Staged reconstruction for middle aortic syndrome

Middle aortic syndrome is a rare condition that involves narrowing of the abdominal aorta and its visceral branches. The authors propose staged vascular repair to minimize renal ischemia and facilitate use of native arterial tissue for reconstruction.

Three adolescents (age 8(1/2), 12(1/2), 13(1/2)) presented with severe hypertension. Subsequent evaluation showed coarctation of the abdominal aorta extending above the celiac axis. All 3 patients had bilateral renal artery stenoses. There also were tight stenoses of the celiac or superior mesenteric arteries. In the first stage the right renal artery stenosis was relieved. In the youngest patient, this was accomplished by balloon angioplasty. However, in the other 2, right renal autotransplantation was performed to the right iliac vessels using end-to-side anastomoses of the renal artery and vein. Cold perfusion was used. The second stage was performed 2 to 5 months later via a thoracoabdominal approach in 2 patients. A Dacron tube graft was utilized from above the coarctation to the iliac bifurcation. The left renal arteries were detached and anastomosed end to side to the bypass graft. In 1 child there were actually 3 separate renal arteries that required reimplantation. In the youngest patient the aortic narrowing was relieved by a long Dacron patch aortoplasty and interposition of an internal iliac artery graft to the left renal artery.

All 3 patients recovered well and returned to full activities. There was no measurable rise of BUN or serum creatinine postoperatively. Postoperative renal scans showed good renal perfusion bilaterally. Follow-up results 2 to 10 years later continue to show well functioning reconstructions.

A staged approach is an effective reconstruction for children with middle aortic syndrome which minimizes risk to renal function.