Middle Aortic Syndrome |
| Middle
aortic syndrome (MAS) is a rare condition involving diffuse narrowing
of the distal thoracic or abdominal aorta involving its visceral and
renal branches. The etiology of MAS is unknown and occurs in young
patients. MAS have been associated with neurofibromatosis, Alagille's
and William syndrome. The mesenteric vascular stenosis is clinically
silent, while the renal stenosis is responsible for the clinical
picture of hypertension in these children. Absent femoral pulses and an
abdominal bruit are also present. Bilateral involvement is common.
Ultrasonography is the primary screening technique. Biplanar
arteriography is the standard imaging to establish the diagnosis and
involvement of the disease. Arteriography shows a smooth segmental
narrowing of the abdominal aorta with concomitant stenosis at the
origins of the renal arteries. Primary treatment goals are preservation
of functional renal tissue and amelioration of the renal hypertension.
Therapeutic approaches included medical management, percutaneous
transluminal angioplasty and/or surgical intervention. Repair of the
narrowing requires prosthetic material for bypass or patch
reconstruction. Techniques used to revascularize the kidney included
thoracoabdominal to infrarenal aortic bypass with renal artery
reimplantation, splenorenal bypass, gastroduodenal to renal bypass,
aortorenal bypass and autotransplantation. References: 1- Panayiotopoulos YP, Tyrrell MR, Koffman G, Reidy JF, Haycock GB, Taylor PR: Mid-aortic syndrome presenting in childhood. Br J Surg. 83(2):235-40, 1996 2- Tummolo A, Marks SD, Stadermann M, Roebuck DJ, McLaren CA, Hamilton G, Dillon MJ, Tullus K: Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol. 24(11):2225-32, 2009 3- Lin YJ, Hwang B, Lee PC, Yang LY, Meng CC: Mid-aortic syndrome: a case report and review of the literature. Int J Cardiol. 123(3):348-52, 2008 4- Chocrón de Benzaquen S, Munoz Lopez M, Madrid Aris AD, Castellote Alonso A, Enriquez G, Nieto Rey JL: [Mid-aortic syndrome: case studies from a paediatric nephrology department]. An Pediatr (Barc). 75(1):33-9, 2011 5- Bleacher J, Turner ME, Quivers E, Schwartz MZ: Renal autotransplantation for renovascular hypertension caused by midaortic syndrome. J Pediatr Surg. 32(2):248-50, 1997 6- Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KE: Idiopathic mid-aortic syndrome in children. Pediatr Nephrol. 23(7):1135-42, 2008 |
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