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Coarctation of the Thoracic Associated with Cerebral Arterial Occlusive Disease

3 Harold A. Baltaxe," 2 Solomon Bloch,' and Paul K. Mooring

Since coarctation of the aorta was first described by extremely poor and no bl ood pressure could be obtained in them. Johann Friederich Meckel in 1760 [1], a vast literature has Blood pressure in his arm s was 122 / 58 mm Hg. appeared on its associated conditions and treatment. This Laboratory findings were noncontributory. Chest rad iography anomaly of the descending thoracic aorta is usually located revealed left ventricular prominence and a notch-shaped left con­ distal to the left and is commonly found in tour of the suggesti ve of coarctation. El ectro­ cardiography revealed left ventricular and left atrial hypertrophy. coexistence with intra- and extracardiac pathology. Ventric­ catheterization and angiocard iography revealed a 90% coarc­ ular and/ or atrial septal defects [2], as well as discrete tation of the aorta distal to the left subclavi an artery with poststen­ subvalvular , represent the accompanying intracar­ otic dilatation of th e descending th oracic aorta. The vessels to the diac conditions [3, 4]. The presence of a bicuspid aortic head appeared anatomically norm al in their takeoff from the arch. valve was first noted by Edwards [5] and is found in 85% of Selective cerebral arteriography was perform ed. Injecti on of the patients afflicted by coarctation of the aorta [6]. Supraval­ right common carotid artery demonstrated a norm al extracranial vular stenosis is only rarely an associated anomaly [7], course; intracranially, multiple discrete stenoses were noted mainly whereas the presence of patent is rather in the horizontal part of the ri ght middle cerebral artery and its common [8]. Dilatation of the ascending aorta and aneu­ angular branch (figs. 1 A- 1 C). The appearance of these stenoses rysms of the sinus of Valsalva combined with coarctation was consistent with th at of fibromuscular dysplasia or some other form of vasculopath y. A left carotid arteriogram with cross compres­ have also been reported [9]. Whenever the patient does not sion of the right common caroti d artery revealed a norm al ri ght suffer from [10], anomalies involving or­ anteri or cerebral artery and norm al left carotid branches. gans other than the heart and the thoracic aorta are rarely Computed tomography (CT) of th e head demonstrated an area of seen in coarctation. However, aneurysms of the circle of increased uptake of contrast materi al in the ri ght hemisphere that Willis were noted by Hodes et al. [11] and are the cause of was consistent with an ischemi c infarct. A brain scan supported this cerebral hemorrhage, which is a major complication in the diagnosis. natural history of coarctation. Coarctation of the aorta in A mu scle bi opsy of the gastrocnemius muscle revealed no ab­ association with cerebral arterial occlusive disease, to our norm alities. The coarctation of the aorta was repaired leaving a 25 knowledge, has not been reported previously. We report mm Hg residual gradient. Hi stologic examination of aortic tissue was not made. two patients with such a combination. The question to be The patient underwent reassessment of his cerebral status 5 considered is whether this represents a fortuitous associa­ years later. Cerebral angiography revealed an occlusion of the ri ght tion or whether both entities have a common etiology. middle cerebral artery at its ori gin . Its branches filled distall y in a retrograde fashion mainly from the ri ght posteri or cerebral artery (figs. 1 E and 1 F). CT showed a well defined area of encephaloma­ Case Reports lacia on the right side in the middle cerebral artery distribution consistent with an old infarct (fig. 10). Case 1

A 4-year-old boy was admitted 2 weeks after ac ute onset of a left Case 2 hemiparesis. His father found him lying on the bathroom floor with what seemed to be choreoathetotic movements on the right, while A 2-year-old boy underwent cardiac catheteri zati on and angiog­ the left side of his body was limp. He was alert and cooperative with raphy, revealing coarctation of the aorta distal to th e left subclavian left hemiparesis and left facial paralysis. A grade III / VI harsh systolic artery. At age 5 years, he sustain ed a cerebrovascul ar accident murmur heard at the upper left sternal border was transmitted to resulting in left hemiparesis. He underwent surgical correction of the back and to both cflrotid arteri es. The in the legs were his coarctation 1 month later. The procedure had to be repeated at

This article appears in the September/ October 1982 issue of AJNR and the December 1982 issue of AJR.

I Department of Radiology, University of Nebraska Medical Center, Omaha, NB 68 105. 2Present address: Department of Radiology, University of California, Davi s, UCD Professional Bldg., 4301 X St. , Sacramento, CA 95817 . Address reprint requests to H. A. Baltaxe. 3Department of Pediatric Cardiology, University of Nebraska Medical Center, Omaha, NB 68105. AJNR 3:577-580, September/ October 1982 0 195- 6 108 / 82/ 0305-0577 $00.00 © American Roentg en Ray Society 578 BALTA XE ET AL. AJNR:3, September/ October 1982

B c

D E F Fig. 1.-Case 1. A, Right lateral common carotid arteriogram. Multiple angiogram. Almost complete obstruction at origin of the middle cerebral discrete linea r filling defects with mild poststenotic dilatations in angular artery (straight arrow). Distally there is earl y retrograde filling of distal middle branch of middle cerebral art ery. Anterior cerebral artery not visible. B, Local cerebral artery branch via anterior and posterior cerebral arteries (curved mag nified view. Angular branch with multiple defects (arrows). C, AP right arrows ). F, AP right carotid angiogram . Almost complete block at right middle carotid angiogram. Multiple discrete linear fi ll in g defects in middle cerebral cerebral artery origin with opacificati on onl y of ascending frontoparietal artery (arrows ). D, 5 years later. CT scan with intravenous contrast enhance­ branch. ment demonstrates old right frontal in farct. E, Right lateral common carotid

Fig. 2.-Case 2. A, Left lateral com­ mon carotid angiogram. Almost com­ plete occlusion at supraclinoid part of internal carotid artery (arrowhead). Me­ dial posterior choroidal artery (straight arrow) and perforating arteries of pos­ terior cerebral artery show marked dila­ tation (curved arrow). B, Right vertebral angiogram. Prominent perforating ves­ sels within basal ganglia consistent with Moya Moya syndrome.

A B AJNR:3, September I Octaber 1982 COARCTATION OF THE AORTA 579

age 9 years due to restenosis. Aortic tissue was not examined have been reported in neurofibromatosis histologically. In the interim he apparently sustained one or two [15]. The renal artery stenosis in neurofibromatosis is more cerebrovascular accidents. caused by the mesenchymal abnormali ty. Cerebrovascular At age 9 years the residual gradient across the site of his occlusive disease has also been described in neurofibro­ previously repaired coarctation was 30 mm Hg. Blood pressure in matosis [16]. Levisohn et al. [17] reported two such cases both arms was normal. A left common carotid angiogram revealed with brain infarction diagnosed by CT, caused by occlusive an almost complete occlusion at the level of the supraclinoid part of the internal carotid artery with dilatation of the perforating arterial intracranial disease with resulting extensive coll aterali za­ supply to the basal ganglia (fig. 2A). A right vertebral angiogram tion. Angiographically, the vessels presented with segmental revealed marked dilatation of the thalamoperforating arteries to the constrictions and areas of ectasia, similar to the cerebral basal ganglia producing an appearance consistent with a Moya arteries of our patients. Neurofibromatosis, however, is a Moya syndrome (fig. 2B). heredofamilial disorder with maldevelopment of the neural At age 14 years, he still had a left hemiparesis and appeared ectodermal tissue. Nothing suggested that our patients were somewhat mentally diminished. There appeared to be no progres­ afflicted by such a condition. However, it is possible that the sion of the neurologic deficit. abnormality that affects th e fibroelastic tissue in coarctati on may sometimes also involve th e intracerebral vessels. Coarctation of the aorta has been reported in association Discussion with aneurysms of the cerebral vessels [11]. The mechani sm Whenever several pathologic conditions coexist, it is im­ for the formation of these aneu rysms has never been eluci­ portant to determine whether their association is fortuitous dated. Long-standing hypertension restri cted to the upper or whether they have a common etiology. Because coarc­ body as seen in coarctation may produce changes in the tation of the aorta is a congenital disease, an initial assump­ cerebral vessels that eventually may result in the formation tion would be that the intracranial lesions in our patients are of aneurysms. Could hypertension have produced occlusive due to an inherent rather than an acquired defect. Never­ vascular disease in our patients? Even if long-standing theless, the possibility that the cerebral arterial occlusions hypertension would have been present, the cerebrovascular were secondary to the hypertension caused by coarctation changes noted in our patients could probably not have taken must be considered also. place without an underl ying disposition. The exact etiology of coarctation of the aorta is unknown. These two cases cannot be dismissed without suggesting In 1841, Craigie (cited in [2]) first proposed the " skodiac the possibility of a vasculitis. Ferri s and Levine [18] classi­ theory, " which stipulates that the aortic narrowing is sec­ fied cerebral arteritides according to their etiology. Bacte­ ondary to the closure of the ductus. Coarctation of the aorta, rial, viral, and collagen diseases must be considered when however, is often associated with a patent ductus, and , faced with intracranial vascular changes in children. Colla­ therefore, this theory seems to be invalid. In 1 965, Brom gen diseases were excluded in our two cases. Harwood­ [12] suggested that the tissue encircling the aorta at the site Nash et al. [19] reviewed th e cerebral arteri al diseases in of the coarctation was similar to that of ductal tissue, and 40 children. None of them were associated with coarctation he also tried to relate coarctation of the aorta to the pres­ of the aorta and most seemed to be the sequelae of an ence of the ductus. inflammatory process resulting from a nasopharyngeal in­ Classically, coarctation is not a concentric narrowing, but fection. No such infections were reported in ou r two pa­ primarily a posterolateral narrowing. Therefore, Edwards et ti ents. al. [13] believed that this eccentric thickening was related Lastly, Slagsvold et al. [20] reported th e case of 32-year­ to a developmental change of the intima. More recently old woman in whom the carotid angiogram was consistent Kennedy et al. [14] studied the intima in coarctation of the with fibromuscular dysplasia of the left anteri or and middle aorta by using light and scanning electron microscopy. They cerebral arteries. Until then, it was commonly beli eved that found that the intima immediately proximal to the stenosis fibromuscular dysplasia did not occur in cerebral vessels. ran in fine longitudinal folds radiating into the orifice. The Although this concept has been disproven, fibromuscular intima on the distal side was grossly irregular with deep dysplasia is not known to be associated with coarctation of longitudinal and transverse folds producing a convoluted the aorta. appearance resembling the surface of the brain. The intima Analysis of our two cases does not permit us to suggest immediately distal to the orifice was covered with a lami­ a single eti ology for these findings. Coexistence of coarc­ nated layer distinct from the elastica, which often gave tation of the aorta and cerebral arterial occlu sive disease positive staining reactions for fibrin . Intimal thickness could be fortuitous, however, our purpose was to speculate seemed to increase with age. These authors concluded that that such an association may not occur entirely at random. their findings were consistent with the view that the constric­ REFERENCES tion had a fibroelastic component that is congenital and a 1. Jarchos S. Coarctation of the aorta (Meckel 1750; Pari s 1 791). fibrous part that is acquired and progressive. They believed Am J Cardio/1 961 ;7 : 844-852 that the progressive element is caused by deposition of 2. Duffie ER , Wilson JR. Coarctation of th e aorta. In: Moss AJ, fibrin on an abnormal intimal surface in a region of turbulent Adams FH , eds. Heart disease in infants, children and adoles­ flow. This congenital abnormality may be similar to the cents. 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