Idiopathic midaortic syndrome with malignant hypertension in 3-year-old boy.
10.3345/kjp.2016.59.11.S84
- Author:
Kyung Jin AHN
1
;
Ja Kyoung YOON
;
Gi Beom KIM
;
Bo Sang KWON
;
Eun Jung BAE
;
Chung Il NOH
Author Information
1. Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea. ped9526@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Aortic coarctation;
Renovascular hypertension;
Renal artery obstruction
- MeSH:
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
- From:Korean Journal of Pediatrics
2016;59(Suppl 1):S84-S87
- CountryRepublic of Korea
- Language:English
-
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.
