Middle aortic syndrome (MAS) is rare entity described as a progressive narrowing of the aorta between the aortic arch and terminal bifurcation of the aorta. Surgical treatment is with the use of grafts for bypassing the narrow aortic segment. Rarely, graft complications may occur over long periods of time due to the axial growth of the patient and loss of tensile strength of the graft material. This is a case of MAS, presenting 34 years after initial surgery due to aneurysmal dilation of the graft, treated with redo surgery and complete recovery of the patient

Bhate, Sameer

Bhate, Sudhir G

Goel, Ridhima

Kamerkar, Dhanesh R

English

Atharva Scientific Publications (E-Jounals)

Vol 4 | Issue 3 | May - Jun 2018 Indian J Case Reports 179
Case Report
A rare case of long-term graft complication in a patient of middle aortic syndrome
Ridhima Goel1, Sameer Bhate2, Sudhir G Bhate3, Dhanesh R Kamerkar4
From 1Intern, 2Head, Department of Cardio-Vascular-Thoracic Surgery, Maharishi Markandeshwar Institute of Medical Sciences and Research, 
Mullana, Haryana, 3Head and Consultant, Department of Cardio-Thoracic Surgery, 4Head, Department of Vascular Surgery, Ruby Hall Clinic, Pune, 
Maharashtra, India
Correspondence to: Ridhima Goel, Dr. Naresh Hospital and Heart Centre, K.C. Road Barnala, Punjab - 148 101, India. 
E-mail: ridhima.goel547@gmail.com
Received – 02 May 2018 Initial Review – 04 June 2018 Published Online – 22 June 2018
Middle aortic syndrome (MAS) is a rare condition, seen typically in the pediatric population as a stenotic aorto-arteriopathy, which was originally described 
by Sen et al. [1] as a progressive aortic narrowing occurring 
between the arch and terminal bifurcation of the aorta. It most 
commonly presents in children and young adults with refractory 
hypertension [1]. Management of these patients usually requires 
surgical intervention, although antihypertensive medications 
may be sufficient in patients with milder diseases and no other 
symptoms [2,3].
Despite minimally invasive endovascular treatment options, 
aortoaortic bypass by the use of autologous or prosthetic grafts of 
the stenosed segment is the most definitive treatment option [2,3]. 
Long-term complications may arise later in life when the graft 
material undergoes degeneration as the patient grows in a linear 
fashion, although the reported rates of such instances are very 
low [4].
We report a case of MAS in a 45-year-old female who was 
treated with extra-anatomic bypass grafting as an 11-year-old 
child and presented 34 years later with a pulsatile pseudoaneurysm 
of the graft due to degeneration of the graft.
CASE REPORT
A 45-year-old female presented with a pulsatile swelling in the 
abdomen, of 3 months duration. She did not report any other 
complaints; there was no history of fever, extremity pain or 
claudication, and peripheral edema. The patient had no history 
of smoking, tobacco use, or alcohol intake. The patient had 
been diagnosed to have MAS, for which she had been treated 
with extra-anatomic aortoaortic bypass surgery 34 years back, 
using a Dacron graft and had an uneventful post-operative 
recovery.
She had been asymptomatic and has also had two normal, 
full-term deliveries since then, without any complications. On 
clinical examination, the patient had stable vital signs. There 
was a pulsatile swelling in hypochondrium, non-tender, and on 
auscultation, a bruit could be heard over the swelling. Peripheral 
pulses were normal. She was not on any medication.
The patient was investigated with contrast-enhanced computed 
tomography (CT), which showed a dilatation of the graft material, 
measuring 7 cm×4 cm (Fig. 1, Panel A and Fig. 2, Panel A and B). 
The most important finding in addition to the aneurysm was that 
there was no antegrade flow in the descending aorta and the flow 
in the aorta post the occluded segment was dependent on the graft 
in a retrograde manner.
The patient was treated with redo aortoaortic bypass surgery, 
wherein the old graft was dissected and replaced using a new 
18 mm silver-coated graft using deep hypothermic circulatory 
arrest, where the body temperature is lowered to 18°C.
The patient had an uneventful post-operative course. The post-
operative CT shows a normal graft and the patient was discharged 
on the 10th post-operative day (Fig. 1 Panel B and Fig. 2 Panel C). 
At follow-up, the patient was doing well, with no complaints.
DISCUSSION
MAS is a rare cause of refractory hypertension in the pediatric 
population, and diagnosis of this entity requires a high degree 
of suspicion on the part of the treating physician in any patient 
who presents with the triad of the thoracoabdominal bruit, 
decreased femoral pulses, and absence of characteristic clinical or 
ABSTRACT
Middle aortic syndrome (MAS) is rare entity described as a progressive narrowing of the aorta between the aortic arch and 
terminal bifurcation of the aorta. Surgical treatment is with the use of grafts for bypassing the narrow aortic segment. Rarely, graft 
complications may occur over long periods of time due to the axial growth of the patient and loss of tensile strength of the graft 
material. This is a case of MAS, presenting 34 years after initial surgery due to aneurysmal dilation of the graft, treated with redo 
surgery and complete recovery of the patient.
Key words: Dacron graft, Middle aortic syndrome, Redo surgery
Goel et al. Middle aortic graft long-term complication
Vol 4 | Issue 3 | May - Jun 2018 Indian J Case Reports 180
radiological features of other thoracic coarctations [5]. Surgical 
management provides the best outcome for the patient, with 
adequate control of hypertension and end-organ functioning.
This entity forms <2% of all aortic stenosis [6] and classically 
presents as refractory hypertension proximal to stenosis and 
hypotension distally, along with symptoms of nosebleeds, 
severe headaches, chest pains, lower limb claudication, and 
even cardiac failure [2]. Left untreated, this condition can 
progress to severe complications such as coronary artery 
disease, congestive heart failure, left ventricular hypertrophy, 
cerebrovascular accidents, hypertensive encephalopathy, and 
retinopathy, with most patients succumbing to the disease 
before 40 years of age [5]. On examination, absent femoral 
pulses and an abdominal bruit may be evident, depending on 
the degree of stenosis [2].
The etiology of this disease remains idiopathic for the 
majority of cases, although it is postulated to be due to an 
embryological defect, or associated with Mendelian disorders 
such as neurofibromatosis Type 1, Alagille syndrome, and 
Williams’ syndrome [2]. The presentation of this disease may 
mimic other syndromes or present along with them, such as 
Takayasu arteritis, neurofibromatosis, fibromuscular dysplasia, 
retroperitoneal fibrosis, mucopolysaccharidosis, and congenital 
vascular abnormalities [2,3].
This disease process most frequently involves the abdominal 
aorta, along with visceral arteries such as renal (>80%), splanchnic 
(70–50%), superior mesenteric (60–30%), and celiac (60–20%), 
but the inferior mesenteric is almost never involved [2,3]. Based 
on the involvement of renal arteries, this syndrome can be 
classified into four major types: Type I - suprarenal coarctation 
and renal artery stenosis; Type II - infrarenal coarctation and renal 
artery stenosis; Type III - suprarenal coarctation and normal renal 
arteries; and Type IV - infrarenal coarctation and normal renal 
arteries [5]. Only the first three types require intervention for renal 
blood flow. Our patient had a stenosed segment extending from 
the descending aorta to the abdominal aorta with no collaterals.
The gold standard for diagnosis is intra-arterial digital 
subtraction angiography [7], which not only confirms the 
diagnosis but also delineates the sites of stenosis before surgical 
intervention. The stenosis may also be visualized by Color 
Doppler, magnetic resonance angiography, and computed 
tomographic angiography.
Treatment of this modality depends mainly on the severity of 
the disease, with mild cases being managed by medical treatment 
alone, but invasive procedures are required for more severe 
disease [2,3]. Various surgical procedures including aortoaortic 
bypass grafting, graft vascular replacement, patchy angioplasty, 
and renal autotransplantation have been described, of which 
prosthetic or autologous venous surgical arterial bypass grafting 
remains the treatment of choice [2]. Endovascular procedures 
such as percutaneous transluminal angioplasty with or without 
stenting have also been tried in some patients, but the reported 
rates of procedure failure are higher in this group [2].
Surgical correction in a pediatric patient carries the risk 
of graft stenosis or pseudoaneurysm formation as the patient 
grows in an axial fashion. The growth of the axial skeleton is 
comparatively less after the age of 10 years, and the growth of the 
aorta is mainly seen above and below the level of the graft, with 
the minimal lengthening of the bypassed segment [4]. Therefore, 
it is recommended to put the surgical treatment on hold till the 
patient has attained complete growth and manage the patient with 
antihypertensive medications [4]. To prevent these complications, 
measures such as the use of an end-to-side aortoaortic bypass, 
oversizing the length and diameter of the bypass, and use of 
crimped Dacron grafts may be undertaken [4,8]. The extra-
anatomic axillofemoral bypass may be done if other procedures 
fail to provide relief as a last resort measure [9]. Some authors 
have recommended a follow-up with CT at the interval of every 
5 years in patients with aortic surgery as prosthetic materials are 
subjected to wear and tear, similar to autologous grafts, which 
may then lead to graft degeneration [4,10]. In our extensive 
review of the published literature, few cases of non-anastomotic 
aneurysmal graft degeneration have been reported, and out of 
them, only one case was of a patient with MAS, who presented 
years later as an adult with an aortic graft-enteric fistula and went 
into pulseless activity during the redo operation [4,11-13].
Figure 1: Contrast-enhanced computed tomography scan of the 
graft. Panel A: Pre-operative view; Panel B: Post-operative view 
after redo correction surgery
Figure 2: Volume-rendered computed tomography scan of the graft. 
Panel A and B: Pre-operative view, Panel C: Post-operative view 
after redo correction surgery
Goel et al. Middle aortic graft long-term complication
Vol 4 | Issue 3 | May - Jun 2018 Indian J Case Reports 181
CONCLUSION
MAS is a rare cause of unresponsive hypertension in children 
and treatment requires multimodality management, with surgical 
interventions forming the backbone of the treatment. Prosthetic 
grafting done in childhood may experience wear and tear as the 
patient grows and may show complications of graft material 
degeneration such as pseudoaneurysms. Periodic follow-up of the 
patient with a multispecialty team of experienced physicians is 
required to monitor a patient with these rare complications of this 
uncommon disease process.
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Funding: None; Conflict of Interest: None Stated.
How to cite this article: Goel R, Bhate S, Bhate SG, Kamerkar DR. A rare 
case of long-term graft complication in a patient of middle aortic syndrome. 
Indian J Case Reports. 2018;4(3):179-181.
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A rare case of long-term graft complication in a patient of middle aortic syndrome

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A rare case of long-term graft complication in a patient of middle aortic syndrome

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