 190-196
  • 32 Ishikawa K. Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu's arteriopathy. J Am Coll Cardiol 1988; 12 (04) 964-972
  • 33 Arend WP, Michel BA, Bloch DA. , et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33 (08) 1129-1134
  • 34 Pham PP, Moller JH, Hills C, Larson V, Pyles L. Cardiac catheterization and operative outcomes from a multicenter consortium for children with williams syndrome. Pediatr Cardiol 2009; 30 (01) 9-14
  • 35 Eronen M, Peippo M, Hiippala A. , et al. Cardiovascular manifestations in 75 patients with Williams syndrome. J Med Genet 2002; 39 (08) 554-558
  • 36 Collins II RT, Kaplan P, Somes GW, Rome JJ. Long-term outcomes of patients with cardiovascular abnormalities and Williams syndrome. Am J Cardiol 2010; 105 (06) 874-878
  • 37 Onat T, Zeren E. Coarctation of the abdominal aorta. Review of 91 cases. Cardiologia 1969; 54 (03) 140-157
  • 38 Tullus K, Roebuck DJ, McLaren CA, Marks SD. Imaging in the evaluation of renovascular disease. Pediatr Nephrol 2010; 25 (06) 1049-1056
  • 39 Burch TM, McGowan Jr FX, Kussman BD, Powell AJ, DiNardo JA. Congenital supravalvular aortic stenosis and sudden death associated with anesthesia: what's the mystery?. Anesth Analg 2008; 107 (06) 1848-1854
  • 40 Olsen M, Fahy CJ, Costi DA, Kelly AJ, Burgoyne LL. Anaesthesia-related haemodynamic complications in Williams syndrome patients: a review of one institution's experience. Anaesth Intensive Care 2014; 42 (05) 619-624
  • 41 Matisoff AJ, Olivieri L, Schwartz JM, Deutsch N. Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review. Paediatr Anaesth 2015; 25 (12) 1207-1215
  • 42 Latham GJ, Ross FJ, Eisses MJ, Richards MJ, Geiduschek JM, Joffe DC. Perioperative morbidity in children with elastin arteriopathy. Paediatr Anaesth 2016; 26 (09) 926-935
  • 43 Quandt D, Ramchandani B, Bhole V. , et al. Initial experience with the cook formula balloon expandable stent in congenital heart disease. Catheter Cardiovasc Interv 2015; 85 (02) 259-266
  • 44 Suárez de Lezo J, Romero M, Pan M. , et al. Stent repair for complex coarctation of aorta. JACC Cardiovasc Interv 2015; 8 (10) 1368-1379
  • 45 Messina LM, Reilly LM, Goldstone J, Ehrenfeld WK, Ferrell LD, Stoney RJ. Middle aortic syndrome. Effectiveness and durability of complex arterial revascularization techniques. Ann Surg 1986; 204 (03) 331-339
  • 46 D'Souza SJ, Tsai WS, Silver MM. , et al. Diagnosis and management of stenotic aorto-arteriopathy in childhood. J Pediatr 1998; 132 (06) 1016-1022
  • 47 Keith DS, Markey B, Schiedler M. Successful long-term stenting of an atypical descending aortic coarctation. J Vasc Surg 2002; 35 (01) 166-167
  • 48 McMahon CJ, Lambert I, Walsh KP. Transcatheter double stent implantation for treatment of middle aortic coarctation syndrome. Catheter Cardiovasc Interv 2013; 82 (04) 560-563
  • 49 Porras D, Stein DR, Ferguson MA. , et al. Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management. Pediatr Nephrol 2013; 28 (10) 2023-2033
  • 50 Ghazi P, Haji-Zeinali AM, Shafiee N, Qureshi SA. Endovascular abdominal aortic stenosis treatment with the OptiMed self-expandable nitinol stent. Catheter Cardiovasc Interv 2009; 74 (04) 634-641
  • 51 Stadermann MB, Montini G, Hamilton G. , et al. Results of surgical treatment for renovascular hypertension in children: 30 year single centre experience. Nephrol Dial Transplant 2010; 25 (03) 807-813
  • 52 Go MR, Bhende S, Smead WL, Vaccaro PS. Long-term complications in two patients after aortoaortic bypass for midaortic syndrome. Ann Vasc Surg 2013; 27 (04) 499.e9-499.e12
  • 53 Kalman PG, Rappaport DC, Merchant N, Clarke K, Johnston KW. The value of late computed tomographic scanning in identification of vascular abnormalities after abdominal aortic aneurysm repair. J Vasc Surg 1999; 29 (03) 442-450