106 Vertebral-Artery Dissection Following a Judo Session: A Case Report By A. Lannuzezl, T. Moulin 1, D. Amsallem2 , ]. Galmiche1 and L. Rumbach1 lServiee de Neurologie et 2Serviee de Pediatrie, CHU Jean Minjoz, Besanc;on, Franee A few days after a judo session, an 11-yearold boy presented with an isehemie stroke with dizziness, aphasia and ataxia. CCT sean revealed a left thalamie infaret. Angiography showed a fibromuseular dysplasia (FMD) of the left vertebral artery probably eomplieated by disseetion. Subsequent evolution was favorable. This observation points out the faet that the assoeiation of a eervieal pain with neurologieal signs of vertebrobasilar stroke, espeeially oeeurring after a eervieal trauma or rotatory motion, should alert to the possibility of vertebral-artery disseetion. The diagnosis is mainly based on angiographie eriteria. Aeeurate diagnosis has implieations for prognosis and probably for aeute medieal treatment. eervieal eolumn. Immediately he eomplained of a latero-eervieal pain. During the night, he suffered from diffuse headaehes and vomiting. Similar episodes reeurred the following days. He also beeame progressively lethargie, developed a dysphasia and his walking beeame unstable. On lune 16, 1992 he had left ptosis and diplopia during one day. He suffered from a reduetion of speeeh flueney with many perseverations as weIl as axial ataxia and a left eervieal stiffness. An EEG revealed slow aetivity over the left temporo-oeeipital region. A CCT sean demonstrated a low-density lesion in the left thalamus (Fig. 1). Key words Disseetion - Extraeranial vertebral artery Thalamie infaret - Child - Fibromuseular dysplasia Introduction Vertebral-artery disseetion is the separation of the layers of the arterial wall by eireulating blood. Sinee the 1980's vertebral artery disseetion in young adults is wellknown, oeeurring most often after eervieal trauma whereas it is uneommon in ehildren. This report deseribes an 11-year-old boy with a thalamie isehemia seeondary to a left vertebral-artery disseetion. Two etiologie faetors were diseovered: a minor eervieal trauma during a judo session and an FMD of the third segment of the vertebral artery. Case report Fig. 1 Left thalamic infarct involving the tuberothalamic territory (arrowJ, On admission on lune 18, his neurologieal status was the same as two days before. Aseleeted 4-axis angiography performed by transfemoral eatheterization, demonstrated a "string of beads" lesion on the V3 segment of the left vertebral artery (Fig. 2). No abnormalities were noted in the other vessels. An MRI eonfirmed the left thalamie infaret, in the tubero-thalamie territory. An 11-year-old boy was admitted to our hospital in lune, 1992. His personal and family medieal history revealed no abnormalities until lune 2, 1992. On this day, during a judo session, he was vietim of a rotating motion of the Antieoagulation with heparin was performed for 15 days and an oral antieoagulation was given for 3 months. With this treatment, bed rest, and eervieal support with a soft eollar, the symptoms disappeared and the boy was diseharged after two weeks, without eomplaints. Reeeived Getober 11, 1993; aeeepted January 28, 1994 Three months later, the examination was normal. The seleetive angiography performed was unehanged. The oral antieoagulation was stopped and followed by Aspirin. Neuropediatrics 25 (1994) 106-108 © Hippokrates Verlag Stuttgart Downloaded by: Universite Laval. Copyrighted material. Abstract Neuropediatrics 25 (1994) Vertebral-Artery Dissection in Childhood 107 Discussion Children's vertebral-artery dissection is uncommon. The average age for vertebral dissection is about 40 years with a female preponderance. Dnly a few childhood cases have been published. For most patients, the symptoms and signs have a progressive or stuttering onset, varying from hours to days, but they were stabilized within 1 or 2 weeks (3). Transient ischemic attacks are rarely the only manifestation of vertebro-basilar ischemia. Strokes reveal the extracranial vertebral disseetion in 87 % of the cases. The most frequent patterns observed were lateral-medullary infarction (30 % of the cases) or cerebellar infarction (23 % of the cases) (4). Thalamic infarcts are rarely described probably because of the lack of sensitivity of CCT scan in the detection of small thalamic infaretions. Thalamic infarction was suggested in our case by lethargia and speech disturbance explained by the dysfunction of anterior thalamic nucleus (1). The thalamic infarction in this case is probably due to an embolism, arising from the site of dissection up to the tubero-thalamic arteries. In our case it is difficult to ignore the temporal association between the minor cervical trauma with neck torsion and the subsequent symptoms. The clinical course began with a latero-cervical pain occurring just after a necktorsion during a judo session, followed a few days later by symptoms of brain ischemia. The traumatic circumstances of vertebral-artery disseetion are extremely variable. It is commonly a minor, non-penetrating trauma of the neck in connection with ordinary daily activities like ceiling painting, yoga, gymnastics. It can also occur after chiropractic manipulations. The vertebral artery is susceptible to mechanical injury because of its relationship to the neighbouring bony structures and ligaments, especially in the third atlanto-axial segment (V3). Anatomical studies have demonstrated that a flexion or extension of less than 45° along with a tilt of 30° is needed to produce a decrease in vertebral artery-flow (7). The diagnosis of vertebral artery dissection is classically based on angiographic criteria. However, angiographie diagnosis of vertebral artery disseetion cannot always be made with complete confidence (3). In fact, the most common angiographic aspects are stenosis or thrombosis. Pseudoaneurysm is only observed in about 10 % of the cases occurring in V3 or V4 (2) and the typical feature of double- Fig. 2 Angiography: Type 1 fibromuscular dysplasia (lIstring of beads" lesion) (arrow) with arterial dilatations (arrowhead) and strictures on the V3 segment of the lett vertebral artery. lumen is exceptional. At least 85 % of the stenosis resolve or substantially improve within 2 or 3 months (2, 3, 4). Regression of thrombosis of pseudoaneurysm is less frequent. In our case the arteriography alone does not allow to make the diagnosis of arterial dissection with certainty. This can be related to the fact that the arteriography was realized 16 days after the presumed time of the dissection. This delay is sufficient for the disappearance of angiographic signs. However, the typical clinical course, the findings on CCT scan and the presence of FMD lesions predisposing to arterial dissection, allows to make with quasi certainty the diagnosis of traumatic vertebral artery dissection. FMD is a segmental non-atheromatous stenotic disease involving small- to medium-sized arteries. The disease is uncommon, its frequency is estimated to 1 % of cerebral angiograms. First described in the renal artery, it is now known to affect other vessels including cervicocerebral ones. The internal carotid artery is involved most frequently, followed by the vertebral artery. The involvement of the vertebral artery is mostly unilateral (70 % of the cases) and associated with internal carotid artery lesions (85 %). The distribution of the lesions shows a marked predominance of the involvement of the V3 segment (5). Pathologically, the lesion reflects a disorganized fibrosis of the intima, media, or occasionally, adventitia. The involved portions show alternating thick and thin areas of the vessel wall. The narrowed arterial segments show degeneration of elastic tissue, disruption and loss of the muscular coat, as weH as an increase in fibrous tissue. The dilatations are due to atrophy of the vessel wall. Downloaded by: Universite Laval. Copyrighted material. In 1978, Zimmerman et al reported the case of a 7-year-old boy who presented a vertebral-artery dissection occurring after a chiropractic manipulation (10). Perez-Higueras et al in 1988 reported the case of a 9-year-old boy with cerebellar infarct produced by Type 1 fibromuscular dysplasia of the vertebral artery complicated by a spontaneous disseetion (5). The obvious and inaugural symptom of vertebral-artery disseetion in about 80 % of the cases (2, 4) is a pain located in the posterior neck or in the occiput and sometimes accompanied, like in our case, by diffuse headaches and cervical stiffness. 108 N europediatrics 25 (1994) Cerebral ischemia is the regular consequence of the lesion but the cause of ischemia is often unsettled. The FMD is the principal arteriopathy predisposing to arterial dissection. Arterial dissections are observed in 15 % of the cases of FMD, respectively in the series of vertebral arteIY dissections the percentage of FMD can reach 23 % (4). In these cases the dissection is classically medial, involving the V3 segment in 70 % of the cases. Vertebral-arteIY dissections have also been described in association with other arteriopathies such as the Ehlers-Danlos syndrome Type 4 (7) and the Marfan syndrome (9). Dur patient was treated with anticoagulants. This might, at first, appear contraindicated in a disorder with bleeding into arterial wall. However, when the dissection is extracranial, most of the authors agree to prescribe a treatment with heparin for two weeks followed by oral anticoagulation for three months. In the same way, when dissection occurs during unusual activities or minor trauma, it may be advisable to avoid such potential precipitants in the future. References Bogousslavsky, J., F. Regli, A. Ushe: Thalamic infarcts: clinical syndroms, etiology, and prognosis. Neurology 38 (1988) 837-848 2 Crepin-Leblond, T., T. Moulin, J. L. Chopard, F. Cattin, A. Lannuzel, J. F. Bonneville: Dissection of the vertebral artery: A study of 23 patients. Cerebrovasc. Dis. 2 (1992) 230 3 Hart, R. G.: Vertebral artery dissection. Neurology 38 (1988) 987-989 4 Mas, J. L., M. G. Bouser, D. Hasboun, D. Laplane: Extracranial vertebral artery dissections: a review of 13 cases. Stroke 18 (1987) 1037-1047 5 Perez-Higueras, A., F. Alvarez-Ruis, A. Martinez-Bermejo, R. Frutos, O. Villar, E. Diez-Tejedor: Cerebellar infaretion from fibromuscular dysplasia and dissecting aneurysm of vertebral artery. Stroke 19 (1988) 521-524 6 Schievink, W 1., M. Limburg, J. W E. Gorthuys, P. Fleury, M. Pope: Cerebellar disease in Ehlers-Danlos syndrome type IV: Stroke 21 (1990) 626-632 7 Toole, J. F., S. F. Tucker: Influence of the head position upon cerebral circulation: studies on blood flow in cadavers. Arch. Neurol. 2 (1960) 616-623 8 Vega-Molina, J. Chiras, B. Poirier, J. Bories: Dysplasie fibromusculaire de l'artere vertebrale (aspect angiographique). Apropos de 85 cas. J. Neuroradiol. 12 (1985) 123-134 9 Youl, B. D., A. Coutellier, B. Dubois, J. M. Leger, M. G. Bousser: Three cases of spontaneous extracranial vertebral artery dissection. Eur. Neurol. 25 (1990) 388-395 10 Zimmerman, A. W, A. J. Kumar, N. Gadoth, F. J. Hodges: Traumatic vertebrobasilar occlusive disease in childhood. Neurol. 28 (1978) 185188 1 Prof L. Rumbach Service de Neurologie CHU Jean Minjoz Boulevard Fleming F-25030 Besanc;on Cedex France Downloaded by: Universite Laval. Copyrighted material. The most common radiographic finding is the so-called "string of beads" (Type 1) created by the formation of achain of arterial dilatations which are separated from one another by localized strictures. A second pattern, which is less common, is unifocal and characterized by tubular stenosis (Type 2). A third angiographic type of FMD has been called atypical: one wall of the affected segment is spared, and on the opposite wall an elongation, ovoid diverticulum, sharply circumscribed at either end by a noncircumferential narrowing, resembling a localized FMD constriction (8). A. Lannuzel et al