Idiopathic midaortic syndrome with malignant hypertension in 3-year-old boy

• Main Authors: Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
• Format: Article
• Language: English
• Published: Korean Pediatric Society 2016-11-01
• Series: Korean Journal of Pediatrics
• Subjects: Aortic coarctation; Renovascular hypertension; Renal artery obstruction
• Online Access: http://kjp.or.kr/upload/pdf/kjped-59-S84.pdf
• ISSN: 1738-1061; 2092-7258

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.