Mid-aortic dysplastic syndrome as a rare cause of hypertension in young

Abstract

Introduction: Mid-aortic syndrome (MAS), coarctation of abdominal aorta is a rare disease with only 200 reported cases. It is characterized by constriction of distal thoracic and/or abdominal aorta and its branches, therefore is also known as abdominal aortic coarctation. The MAS is characterized radiologically by severe narrowing of abdominal aorta and its branches and most of these patients usually die due to progressive severe hypertension before the age of 35-40 if left untreated. Case Report: A 13-year-old boy was admitted with persistent headache and vomiting for one month and repeated generalized tonic clonic seizures for two days. His past history was unremarkable. The highlight of the clinical examination blood pressure was 240/150 mmHg in both the upper limbs, and all peripheral pulses were palpable. Blood pressure was similar in upper and lower limbs and a systolic bruit heard over the epigastrium. Ultrasonography showed a localized narrowing of a suprarenal segment of the abdominal aorta with an abrupt focal dilatation of the abdominal aorta approx 1.7cm below origin of superior mesenteric artery. Magnetic resonance angiography of the aorta and its branches showed a distinct fusiform dilatation of abdominal aorta just below the origin of the superior mesenteric artery. Focal narrowing of abdominal aorta was noted just proximal to the dilatation. Conclusion: We diagnosed a case of mid-aortic coarctation with post stenotic dilatation associated with left renal artery stenosis leading to secondary hypertension in a teenage boy presenting with reversible posterior leukoencephalopathy and seizures. The boy was referred for vascular surgery but the patient refused any operative procedure.