Skip to main content

Advertisement

SpringerLink
Log in

Idiopathic mid-aortic syndrome in children

  • Original Article
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

Mid-aortic syndrome (MAS) is an uncommon condition characterized by narrowing of the abdominal aorta and stenosis of its major branches. Our goal was to illustrate the presentation, diagnosis and management of six new cases of idiopathic MAS together with 96 cases of idiopathic MAS from the literature. The mean age of the 102 cases was 14.3 years (19 days to 49 years). Our patient who presented at 19 days of age is the youngest reported to date. Clinical presentations included hypertension (94%), claudication (17%), renal failure (4%) and intestinal ischemia (1%). Angiography was the diagnostic imaging study of choice. Renal arteries were involved in 91% of patients, while the superior mesenteric artery and celiac artery were involved in 35%. Thirteen percent of cases were managed medically, and the remainder was treated surgically. Our experience shows that initial conservative blood pressure management of idiopathic MAS is feasible unless medical control of hypertension is unsatisfactory, renal function is at risk or there are symptoms of claudication or intestinal ischemia. Careful timing and planning of a surgical intervention is possible for most cases and may, in select cases, be considered after completion of puberty to allow growth to be completed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Connolly JE, Wilson SE, Lawrence PL, Fujitani RM (2002) Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies. J Am Coll Surg 194:774–781

    Article  Google Scholar 

  2. DeBakey ME, Garrett E, Howell J, Morris G (1967) Coarctation of the abdominal aorta with renal arterial stenosis: surgical considerations. Ann Surg 165:830–843

    Article  CAS  Google Scholar 

  3. Graham LM, Aelenock GB, Erlandson EE, Coran AG, Lindenauer S, Stanley J (1979) Abdominal aortic coarctation and segmental hypoplaisa. Surgery 86:519–529

    CAS  PubMed  Google Scholar 

  4. Hallett JW Jr, Brewster DC, Darling RC, O’Hara PJ (1980) Coarctation of the abdominal aorta: current options in surgical management. Ann Surg 191:430–437

    Article  Google Scholar 

  5. Messina LM, Reilly LM, Goldstone J, Ehrenfeld WK, Ferrell LD, Stoney RJ (1986) Middle aortic syndrome. Effectiveness and durability of complex arterial revascularization techniques. Ann Surg 204:331–339

    Article  CAS  Google Scholar 

  6. O’Neill JA, Berkowitz H, Fellows K, Harmon C (1995) Midaortic syndrome and hypertension in childhood. J Pediatr Surg 30:164–172

    Article  Google Scholar 

  7. Panayiotopoulos YP, Tyrrell MR, Koffman G, Reidy JF, Haycock GB, Taylor PR (1996) Mid-aortic syndrome presenting in childhood. Br J Surg 83:235–240

    Article  CAS  Google Scholar 

  8. Poulias GE, Skoutas B, Doundoulakis N, Haycock GB, Taylor PR (1990) The mid-aortic dysplastic syndrome. Surgical considerations with a 2 to 18 year follow-up and selective histopathological study. Eur J Vasc Surg 4:75–82

    Article  CAS  Google Scholar 

  9. Robicsek F, Daugherty HK, Cook JW, Cafoncelli A (1980) Coarctation of the abdominal aorta with stricture of the major vessels. Surgery 87:545–548

    CAS  PubMed  Google Scholar 

  10. Scott HW Jr, Dean RH, Boerth R, Sawyers JL, Meacham P, Fisher RD (1979) Coarctation of the abdominal aorta: pathophysiologic and therapeutic considerations. Ann Surg 189:746–757

    Article  Google Scholar 

  11. Stanley JC, Graham MD, Whitehouse WM, Zelenock GB, Erlandson EE, Cronenwett JL, Lindenauer SM (1981) Developmental occlusive disease of the abdominal aorta and the splanchnic and renal arteries. Am J Surg 142:190–196

    Article  CAS  Google Scholar 

  12. Brzezinska-Rajszys G, Qureshi SA, Ksiazyk J, Zubrzycka M, Kosciesza A, Kubicka K, Tynan M (1999) Middle aortic syndrome treated by stent implantation. Heart 81:166–170

    Article  CAS  Google Scholar 

  13. Onat T, Zeren E (1969) Coarctation of the abdominal aorta. Review of 91 cases. Cardiologia 54:140–157

    Article  CAS  Google Scholar 

  14. Gupta S (1979) Surgical and haemodynamic considerations in middle aortic syndrome. Thorax 34:470–478

    Article  CAS  Google Scholar 

  15. Lewis VD, Meranze SG, McLean GK, O’Neill JA, Berkowitz HD, Burke D (1988) The midaortic syndrome: diagnosis and treatment. Radiology 167:111–113

    Article  Google Scholar 

  16. Sumboonnanonda A, Robinson BL, Gedroyc WM, Saxton HM, Reidy JF, Haycock GB (1992) Middle aortic syndrome: clinical and radiological findings. Arch Dis Child 67:501–505

    Article  CAS  Google Scholar 

  17. Quain R (1847) Partial coarctation of the abdominal aorta. Trans Path Soc 1:244–246

    Google Scholar 

  18. Sen PK, Kinare SG, Engineer SD, Parulkar GB (1963) The middle aortic syndrome. Br Heart J 25:610–618

    Article  CAS  Google Scholar 

  19. Maycock W (1937) Congenital stenosis of the abdominal aorta. Am Heart J 13:633–646

    Article  Google Scholar 

  20. Siassi B, Klyman G, Emmanoulides GC (1970) Hypoplasia of the abdominal aorta associated with the rubella syndrome. Am Dis Child 120:476–479

    CAS  Google Scholar 

  21. Steen H, Schwenger V (2007) Good MRI images: to Gad or not to Gad? Pediatr Nephrol 22:1239–1242

    Article  Google Scholar 

Download references

Acknowledgements

We thank Kenneth Brayman, MD and Jeff Hellinger, MD for their care and evaluation of two patients.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Division of Nephrology, The Children’s Hospital of Philadelphia, The University of Pennsylvania School of Medicine, 34th Street and Civic Center Boulevard, Philadelphia, PA, 19104, USA

    Christine B. Sethna, Bernard S. Kaplan & Kevin E. C. Meyers

  2. Department of Radiology, The Children’s Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, PA, USA

    Anne Marie Cahill

  3. Department of Surgery, Division of Vascular Surgery, University of Pennsylvania School of Medicine, Philadelphia, PA, USA

    Omaida C. Velazquez

Authors
  1. Christine B. Sethna
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Bernard S. Kaplan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Anne Marie Cahill
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Omaida C. Velazquez
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Kevin E. C. Meyers
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Kevin E. C. Meyers.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sethna, C.B., Kaplan, B.S., Cahill, A.M. et al. Idiopathic mid-aortic syndrome in children. Pediatr Nephrol 23, 1135–1142 (2008). https://doi.org/10.1007/s00467-008-0767-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-008-0767-4

Keywords

Search

Navigation