Mid aortic dysplastic syndrome - A rare case presentation of renovascular hypertension

Clinical History

A 17-year-old male patient was admitted with breathlessness and heaviness in chest for 2-3 months. His past history was unremarkable. Blood pressure was raised around 200/120 mm Hg in both the upper limbs with feeble femoral pulse.

Imaging Findings

Ultrasonography showed a localised narrowing of a supra renal segment of the abdominal aorta with atherosclerotic plaque with few foci of calcification involving aortic wall. Flow velocity in narrowed segment was very high exceeding 180cm/s with aliasing pattern on Doppler study. Lower abdominal aorta and arteries of both lower limbs showed loss of normal triphasic wave form pattern with decreased flow velocity.
Computed Tomography Aortography (CTA) of the aorta and its branches showed a diffuse narrowing of lower descending thoracic aorta, upper abdominal aorta and coeliac trunk due to calcified plaque involving vessel wall. Focal dilatation of the arch of aorta and upper descending thoracic aorta as well as post stenotic dilatation was noted involving abdominal aorta distal to the narrowed segment.
Multiple arterial collaterals noted involving anterior abdominal wall with dilated feeding artery as superior and inferior epigastric artery.

Discussion

Vascular aetiology of hypertension in young patients can be crucial in management of patient as to differentiate non-treatable idiopathic cause from treatable pathology. Mid-aortic syndrome (MAs) also known as coarctation of the abdominal aorta is a rare disease with only 200-250 reported cases with a male to female ratio of 3:1. It is characterised by constriction of distal thoracic and/or upper abdominal aorta and its branches without extension into iliac arteries. We report a case of a young boy admitted with dyspnoea and chest heaviness who was found to have this rare cause of secondary hypertension.
The most common anatomic form in idiopathic middle aortic syndrome is inter renal, followed by supra-renal, infra-renal and diffuse. Stenosis of the renal arteries is common, with less common involvement of the coeliac and superior mesenteric arteries, and infrequent involvement of the inferior mesenteric arteries. [1]
It usually presents as hypertension in young age group which is typically unresponsive to medical therapy. Rarely, it presents as lower limb claudication or abdominal angina due to proximal stenosis (hypertension) and distal stenosis causing hypotension. The life expectancy of patients with untreated MAS is 30-40 years. The main reason of death is cardiovascular complications of progressive hypertension including cerebrovascular accidents, cardiomegaly, left heart failure and coronary artery disease. [2]
Initial management with oral antihypertensives to control hypertension is followed by definite surgical therapy. The severity of hypertension is the primary indication for intervention and the factor determining procedural timing. Endovascular therapy may provide a sound minimally invasive treatment in MAS caused by discrete aortic stenosis that do not encompass the mesenteric and renal arteries.
Open surgery is the primary treatment of tubular aortic narrowing associated with renovascular hypertension and visceral artery stenosis.
Young patient with breathlessness was admitted in pulmonary medicine was clinically having increased blood pressure. Subsequently, USG examination and followed by CT aortography diagnosed atherosclerotic wall thickening involving aorta and coeliac trunk. Blood pressure measurement in all four limbs in patient with thoracic complaint can give clues about most appropriate management approach.

Differential Diagnosis List

Final Diagnosis

Mid aortic dysplastic syndrome with coeliac trunk involvement

URL:	https://www.eurorad.org/case/15485
DOI:	10.1594/EURORAD/CASE.15485
ISSN:	1563-4086