Background: Middle aortic syndrome (MAS) is a rare condition characterized by stenosis of the proximal abdominal aorta and the origin of the renal and digestive arteries. When medical therapy and interventional radiology fail to control threatening reno-vascular arterial hypertension (AHT), surgery is required and may need several interventions, which are usually delayed until late childhood.
Case: We report on a 3-year-old girl with severe AHT (180/130 mmHg) caused by MAS. There was no evidence of generalized vascular disease or complications of AHT. AHT failed to respond to medical therapy (five drugs), endovascular dilatation, and stenting was considered unfeasible due to the complex multiple strictures. Surgery consisted of: explantation of the two kidneys; aortic bypass between the lower thoracic and lower abdominal aorta using a prosthetic graft; reimplantation of the kidneys onto the normal iliac arteries. The post-operative course was uneventful. Owing to recurrent stenosis of the re-implanted renal arteries, endoluminal dilatations were performed 4 and 5 months after surgery. Two years after surgery, the child is alive and well, off anti-hypertensive therapy, with normal blood pressure.
Conclusion: Mild aortic syndrome can be treated with a one-stage surgical repair with aorto-aortic bypass and bilateral auto-transplantation, even in young children.