Idiopathic Middle Aortic Syndrome Causing Pediatric Resistant Hypertension: Beyond Coarctation

Middle aortic syndrome (MAS) is a rare cause of severe hypertension in children, characterized by segmental narrowing of the distal thoracic and abdominal aorta. Early recognition is essential to prevent irreversible end-organ damage.

A 12-year-old girl presented with resistant hypertension, headache, facial edema, blurred vision, and postprandial abdominal pain. Marked upper-to-lower limb blood pressure discrepancy, with absent femoral pulses. Computed tomography angiography demonstrated long-segment thoracoabdominal aortic narrowing extending to the iliac arteries, consistent with MAS. Because of unfavorable anatomy for endovascular treatment. Thoracoabdominal extra-anatomic aorto-aortic bypass with polytetrafluoroethylene graft was successfully performed.

This case highlights the diagnostic challenge of idiopathic MAS and demonstrates that individualized management based on vascular anatomy can achieve excellent outcomes.

Cross-sectional vascular imaging should be considered in children with resistant hypertension and diminished femoral pulses. Open aorto-aortic bypass remains effective when endovascular treatment is unsuitable.