Journal of Neuro-Ophthalmology, March 1983, Volume 3, Issue 1

Complete bilateral internal carotid artery occlusion in a young man.

Partial or complete occlusion of the internal carotid artery is a familiar consequence of severe atherosclerosis seen in the elderly. Complete obstruction of both internal carotids is rare, particularly in the young or middle-aged. The rapid onset of bilateral internal carotid occlusion would be expected to produce devastating neurological sequelae and probably not be compatible with survival. We present a case of a young man with complete obstruction of both internal carotid arteries whose presenting symptoms were those of a visual field cut. The history suggests that the carotid occlusion occurred as a result of blunt trauma. The patient had no known predisposition to vascular abnormalities (no history of hypertension, hyperlipidemia, signs of systematic arteriosclerosis or vasculitis, and an unremarkable family history for vascular abnormalities). Computerized tomography revealed an infarct in his right parietal lobe. Angiography demonstrated complete occlusion of both internal carotid arteries and the right posterior communicating artery and failed to disclose the development of extensive collatorals, adding further evidence to the acuteness of the occlusion. The patient was followed by noninvasive studies and in the subsequent year showed marked neurological and ophthalmological improvement.

]. Clin. Neuro-ophthalmol. 3: 63-66, 1983. Complete Bilateral Internal Carotid Artery Occlusion in a Young Man ALFREDO A. SADUN, MD., PhD. ]. SEBAG, MD. DON C. BIENFANG, MD. Abstract Partial or complete occlusion of the internal carotid artery is a familiar consequence of severe atheroscle­rosis seen in the elderly. Complete obstruction of both internal carotids is rare, particularly in the young or middle-aged. The rapid onset of bilateral internal ca­rotid occlusion would be expected to produce devas­tating neurological sequelae and probably not be com­patible with survival. We present a case of a young man with complete obstruction of both internal ca­rotid arteries whose presenting symptoms were those of a visual field cut. The history suggests that the carotid occlusion occurred as a result of blunt trauma. The patient had no known predisposition to vascular abnormalities (no history of hypertension, hyperlipid­emia, signs of systematic arteriosclerosis or vasculitis, and an unremarkable family history for vascular ab­normalities). Computerized tomography revealed an infarct in his right parietal lobe. Angiography demon­strated complete occlusion of both internal carotid ar­teries and the right posterior communicating artery and failed to disclose the development of extensive collatorals, adding further evidence to the acuteness of the occlusion. The patient was followed by noninva­sive studies and in the subsequent year showed marked neurological and ophthalmological improve­ment. Case Report R.B., a 39-year-old, right-handed white man, presented to Massachusetts Eye and Ear Infirmary with the chief complaint of blurred vision in the left eye of 4 days duration. While driving his cab he noted difficulty judging distances. He also com­plained of dull right frontal headaches of 5 days duration exacerbated by alcohol consumption. He denied any other neurological, visual, or constitu­tional symptoms. The family history was negative for diabetes, hypertension, or stroke. Past medical history was remarkable for a motor vehicle accident 4 months previously with blunt trauma to the left lower face and left shoulder that left him with left-hand clumsiness and paresthe- From the Department of Ophthalmology, Harvard Medical School, Massachusetts Eye and Ear Infirmary and Brigham and Women's Hospital, Boston, Massachusetts. March 1983 sias, felt by a neurologist to be due to damage of the left brachial plexus. On physical examination he was an alert, coop­erative, well-developed, well-nourished white man in no apparent distress. BP was 142/100 left arm and 138/100 right arm. Visual acuity was 20/25 in both eyes, though he seemed to ignore letters to the left. Color vision was intact in both eyes. Extraocular movements including smooth pursuit and voluntary saccades and pupil responses were normal in both eyes. Optokinetic nystagmus test­ing demonstrated a slight difficulty in making sac­cades to the left. Slit lamp exam, tonometry, and fundus exams were all normal. Visual field testing by Goldmann perimetry revealed incongruous homonymous left field defects most pronounced in the left inferior quadrants (Figs. 1 A and 1 B). Neurological exam was significant for inabilities to reproduce spatial figures and left arm weakness and hypoesthesia. A right parietal lobe lesion was suspected and subsequently confirmed by computerized tomog­raphy (CT). As seen in Figure 1 B, there was an area of reduced enhancement in the right parietal lobe consistent with encephalomalacia. Skull x-rays, Chest x-ray, CBC with differential, lipid profile, and ESR were all normal. The patient was admitted to the Massachusetts General Hospital with suspicions of a parietal lobe astrocytoma and underwent cerebral angiography. Injections into the right and left common carotid arteries demonstrated complete occlusion of both internal carotid arteries just beyond their bifurca­tion sites (Figs. 2A and 2B). Posterior circulation injections demonstrated occlusion of the PI seg­ment of the right posterior cerebral artery, with no filling of the right posterior communicating artery (Fig. 2C). The left posterior communicating artery filled and supplied the left middle and anterior cerebral arteries. Doppler ultrasonography studies of the neck, 6 and 12 months after the onset of symptoms, confirmed the bilateral internal carotid occlusion without evidence of recanalization. Dig­ital subtraction films (Fig. 2 D) 1 year later also showed persistent complete internal carotid artery occlusion. Examination of the anterior segment by 63 Carotid Artery Occlusion in a Young Man Figure 1A. Visual fields obtained with a Goldman perimeter 2 days after presentation. Note the incongruous left homonymous hemianopsia with field loss predominantly inferiorly. Figure 1 B. Axial nonenhanced computer tomography (eT) scans at different levels demonstrating area of encephalomalacia in the right parietal lobe near the occipital border. slit lamp exam and of the fundus by indirect exam as well as fluorescein angiography failed to disclose any ocular abnormalities. In summary, an ostensibly healthy, relatively young man presented with uniocular blurred vision and was found to have visual field defects referr­able to a parietal lobe lesion. Computerized to­mography demonstrated encephalomalacia of the right parietal lobe. Angiographic evidence of mark­edly advanced cerebral arterial stenosis led to the conclusion that the parietal lobe lesion was due to old cerebral infarct. The widespread nature of the disease was felt to be inoperable; the patient was placed on aspirin, advised to change vocations, and has been followed with noninvasive studies of his cerebral blood flow. Discussion One of the first clinical-pathological studies of a case shown at autopsy to have complete occlusion of one internal carotid artery was described by Yirchow (1859). I The ophthalmic and central reti­nal arteries were patent, yet the patient had lost all vision in the eye ipsilateral to the thrombosed carotid artery. In 1893, Elshnig~ noted frequent findings of com­plete internal carotid artery occlusion at necropsy in patients without any visual symptoms. He per­formed injection studies on cadavers and demon­strated the profuse collateral supply of the oph­thalmic artery. Elshnig concluded that an occlusion of the internal carotid artery would have to be sudden to produce ocular symptoms. In 1905, Chiari l noted that mural thrombi or atheromatous 64 plaques in the internal carotid arteries of the el­derly could be a source of emboli which produced lesions more peripherally. In 1951, C. M. Fisher l described 200 cases of middle cerebral artery cerebral symptoms which were shown at necropsy to be due to the occlusion of the internal carotid artery. He concluded that internal carotid artery stenosis was often unrecog­nized, unsuspected, and, therefore, far more fre­quent than previously suggested. The clinical picture of carotid artery disease was addressed by Ramsy Hunt (1914), 4 who mentioned intermittent attacks and Denny-Brown (1951),:' who described transient ischemic attacks as a symptom of carotid artery or circle of Willis dis­ease. The symptomatology of such transient is­chemic attacks consisted of contralateral cerebral or ipsilateral ocular (amourosis fuga x) dysfunc­tions. The most common cerebral symptoms were transient hemiparesis or limb weakness, fleeting somatosensory disturbances on one side of the body, and mental confusion. Transient homony­mous hemianopsia was uncommon in cases of carotid system disease. I Complete occlusion of the internal carotid artery may be neurologically indistinguishable from mid­dle cerebral or anterior cerebral thrombosis. The middle cerebral picture is characterized by hemi­plegia, ~emianaesthesia, and hemianopsia.1i Hol­lenhorst' noted visual field defects in 45 of 235 cases of carotid artery occlusions. The homony­mous hemianopsia was moderately incongruous and always seen with hemiplegia. It is interesting to note that in the present report, the patient did present with incongruous homony- Journal of Clinical Neuro-ophthalmology Sadun, Sebag, Bienfang Figure 2A. Right cdrotid ,mgiogram. The right common carotid was filled revealing complete blockage of the right internal cdrotid drtery (drrow) near the site of bifurcation. Note the vertebral artery filling by reflux (double arrow). Figure 2B. Left carotid angiogram. The left common carotid was injected revealing complete blockage of the left internal carotid artery near the site of bifurcation (arrow). Figure 2C. Left vertebral angiogram, lateral view. The left vertebral artery was injected revealing filling the basildr drtery (drrow) and filling of the intracranial internal carotid artery circulation via the posterior communicating drteries with subsequent op.Kification of the middle and anterior cerebral drteries (double arrows). Figure 2D. Digital subtraction angiogram following venous injection of contrast mate­rial, 1 yedr after presentation. Note continued complete occlusion of right (arrow) and left (double arrows) internal carotid arteries. Compare with Figs. 2A .1I1d 2 B. mous hemianopsia with a partial left hemiparesis and hemianesthesia, thus mimicking a middle cer­ebral artery infarct. Angiography demonstrated that the right middle cerebral artery filled sequentially by way of the posterior circulation (vertebral, basilar), the left circle of Willis, and then the anterior communicat­ing artery, Such a circuitous route resulted in is­chemia of the right parietal lobe as documented in the CT scan. It would be unlikely that the internal occlusions developed because of atherosclerotic changes in March 1983 such a young man without medical or family his­tory, We suggest as an etiology for the bilateral carotid occlusions slowly developing dissecting aneurysms in the internal carotid vessel walls with retrograde thrombosis developing later.'" Hughes and Brownell" describe two cases of blunt trauma which produced a dissecting aneu­rysm of the internal carotid artery. In each case, the patient developed neurological symptoms and died within 48 hours from distal emboli. The fact that our patient remained alive and relatively intact is striking and suggests that either the dissection 65 Carotid Artery Occlusion in a Young Man occurred slowly or that a thrombus did not im­mediately develop after the blunt trauma 11J . II Fisher et alL' describe a "string sign" which can be seen on angiography of an internal carotid artery occluded by spontaneous dissection. They saw this angiographic pattern in about half of their cases which were ultimately diagnosed in surgery or pathology as dissecting aneurysms. They noted, however, that late after the dissection, the angio­graphic appearance is often that of a complete occlusion. Our patient's angiograms did not show a "string sign," suggesting complete occlusion of the lumen by extension of the aneurysm or retro­grade thrombosis. There were no ocular symptoms or signs; this is probably due to the rich collateral supply to the ophthalmic artery. However, ophthalmic testing revealed cerebral deficits produced by the abnor­mal circulatory pattern. It is evident that the oph­thalmologist or neuro-ophthalmologist often is in position to suggest the diagnosis of internal carotid artery stenosis. This case points out the sensitivity of visual field testing, the power of angiography for definitive description of the cerebral hemody­namics, and also of the value of noninvasive studies such as ultrasound-doppler and digital subtraction, for the long-term follow-up of a patient with vas­cular abnormalities. References 1. Walsh, F.B., and Hoyt, W.F.: Clinical Neuro-Oph­thalmology, Vol. 2 (3rd ed.). Williams & Wilkins, Baltimore, 1969, pp. 1802-1819. 2. Elschnig. A: Uber den Einfluss des Verschlusses der Arteria Ophthalmica und der Carotis auf des Schor­gan. Graefe Arch. Ophthalmol. 39: 151-177, 1893. 3. Fisher. eM.: Occlusion of the internal carotid artery. A.M.A. Arch. Neural. Psychiatry 65: 346-377, 1951. 4. Hunt, J.R.: The role of the car~tid arteries. in the 66 causation of vascular lesions of the brain, with re­marks on certain special features of the symptoma­tology. Am.]. Med. Sci. 147: 704-713, 1914. 5. Denny-Brown, D.: Treatment of recurrent cerebro­vascular symptoms and the question of "vasospasm." Med. Clin. North Am. 35: 1457-1474, 1951. 6. Ford, F.R.: Diseases of the Nervous System in In­fancy, Childhood and Adolescence (3rd ed.), Charles C Thomas, Springfield III., 1952, pp. 176-177. 7. Hollenhorst, R. W.: Ophthalmic aspects of stroke: Symposium on neuro-ophthalmology. [n Transac­tions of the New Orleans Academy of Ophthalmol­ogy. e V. Mosby, Saint Louis, Missouri, 1976, Chap­ter 19, pp. 255-267. 8. Ojemann, P.G., Fisher, eM., and Rich, J.e: Spon­taneous dissecting aneurysms of the internal carotid artery. Strake 3: 434, 1972. 9. Hughes, J.T., and Brownell, B.: Traumatic thrombo­sis of the internal carotid artery in the neck. ]. Neural. Neurosurg. Psychiatry 31: 307-314, 1968. 10. Meyer, J.5., Guiraud, B., and Bauer, R.: Clinical and patho-physiological considerations of athero­sclerotic and thrombotic disease of the carotid arter­ies. [n Handbook of Clinical Neurology, Vol. 2, Part 1, P.J. Vinken and G. W. Bruyn, Eds. Elsevier. New York, 1972, pp. 327-365. 11. Gold, AP., and Carter, S.: Acute hemiplegia of infancy and childhood. Pediatr. Clin North Am. 23: 413-433, 1976. 12. Fisher, eM., Ojemann, KG., and Roberson, C.H. Spontaneous dissection of cervico-cerebral arteries. Can. ]. Neurol. Sci. 5: 9-19, 1978. Acknowledgment The authors thank Dr. Alfred Weber for his help in the interpretation and descri ption of the cerebral angiog­raphy. Write for reprints to: Alfredo A Sadun, M.D., PhD., 243 Charles Street. Boston, Masssachusetts 02114. fournal of Clinical Neuro-ophthalmology