Type:
Educational Exhibit
Keywords:
Obstruction / Occlusion, Hypertension, Dilation, Ultrasound-Spectral Doppler, MR-Angiography, CT-Angiography, Vascular, Paediatric, Arteries / Aorta
Authors:
G. A. Finol1, M. Castano Reyero2, V. S. Teran Pareja2, I. NAVAS FERNANDEZ-SILGADO2, M. De la Puente Herraiz2, P. A. Encinas Escobar2; 1MADRID, MADRID/ES, 2Madrid/ES
DOI:
10.1594/ecr2018/C-0798
Background
Mid-Aortic syndrome (MAS) is a rare entity characterized by narrowing of the abdominal aorta and its major branches.
There will be involvement of the renal arteries in most patients.
Although stenosis of the thoracic aorta is possible,
it is uncommon.
Tipically affects children and young adults.
Most cases are idiopathic.
There are several theories about its possible origin being the most accepted a probable lack of fusion of both dorsal aortas in embryonic life with atrophy and stenosis of one of them.
MAS may also be secondary to other diseases such as Takayasu arteritis,
Williams syndrome,
mucolipidosis and neurofibromatosis.
However,
morphological findings are similar regardless of the etiology.
Hypertension is the most common symptom.
The course of the disease depends on the degree of stenosis,
the affected arteries and the progressive damage caused by renovascular hypertension. If left untreated,
patients usually die due to renal failure or severe ischemic complications.