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Year : 2012  |  Volume : 13  |  Issue : 3  |  Page : 116-117  

Middle aortic syndrome caused by Takayasu arteritis

Department of Pediatrics, Lokmanya Tilak Municipal ­General Hospital and Medical College, Sion, Mumbai, India

Date of Web Publication9-Oct-2012

Correspondence Address:
Syed Ahmed Zaki
Room no.509, new RMO quarters, Sion, Mumbai-400022
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1995-705X.102158

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The middle aortic syndrome (MAS) is a rare condition characterized by diffuse narrowing of the descending thoracic aorta, abdominal aorta, or both. It can be congenital or acquired due to several conditions. We report an 8-year-old girl who developed middle aortic syndrome due to Takayasu arteritis.

Keywords: Takayasu arteritis, middle aortic syndrome, descending aorta, abdominal aorta

How to cite this article:
Zaki SA. Middle aortic syndrome caused by Takayasu arteritis. Heart Views 2012;13:116-7

How to cite this URL:
Zaki SA. Middle aortic syndrome caused by Takayasu arteritis. Heart Views [serial online] 2012 [cited 2020 Sep 23];13:116-7. Available from: http://www.heartviews.org/text.asp?2012/13/3/116/102158

   Introduction Top

The middle aortic syndrome (MAS) is an uncommon lesion characterized by severe aortic narrowing in the descending thoracic aorta, abdominal aorta, or both. [1],[2] Takayasu's arteritis is a chronic, autoimmune, and potentially progressive inflammatory disease of large vessels with a preference for the aorta and its major branches. [3]

   Case Presentation Top

An 8-year-old girl presented with vomiting and headache of 3 days duration. There was no history of rash, abdominal pain, visual blurring, altered sensorium, convulsion urinary, or bowel complaints. There was history of loss of appetite and low grade fever since 1 month. Her uncle had pulmonary tuberculosis and was on antituberculous therapy. Birth history, developmental history, and past history were normal. On examination, she was afebrile with a heart rate of 106/min, respiratory rate of 24/min, and blood pressure of 180/110 mmHg (>95 th percentile for age and sex). All her peripheral pulses were felt normally. Fundoscopic examination was normal. General examination and systemic examination were normal. Investigations revealed a normal hemogram. Erythocyte sedimentation rate was 110 mm at the end of 1h; serum electrolytes, renal and liver function tests, serum calcium, and serum phosphorous were normal. Urine microscopy, cerebrospinal fluid examination, and chest X-ray were normal. Antinuclear antibody titers and anti-double stranded DNA titers were negative. Mantoux test was strongly positive (28 mm). Ultrasonographic examination of the abdomen revealed multiple necrotic mesenteric and retro-peritoneal lymph nodes with the largest being 1.7 × 1.1 cm. Her blood pressure was gradually brought under control with oral nifedepine (0.5 mg/kg, 6 hourly), hydrallazine (6 mg/kg/day), and propranolol (0.5 mg/kg/dose, 6 hourly). In view of persistent hypertension, a computed tomography aortogram was done, which showed narrowing of the Ostia of Celiac, superior mesenteric artery, and bilateral renal arteries, with post-stenotic dilatation and intima-media thickening of the abdominal aorta [Figure 1] and [Figure 2]. Color Doppler of the neck vessels and bilateral subclavian arteries showed normal flow. As per EULAR (European League Against Rheumatism)/PReS (Paediatric Rheumatology European Society) consensus criteria, the diagnosis of Takayasu's arteritis was confirmed as the child had angiographic abnormalities and hypertension. [4] She was started on immunosuppressant therapy, i.e. oral prednisolone (2 mg/kg/ day) and methotrexate (2.5 mg/kg/day twice weekly). In view of the strongly positive Mantoux test, elevated ESR, and abdominal lymphadenopathy, a diagnosis of abdominal tuberculosis was made and the child was started on anti-tuberculosis treatment. Percutaneous transluminal balloon angioplasty was planned. However, due to financial constraints, she could not undergo surgery immediately and was discharged. On follow up after 1 month, her blood pressure is well controlled. She is asymptomatic and awaiting her surgery.
Figure 1: Computed tomography angiography of the abdomen showing bilateral renal arteries severely narrowed at their Ostia with post-stenotic dilatation. Also the abdominal aorta shows increased intima and media thickness

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Figure 2: Computed tomography angiography of the abdomen showing superior mesenteric artery narrowed at its origin with normal distal course. Also seen is coeliac artery narrowed just beyond its origin with post-stenotic dilatation

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   Discussion Top

MAS was first described by Sen et al. in 1963. [2] Although the etiology of MAS is not well known, congenital, inflammation, developmental disorder, and infection are thought to be some of the causes. [1],[5] The disease is an important cause of hypertension in children and young adults and is associated with a high morbidity and mortality. It typically presents as severe hypertension in young patients who have weak or absent femoral pulses and an abdominal or lower back bruit. [1],[2],[5] Renal artery stenosis is found in 80% of patients, and 25% have involvement of the superior mesenteric artery, inferior mesenteric artery, or celiac axis. [5] MAS has been described in Takayasu arteritis, fibromuscular dysplasia, a number of genetic diseases, such as neurofibromatosis, mucopolysaccharidosis, Williams syndrome, Turner syndrome, Alagille syndrome, and congenital rubella syndrome. [1],[5] In addition, irradiation of abdominal or retroperitoneal neoplasm early in life, particularly in the case of Wilms tumor, has resulted in a similar angiographic appearance. [5] The diagnosis of this rare syndrome is made by computed tomography, magnetic resonance imaging, or angiography. [5]

   Conclusion Top

The natural history of MAS is unknown. A majority agree that these patients should undergo revascularization whenever feasible. Balloon angioplasty or autotransplantation for renal artery stenosis and stent implantation or bypass graft for stenosed aortic segments are the suggested therapeutic options in the treatment of middle aortic syndrome. [5] Staged vascular repair has been recommended by some authors to minimize renal ischemia. [6]

   Acknowledgement Top

We would like to thank the Dean of our institution for permitting us to publish this manuscript.

   References Top

1.Uwabe K, Okada O, Harada M. Ascending to descending aorta bypass for middle aortic syndrome. Circ J 2007;71:1162-3.  Back to cited text no. 1
2.Sen PK, KInare SG, Engineer SD, Parulkar GB. The middle aortic syndrome. Br Heart J 1963;25:610-8.  Back to cited text no. 2
3.Saab F, Giugliano RP, Giugliano GR. Takayasu arteritis in a young woman: A 4-year case history. Tex Heart Inst J 2009;36:470-4.  Back to cited text no. 3
4.Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006;65:936-41.  Back to cited text no. 4
5.Alehan D, Kafali G, Demircin M. Middle aortic syndrome as a cause of dilated cardiomyopathy. Anadolu Kardiyol Derg 2004;4:178-80.  Back to cited text no. 5
6.Lillehei CW, Shamberger RC. Staged reconstruction for middle aortic syndrome. J Pediatr Surg 2001;36:1252-4.  Back to cited text no. 6


  [Figure 1], [Figure 2]

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