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Idiopathic midaortic syndrome with malignant hypertension in 3-year-old boy

  • Ahn, Kyung Jin (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Yoon, Ja Kyoung (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Kim, Gi Beom (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Kwon, Bo Sang (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Bae, Eun Jung (Department of Pediatrics, Seoul National University Children's Hospital) ;
  • Noh, Chung Il (Department of Pediatrics, Seoul National University Children's Hospital)
  • Received : 2014.07.30
  • Accepted : 2014.08.26
  • Published : 2016.11.15

Abstract

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.

Keywords

References

  1. Sen PK, Kinare SG, Engineer SD, Parulkar GB. The middle aortic syndrome. Br Heart J 1963;25:610-8. https://doi.org/10.1136/hrt.25.5.610
  2. Panayiotopoulos YP, Tyrrell MR, Koffman G, Reidy JF, Haycock GB, Taylor PR. Mid-aortic syndrome presenting in childhood. Br J Surg 1996;83:235-40. https://doi.org/10.1002/bjs.1800830228
  3. Tummolo A, Marks SD, Stadermann M, Roebuck DJ, McLaren CA, Hamilton G, et al. Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol 2009;24:2225-32. https://doi.org/10.1007/s00467-009-1242-6
  4. Sumboonnanonda A, Robinson BL, Gedroyc WM, Saxton HM, Reidy JF, Haycock GB. Middle aortic syndrome: clinical and radiological findings. Arch Dis Child 1992;67:501-5. https://doi.org/10.1136/adc.67.4.501
  5. ten Dam K, van der Palen RL, Tanke RB, Schreuder MF, de Jong H. Clinical recognition of mid-aortic syndrome in children. Eur J Pediatr 2013;172:413-6. https://doi.org/10.1007/s00431-012-1800-y
  6. Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KE. Idiopathic mid-aortic syndrome in children. Pediatr Nephrol 2008;23:1135-42. https://doi.org/10.1007/s00467-008-0767-4
  7. O'Neill JA Jr, Berkowitz H, Fellows KJ, Harmon CM. Midaortic syndrome and hypertension in childhood. J Pediatr Surg 1995;30:164-71. https://doi.org/10.1016/0022-3468(95)90555-3
  8. Terramani TT, Salim A, Hood DB, Rowe VL, Weaver FA. Hypoplasia of the descending thoracic and abdominal aorta: a report of two cases and review of the literature. J Vasc Surg 2002;36:844-8. https://doi.org/10.1016/S0741-5214(02)00143-X
  9. De Bakey ME, Garrett HE, Howell JF, Morris GC Jr. Coarctation of the abdominal aorta with renal arterial stenosis: surgical considerations. Ann Surg 1967;165:830-43. https://doi.org/10.1097/00000658-196705000-00019
  10. Maycock WdA. Congenital stenosis of the abdominal aorta. Am Heart J 1937;13:633-46. https://doi.org/10.1016/S0002-8703(37)91084-6
  11. Cohen JR, Birnbaum E. Coarctation of the abdominal aorta. J Vasc Surg 1988;8:160-4. https://doi.org/10.1016/0741-5214(88)90404-1
  12. Graham LM, Zelenock GB, Erlandson EE, Coran AG, Lindenauer SM, Stanley JC. Abdominal aortic coarctation and segmental hypoplasia. Surgery 1979;86:519-29.
  13. Delis KT, Gloviczki P. Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Perspect Vasc Surg Endovasc Ther 2005;17:187-203. https://doi.org/10.1177/153100350501700302
  14. Stanley JC, Zelenock GB, Messina LM, Wakefield TW. Pediatric renovascular hypertension: a thirty-year experience of operative treatment. J Vasc Surg 1995;21:212-26. https://doi.org/10.1016/S0741-5214(95)70263-6
  15. Lin YJ, Hwang B, Lee PC, Yang LY, Meng CC. Mid-aortic syndrome: a case report and review of the literature. Int J Cardiol 2008;123:348-52. https://doi.org/10.1016/j.ijcard.2006.11.167