Abstract
Mid aortic syndrome (MAS) is a rare condition often presenting with severe hypertension. It is characterized by narrowing of the abdominal aorta. We here describe a case of complete occlusion of the abdominal aorta presenting in infancy. This child presented at four months of age with heart failure and hypertension. CT angiogram showed total narrowing of the abdominal aorta. This was initially felt to be too severe for surgical treatment and he was planned for palliative care. We were however able to improve his blood pressure with antihypertensive agents and he underwent succesful angioplasty at five and a half months of age. He has required three further angioplasty procedures and still needs two antihypertensive agents to control his blood pressure. His renal function remains normal and at age six years he has excellent quality of life with normal growth and development. This case illustrates that the combination of medical treatment and angioplasty can give an excellent long-term treatment response even in children with extremely severe MAS. This boy now has normal blood pressure and has experienced normal growth, development and quality of life.
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References
Lewis VD, Meranze SG, McLean GK, O’Neill JA Jr, Berkowitz HD, Burke DR (1988) The midaortic syndrome: diagnosis and treatment. Radiology 167:111–113
Panayiotoupoulos YP, Tyrrell MR, Koffman G, Reidy JF, Haycock GB, Taylor PR (1996) Midaortic syndrome presenting in childhood. Br J Surg 83:235–240
Flynn PM, Robinson MB, Stapleton FB, Roy S 3rd, Koh G, Tonkin IL (1984) Coarctation of the aorta and renal artery stenosis in tuberous sclerosis. Pediat Radiol 14:337–339
Tullus K, Brennan E, Hamilton G, Lord R, McLaren CA, Marks SD, Roebuck DJ (2008) Renovascular hypertension in children. Lancet 26:371(9622):1453–1463
Fava MP, Foradori GB, Garcia CB, Cruz FO, Aguilar JG, Kramer AS, Valdes FE (1993) Percutaneous transluminal angioplasty in patients with Takayasu arteritis: five-year experience. J Vasc Interv Radiol 4(5):649–652
Tummolo A, Marks SD, Stadermann M, Roebuck DJ, McLaren CA, Hamilton G, Dillon MJ, Tullus K (2009) Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol 24(11):2225–2232
Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KE (2008) Idiopathic mid-aortic syndrome in children. Pediatr Nephrol 23(7):1135–1142
Delis KT, Gloviczki P (2005) Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Perspect Vasc Surg Endovasc Ther 17(3):187–203
Das BB, Recto M, Shoemaker L (2008) Midaortic syndrome presenting as neonatal hypertension. Pediatr Cardiol 29(5):1000–1001
Siwik ES, Perry SB, Lock JE (2003) Endovascular stent implantation in patients with stenotic aortoarteriopathies: early and medium-term results. Catheter Cardiovasc Interv 59(3):380–386
Ballweg J, Liniger R, Rocchini A, Gajarski R (2006) Use of Palmaz stents in a newborn with congenital aneurysms and coarctation of the abdominal aorta. Catherter Cardiovasc Interv 68(4):648–652
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Minson, S., McLaren, C.A., Roebuck, D.J. et al. Infantile midaortic syndrome with aortic occlusion. Pediatr Nephrol 27, 321–324 (2012). https://doi.org/10.1007/s00467-011-2039-y
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DOI: https://doi.org/10.1007/s00467-011-2039-y