Indian J Pediatr (March 2014) 81(3):285–287 DOI 10.1007/s12098-013-1202-9 CLINICAL BRIEF Hypertensive Intracranial Bleed due to Mid Aortic Syndrome Varadarajan Poovazhagi & Leema Pauline & N. Balakrishnan Received: 22 November 2012 / Accepted: 31 July 2013 / Published online: 26 September 2013 # Dr. K C Chaudhuri Foundation 2013 Abstract The authors describe an 11-y-old child with intracranial bleed due to malignant hypertension. Child presented with hypertension, right hemiparesis, feeble femoral pulses and lower limb blood pressure less than the upper limb. CT angiogram revealed narrowing of the abdominal aorta with thinned out left renal artery and hypoplasia of the left kidney. A diagnosis of Mid aortic syndrome was arrived at. CT brain revealed left ganglio capsular bleed. Child was treated with antihypertensives and steriods in view of suspected Takayasu arteritis. Child recovered with minimal hemiparesis and is being followed up. Keywords Intracranial bleed . Mid aortic syndrome . Hypertension Introduction Hemorrhagic stroke due to intracranial bleed is a pediatric emergency which needs intensive care treatment. Case Report An 11-y-old child presented with acute onset weakness of right hemiparesis with facial asymmetry. There was no history V. Poovazhagi (*) Department of Pediatric Intensive Care, Institute of Child Health and Hospital for Children, Egmore, Chennai, Tamil Nadu 600008, India e-mail: poomuthu@yahoo.com L. Pauline Department of Pediatric Neurology, Institute of Child Health and Hospital for Children, Egmore, Chennai, Tamil Nadu, India N. Balakrishnan Department of Pediatric Intensive Care, Institute of Child Health and Hospital for Children, Egmore, Chennai, Tamil Nadu, India of fever, headache or vomiting. Child was lethargic on admission. The upper limb pulses were felt normal but the femoral and dorsalis pedis were feeble on both sides. Blood pressure recorded was 160/120 mmHg (Rt upper limb), 164/ 122 mmHg (Lt upper limb), 120/90 mmHg (Rt lower limb) and 120/100 mmHg (Lt lower limb). He had a supraumblical bruit. Clinical examination revealed right hemiparesis with right seventh nerve paralysis. Fundus was normal. There was no café au lait spot. His chest radiograph was normal. ECG revealed features of left ventricular hypertrophy (LVH). Echocardiogram revealed concentric LVH and diminished flow in the abdominal aorta. Ultra sonogram of the abdomen revealed non visualisation of the left kidney with a right kidney measuring 9.4 cm, increased cortical echoes with grade 1 renal parenchymal disease. CT brain (Fig. 1) and MRI brain revealed left ganglio capsular hemorrhage with surrounding edema suggestive of subacute haemorrhage. Urea, creatinine, blood glucose, prothrombin time and activated thromboplastin time were normal. Lipid profile ASO, CRP and ESR, ANAwere normal. A renovascular cause of hypertension was arrived at. Child was treated with methyldopa and nifedepine. As the blood pressure was controlled, child developed diminishing femoral pulse which was not palpable at the end of 10 d of hospital stay and his blood pressure was not recordable by then in both the lower limbs. The blood pressure was stabilised at 110/80 mmHg over a period of 7 d CT angiogram (Fig. 2) revealed significantly narrowed abdominal aorta up to the bifurcation, thickening of the walls of aorta with thinned out left renal artery, with hypoplasia of the left kidney with multiple extensive collaterals. Doppler revealed aortic occlusion with bilateral monophonic flow in both lower limb arteries with reduced velocities suggestive of a proximal occlusion. The diagnosis of Mid Aortic syndrome (MAS) was arrived at. He has partially recovered from the hemi paresis. He was started on oral steroids. His repeat CT brain showed porencephalic cyst in the region of the bleed. His blood pressure is controlled with medications. 286 Fig. 1 CT brain showing the left gangliocapsular bleed Discussion Hemorrhagic stroke due to hypertension is a rare event in children. Though common in adults, children rarely develop hemorrhage due to hypertension. Stroke in children comprises of acute ischemic stroke (55 %) and hemorrhagic stroke (45 %). The average incidence of hemorrhagic stroke is 0.7– 5.1/100,000 including subarachnoid bleed [1]. Hemorrhagic stroke is due to arterio venous malformations, aneurysms, Fig. 2 CT angiograph showing the coarctation of aorta. ABD AO Abdominal aorta; SPL A Splenic artery; RT RA Right renal artery; SMA Superior mesentric artery; RT CIA Right common iliac artery; LT CIA Left common iliac artery Indian J Pediatr (March 2014) 81(3):285–287 hemophilia, brain tumors and cerebral venous sinus thrombosis. Hypertension is a risk factor for intracranial parenchymal bleed in children [2], but occurs less frequently in children than in adults [3, 4]. Renal parenchymal and renovascular hypertension are the common causes of hypertensive bleed. Aorto arteritis is the commonest in the latter. Mid aortic syndrome (MAS) is due to segmental narrowing of the abdominal aorta with narrowing of the visceral and renal arteries. This is commonly acquired due to neurofibroma, retroperitoneal fibrosis, fibrous dysplasia, mucopolysacharidosis and Takayasu arteritis or rarely, due to a congenital anomaly of the descending aorta. The disease has been associated with Turner, Williams and Alagille syndromes. MAS is a rare cause of secondary arterial hypertension and stroke in children. It is difficult to differentiate between congenital and acquired causes. Coarctation of aorta in the atypical sites is uncommon. Abdominal coarctation accounts for 0.5–2 % of all coarctation. In the index child authors were able to rule out the acquired causes like neurofibroma, retroperitoneal fibrosis and mucopolysaccharidosis but the possibility of congenital abdominal coarctation or Takayasu arteritis is difficult to delineate. Granulamatous vasculitis causing intimal proliferation and occlusion of aorta and its branches in encountered in Takayasu arteritis. Associated involvement of renal arteries is commonly reported and less common is the involvement of the celiac and superior mesenteric arteries. Based on the American College of Rheumatology, the criteria of Takayasu arteritis would be: age of onset<40 y, claudication of extremities, blood pressure difference of >10 mmHg between the two arms, bruit over the aorta and arteriogram abnormalities. The narrowing is not due to atherosclerosis, fibrous dysplasia or similar causes. More than 3 of the above is diagnostic. The index child satisfies the criteria for Takayasu arteritis. In MAS, the first manifestation is due to hypertension. Later manifestations are claudication, congestive cardiac failure and renal involvement, systemic hypertension and end organ damage [5]. Mid aortic syndrome can be associated with cerebral aneurysms in 10 % [6]. Reported mortality is 8 % [7]. MAS can be treated by balloon angioplasty with risk for re-stenosis. MAS, in the absence of renal involvement or target organ damage has been treated with medications in literature [8]. Takayasu arteritis is a chronic condition which needs to be followed up for relapse and treatment with oral steroids. Conflict of Interest None. Role of Funding Source None. Indian J Pediatr (March 2014) 81(3):285–287 References 1. Mallick AA, O’Callaghan FJ. The epidemiology of childhood stroke. Eur J Paediatr Neurol. 2010;14:197–205. 2. Pavlou E, Gkampeta A. Stroke in childhood. J Pediatr Sci. 2011;3:e75. 3. Jordan LC, Kleinman JT, Hillis AE. 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