Pediatric Midaortic Syndrome

  • Etiology: Probably due to in utero insult
  • Imaging:
    — Progressive narrowing of abdominal aorta and its major branches
    — Classically the narrowing is near the origins of the renal arteries with sparing of the aortic bifurcation and iliac arteries
  • DDX:
  • Complications: Without treatment most die of ischemic or hypertensive complications by age 40
  • Treatment: Endovascular stenting
  • Clinical: Present during childhood or young adulthood with hypertension, intermittent claudication, renal failure

Radiology Cases of Midaortic Syndrome

CT of midaortic syndrome and bilateral renal artery stenosis
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.

Radiology Cases of Bilateral Renal Artery Stenosis Due to Midaortic Syndrome in Neurofibromatosis Type 1

CT angiogram and spectral doppler US of bilateral renal artery stenosis in a patient with neurofibromatosis Type 1
3D reconstruction of a CT angiogram with contrast of the chest and abdomen (left) shows diffuse moderate narrowing of the mid aorta and marked narrowing of the origins of the renal arteries bilaterally. Spectral doppler US of the renal arteries (right) shows parvus tardis waveforms within them bilaterally.
US and angiogram of bilateral renal artery stenosis due to midaortic syndrome in a patient with Neurofibromatosis Type I
Spectral doppler US of the kidneys (above) shows tardus parvus waveforms in the renal arteries bilaterally. AP image from an abdominal angiogram (below) shows bilateral stenosis of the renal arteries at their origins and progressive narrowing of the mid abdominal aorta centered near the renal artery origins with sparing of the aortic bifurcation.

Radiology Cases of Celiac Artery and Superior Mesenteric Artery and Bilateral Renal Artery Stenosis Due to Midaortic Syndrome in Neurofibromatosis Type 1

CT of midaortic syndrome
Axial CT with contrast of the abdomen shows the aorta to have a normal caliber in the upper abdomen (above left) and a markedly decreased caliber in the mid-abdomen (above right). Sagittal CT (below left) shows a long length of progressive narrowing of the mid-abdominal aorta. 3D CT (below right) shows moderate stenosis of the abdominal aorta from T12 to L2 and severe ostial stenosis of the celiac artery and superior mesenteric artery and the bilateral renal arteries. The inferior mesenteric artery is enlarged with robust collaterals to the celiac and superior mesenteric circulations. There is sparing of the aortic bifurcation and iliac arteries.