Abstract
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A 49-year-old woman with past medical history of neurofibromatosis type 1 (NF1) presented with symptoms of claudication and hypertension.
CT angiography of the abdomen and pelvis was performed (Figure). Cinematic rendering (1) depicts moderate narrowing of the suprarenal through infrarenal abdominal aorta spanning the L1 through L4 vertebral bodies. The origins of the two right and first and third left main renal arteries and inferior mesenteric artery are also stenotic. The second left main renal artery is occluded after its takeoff from the aorta, with reconstitution in the left renal hilum. Variant origins of the hepatic and splenic arteries arising directly from the aorta are also noted and are otherwise normal caliber. Similarly, the superior mesenteric artery is normal caliber.
(a) Cinematic rendered image from CT angiography of the abdomen and pelvis demonstrates midaortic syndrome with moderate aortic narrowing spanning from L1 through L4 vertebral bodies (black arrow) with associated stenoses of the two right and first and third left main renal arteries (white arrowheads) and inferior mesenteric artery (white arrow) at the aortic origins. The second left main renal artery is occluded (black arrowhead) after its origin with reconstitution in the left renal hilum. Hypoenhancement of the lower pole of the left kidney is noted (Movie 1). (b) Cinematic rendered image from CT angiography of the abdomen and pelvis following open repair depicts normal-caliber aortic graft (black arrow) with transplanted bilateral aortorenal bypass (arrowheads) and inferior mesenteric artery (white arrow). Hypoenhancement of the lower poles of both kidneys is also noted and is attributed to the accessory renal arteries not being transplanted (Movie 2).
(a) Cinematic rendered image from CT angiography of the abdomen and pelvis demonstrates midaortic syndrome with moderate aortic narrowing spanning from L1 through L4 vertebral bodies (black arrow) with associated stenoses of the two right and first and third left main renal arteries (white arrowheads) and inferior mesenteric artery (white arrow) at the aortic origins. The second left main renal artery is occluded (black arrowhead) after its origin with reconstitution in the left renal hilum. Hypoenhancement of the lower pole of the left kidney is noted (Movie 1). (b) Cinematic rendered image from CT angiography of the abdomen and pelvis following open repair depicts normal-caliber aortic graft (black arrow) with transplanted bilateral aortorenal bypass (arrowheads) and inferior mesenteric artery (white arrow). Hypoenhancement of the lower poles of both kidneys is also noted and is attributed to the accessory renal arteries not being transplanted (Movie 2).
Movie 1:
Cinematic rendering of preoperative appearance of the aorta, renal arteries, and hepatic, splenic, and inferior mesenteric arteries.
Movie 2:
Cinematic rendering of postoperative appearance of the aorta with transplanted bilateral aortorenal bypass and inferior mesenteric artery.
Neurofibromin protein is normally found in both endothelial and smooth muscle cells of blood vessels. Patients with NF1 may have abnormal healing of their vessels following vessel wall injury, resulting in muscle loss and fibrosis with fibromuscular hyperplasia and neural proliferation. This process can affect the aorta through the arterioles and may manifest as vascular stenosis, occlusion, aneurysm, pseudoaneurysm, rupture, and fistula (2). The renal artery is the most common site of vascular complications in NF1 with resultant hypertension. Abdominal aortic coarctation is also associated with NF1 (3).
The patient underwent open repair of the abdominal aorta with renal and inferior mesenteric artery bypass for treatment of midaortic syndrome with renal and inferior mesenteric artery stenoses. Follow-up CT angiogram of the abdomen with cinematic rendering shows patent, normal-caliber aortic graft with transplanted bilateral aortorenal bypass and inferior mesenteric artery. Decreased enhancement in the inferior poles of both kidneys is also noted and is attributed to loss of the additional duplicated renal arteries during renal artery transplantation.
Footnotes
Disclosures of Conflicts of Interest: J.R.A. disclosed no relevant relationships. E.K.F. Activities related to the present article: disclosed grant to author’s institution from Siemens. Activities not related to the present article: disclosed money paid to author as co-founder and shareholder in HipGraphics; disclosed money paid to author’s institution from GE for research and education support. Other relationships: disclosed no relevant relationships.
References
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