No Abstract African Health Sciences Vol. 8 (4) 2008: pp. 256-25

Byanyima, RK

Kiguli-Malwadde, E

Opio, James

PubMed

Kiguli-Malwadde, E.

Byanyima, R.K.

University of Toronto Research Repository

Coarctation of aorta presenting as acute haemorrhagic stroke in a
14-year old. A case report.

TSpace Repository

Opio, J

AJOL - African Journals Online

African Health Sciences Vol 8 No 4 December 2008256CASE REPORTSCoarctation of aorta presenting as acute haemorrhagic stroke in a 14-year old. A case report.James Opio, E Kiguli-Malwadde, RK ByanyimaDepartment of Radiology, Faculty of Medicine Makerere University.African Health Sciences 2008; 8(4): 256-258Correspondence:E. Kiguli-MalwaddeMakerere University, Faculty of Medicine,Department of Radiology and Mulago HospitalP.O.Box 7051, Kampala, UgandaEmail  malwadde@med.mak.ac.ugIntroductionCoarctation of the aorta is a relatively common defect.It may occur as an isolated defect or in association with avariety of other lesions, most commonly bicuspid aorticvalve. The diagnosis of coarctation of the aorta may bemissed unless a high index of suspicion is maintained,and diagnosis often is delayed until the patient developscongestive heart failure, which is frequent in infants, orhypertension, which is frequent in older children. Thisadolescent presented for the very first time with acutehaemorrhagic stroke due to severe hypertensionsecondary to Coarctation of the aorta. A chest x-rayshowed features of left ventricular enlargement andcoarctation of aorta.Case presentationA 14 year-old male without any ailments since birthdeveloped sudden onset of loss of consciousnessassociated with right hemiplegia and facial palsy.Significant clinical findings were: bradycardia (56bpm),absent femoral, popliteal and dorsalis pedis pulses,severe hypertension (225/120mmHg) and aorticstenosis murmur.A PA chest x-ray showed gross cardiomegallywith a rounded and laterally displaced apex. Theascending aorta was dilated and there was a notch in theproximal descending aorta demonstrating a ‘figure-of-3’ sign. No features of pulmonary congestion wereobserved. Subtle rib notching was noted on the inferiorborders of the right 5th and 7th ribs (Fig.1). An impressionof left ventricular hypertrophy secondary to severehypertension, complicating coarctation of the aorta wasmade.Cardiac echo revealed left ventricular hypertrophy,aortic stenosis and a small degree of aortic incompetence.Non-enhanced brain CT scans done at 10mm slicethickness showed a well-defined homogeneouslyhyperdense mass lesion of 77 HU (compared to 37 HUof corresponding normal brain) in the left fronto-temporal peri-ventricular region. It measured54x21x50mm and was surrounded by a hypodense rimdue to oedema. The anterior horn of the left ventriclewas deformed and a midline shift of 4mm was noted.The ambiens cistern was preserved (Fig.2). A diagnosisof acute intra-cerebral haemorrhage was made.Fig.1:  Chest x-ray showing cardiomegally, lateral apicaldisplacement, a“figure-of-3”sign (left arrow) and earlyrib notching (upward arrow).African Health Sciences Vol 8 No 4 December 2008 257DiscussionCoarctation of the aorta is a relatively common defectaccounting for 5-8% of all congenital heart defects. Itmay occur as an isolated defect or in association with avariety of other lesions, most commonly bicuspid aorticvalve (in 50% of cases) and ventricular septal defect (1, 2).The diagnosis of coarctation of the aorta may be missedunless an index of suspicion is maintained, and diagnosisoften is delayed until the patient develops congestiveheart failure, which is frequent in infants, orhypertension (2, 3).Coarctation of the aorta imposes significantafter-load on the left ventricle, which results in increasedwall stress and compensatory ventricular hypertrophy.The after-load may be imposed acutely, as occursfollowing closure of the ductus arteriosus in neonateswith severe coarctation. These infants may rapidly developcongestive cardiac failure and shock (1).Left ventricular after-load may also increasegradually, allowing children with less severe coarctationto develop arterial collateral vessels that bypass the aorticobstruction. These children may be asymptomatic untilhypertension is detected or another complication likelife-threatening intracranial haemorrhage develops (4).Chronic hypertension causes lipohyalinosis, fibrinoidnecrosis and development of Charcot-Bouchardaneurysms which rupture to cause intracranialhaemorrhage (5). No such case has been reported inUganda or East Africa. The incidence of coarctation ofthe aorta in East Africa is not known. This case is reportedso as to increase the index of suspicion for it and alsobecause the presentation was unusual in that thehypertension was never detected until the patientpresented with a stroke. The natural history of untreatedcoarctation is that of premature death from stroke andcoronary heart disease or sudden death. However thiscommonly occurs early on in childhood for patientsthat are not treated (6).This was a typical case of an asymptomatichypertensive patient who only presented with intra-cerebral haemorrhage.   Haemorrhagic strokes accountfor 10 -20% of strokes in general, with a one monthfatality of about 42%.  Hypertension is the leading causeof non traumatic intracerebral haemorrhage (7).Radiographs of patients with early onset ofcoarctation of the aorta may reveal cardiomegaly,pulmonary oedema, and other signs of congestive cardiacfailure. In patients with late onset of coarctation of theaorta, cardiomegaly, arch indentation in the area of thecoarctation, and rib notching may be seen (6,8).This patient presented with late onset featuresincluding cardiomegaly, aortic indentation and early ribnotching.Rib notching is the single most reliable sign. Itis unusual below 10 years and the older the person, themore likely to have rib notching. Although rib notchingwas observed in this patient it was not a prominentfeature probably because it is still early on account ofhis young age. Most patients with coarctation beyond 20years of age display this sign(6,9).The indentation in the area of the coarctationgives a ‘figure-of-3’ sign. This appearance is caused bythe tuck of the coarctation itself and may be accentuatedby dilated left subclavian artery or aortic knob, or post-stenotic aortic dilatation (8,9).Cardiomegaly is due to left ventricularhypertrophy, which is seen as rounding of the cardiacapex, and dilatation which manifests as infero-lateralapical displacement (3,4). This was a prominent feature inthis patientEchocardiography delineates intra-cardiacanatomy and allows assessment of associated significantintra-cardiac anomalies. The para-sternal long and shortaxis views enable evaluation of the aortic root, inter-ventricular septum and left ventricular wall. Thesuprasternal notch view allows evaluation of the aorticarch to assess the transverse aortic arch, isthmus, andseverity of coarctation. Doppler echocardiographymeasures the gradient at the area of coarctation andidentifies the pattern of diastolic runoff typically seen inpatients with severe obstruction (8,9). In this case theFig.2: Brain CT scan showing acute intra-cerebralhaematomaAfrican Health Sciences Vol 8 No 4 December 2008258cardiac echo was able to detect left ventricularhypertrophy and associated aortic stenosis.Hypertensive strokes commonly occur in basalganglia, thalamus, pons cerebellum and other brain stemsites.  Bleeding into brain parenchyma causes direct braininjury and in addition, surrounding brain is damaged bypressure from the haematoma (5).  Prognosis ofhaemorrhagic stroke depends on stroke severity,neurological score, extent of haemorrhage at imagingand greater patients age (7).  Epileptic seizures arecommoner following haemorrhagic as compared toischaemic stroke and they are commoner in lober ascompared to basal ganglia haemorrhages.  Few childrenmay recover with no physical impairment buthemiparesis of variable severity, cerebellar ataxia,tetraparesi, paraparesis and cognitive defects are someof impairments that can occur if patients survive.Rebleeding is also one of the feared events (7, 10).References1. Harlan JL, Doty DB, Brandt B 3rd: Coarctation of theaorta in infants. Journal of  Thoracic Cardiovascular Surgery1984 Dec; 88(6): 1012-92. Greenwood RD, Rosenthal A, Parisi L. Extracardiacabnormalities in infants with congenital heart disease.Paediatrics 1975; 55:485-492.3. Ing FF, Starc TJ, Griffiths SP, Gersony Wm. Early diagnosis ofcoarctation of the Aorta in children: a continuing dilemma.Paediatrics 1996; 98: 378-382.4. Campbell M. Natural history of coarctation of the aorta.British Heart Journal 1970; 32: 633-640.5. K A L Carroll, J Chataway. Understanding stroke:Pathophysiology, prevention, and investigations. StudentBritish Medical Journal 2006; 14:309-352.6. Mullen M J. Coarctation of the aorta in adults: do we needsurgeons? Heart 2003: 89: 3-57. Martin Dennis.  Advances in stroke prevention.Cerebrovascular Diseases 2003; 16(Suppl 1): pg 9-138. Kreel L. Congenital heart disease and associated conditions.Outline of Medical Imaging; 28: 196-1989. von Schulthess GK, Higashino SM, Higgins SS. Coarctationof the aorta: Imaging. Radiology 1986; 158:469-474.10. Ingrid Blom, Els, L L M De Schryver, L Jaap Kappelle, et alPrognosis of haemorrhagic stroke in childhood: a long-termfollow-up study. Developmental Medicine & ChildNeurology 2003; 45(4): 233-239

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Coarctation of aorta presenting as acute haemorrhagic stroke in a 14-year old. A case report.

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Coarctation of aorta presenting as acute haemorrhagic stroke in a 14-year old. A case report

Coarctation of aorta presenting as acute haemorrhagic stroke in a 14-year old. A case report

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