Korean J Pediatr.  2016 Nov;59(Suppl 1):S84-S87. 10.3345/kjp.2016.59.11.S84.

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

• Ahn KJ
• Yoon JK
• Kim GB
• Kwon BS
• Bae EJ
• Noh CI

• KMID: 2362263
• DOI: http://doi.org/10.3345/kjp.2016.59.11.S84

Abstract

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.

Keyword

Aortic coarctation; Renovascular hypertension; Renal artery obstruction

MeSH Terms

Antihypertensive Agents
Aorta, Abdominal
Aortic Coarctation
Blood Pressure
Child
Child, Preschool*
Constriction, Pathologic
Diagnosis
Early Diagnosis
Humans
Hypertension
Hypertension, Malignant*
Hypertension, Renovascular
Hypertrophy, Left Ventricular
Korea
Lower Extremity
Male*
Physical Examination
Renal Artery Obstruction
Upper Extremity
Vascular Diseases
Antihypertensive Agents