midaortic syndrome

A case of
Midaortic syndrome secondary to burned out granulomatous aortoarteritis.. Diffuse exaggerated intimo-medial calcifications extending from the root of aorta up to the post renal segment
www.eurorad.org • CC by-nc-sa-4.0
A case of
Midaortic syndrome secondary to burned out granulomatous aortoarteritis.. VRT image showing diffuse long segment narrowing of inter-renal segment of abdominal aorta at proximal lumar level extending for a lenght of approximately 60mm.
www.eurorad.org • CC by-nc-sa-4.0
A case of
Midaortic syndrome secondary to burned out granulomatous aortoarteritis.. VRT image showing diffuse long segment narrowing of inter-renal segment of abdominal aorta at proximal lumar level extending for a lenght of approximately 60mm.
www.eurorad.org • CC by-nc-sa-4.0
Teenager with
malignant hypertension and an abdominal bruit. 3D CT angiogram with contrast of the abdomen shows diffuse narrowing of the mid abdominal aorta and marked narrowing of the origins of the bilateral renal arteries.The diagnosis was midaortic syndrome with bilateral renal artery stenosis.
pediatricimaging.org • CC-by-nc-sa 4.0
Aortic bypass
and orthotopic right renal autotransplantation for midaortic syndrome: a case report. Preoperative imaging showing multiple arterial stenoses. A, Posteroanterior view of three-dimensional computed tomography angiography showing coarctation of the abdominal aorta (middle arrow), bilateral renal artery stents (two upper arrows) and arc of Riolan (lower arrow). B, Superior mesenteric artery stenosis (lower arrow) and celiac artery stenosis (upper arrow).
bmcsurg.biomedcentral.com • CC-by-4.0
Aortic bypass
and orthotopic right renal autotransplantation for midaortic syndrome: a case report. Postoperative imaging showed that all the grafts were patent. A, Postoperative three-dimensional computed tomography angiography showing the extra-anatomic bypass (right arrow) with the autogenous saphenous vein grafts (left arrows). B, Multi-planar reconstruction showing that the new prosthetic graft is patent. C, Curved-planar reformation of the computed tomography angiography showing that the saphenous vein grafts (two arrows) are patent.
bmcsurg.biomedcentral.com • CC-by-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Midaortic syndrome is an uncommon entity affecting children and young adults. It is characterized by progressive narrowing of the abdominal aorta and its major branches.

Clinical presentation

The onset of symptoms is usually during childhood or young adulthood :

  • hypertension (most common)
  • intermittent claudication
  • renal failure

Pathology

The exact cause is unknown in most cases though it is probably due to an insult during intrauterine life. Whilst intimal and subintimal fibrosis and fragmentation of the elastic media are seen, arteritis and atherosclerosis are characteristically absent in this condition.

Elastin arteriopathy in Williams syndrome can rarely be a cause .

Radiographic features

Ultrasound

On gray‐scale imaging, segmental narrowing or tortuosity can be detected in the distal thoracic and the abdominal aorta.

Color Doppler can demonstrate aliasing while spectral Doppler can show elevated peak systolic velocities in the narrowed vessels (often ranging around 230~480 cm/s). Tardus‐parvus waveforms can be seen in the vessels distally of the stenosis.

Angiography (DSA)

Whilst involvement of any portion of the abdominal aorta may be seen, the interrenal segment including the origins of the renal arteries is often narrowed. Typically enlarged splanchnic collaterals (e.g. inferior mesenteric artery, arc of Riolan, the marginal artery of Drummond) are noted. The aortic bifurcation and iliac arteries are spared .

Treatment and prognosis

Endovascular treatment is the preferred mode as it is a minimally invasive treatment option. Balloon expandable stents / stent-grafts are usually placed to expand the lumen of the aorta. Complex endovascular/surgical options such as fenestrated grafts with re-implantation of the renal arteries will be required if the renal arteries are involved.

Without treatment, life expectancy is dramatically shortened with most dying of ischemic/hypertensive complications by age 40 .

Differential diagnosis

Imaging differential considerations include :

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Mid-Aortic-Syndrom:
Takayasu-Arteriitis
 
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