CASE REPORT A Serious Pain in the Neck YEW CHONG YAP, MBBS, MRCSEd(Ophth), SHYAM KODATI, MBBS, FRCS, FRCOphth, ALAN KOSMIN, MB ChB, MSc, MD, FRCOphth, Dr. NISAR DAMANI, MBBS, FRCS(Lond), FFR(RCSI)(Ireland), DAVID J. WERRING, MBBS, MRCP(UK), PhD ABSTRACT We describe a case of internal carotid artery dissection, an important cause of ischemic cerebrovascular disease. Presentations include a headache, which is usually unilateral to the side of carotid dissection, sharp, nonthrobbing in nature with radiation from the neck. The clinical presentations, investigations, and treatment of the disease are discussed. I N T R O DU C T I O N Internal carotid artery dissection is an important cause of ischemic cerebrovascular disease in young patients. It has an annual incidence of 2.6–2.9 per 100,000. The incidence may be higher as the condition remains unrecognized with only two-thirds of the patients developing cerebral or retinal ischemic symptoms. Presentations include having a headache which is usually unilateral to the side of carotid dissection, sharp, nonthrobbing in nature with radiation from the neck (1). This article described the clinical presentations, investigations, and treatment of the disease. CASE REPORT REPRINTS Yew Chong Yap, MD, Department of Ophthalmology, Watford General Hospital, Vicarage Road, Watford, Herts WD18 0HB, UK. E-mail: eyapyc@gmail.com. Drs. Chong, Kodati, Kosmin, Damani, and Werring are with the Department of Ophthalmology, Watford General Hospital, Watford, UK. Dr. Werring is also with the National Hospital for Neurology and Neurosurgery and Stroke Research Group, Institute of Neurology, Queen Square, London WC1N 3BG, UK. The authors have stated that they do not have a significant financial interest or other relationship with any product manufacturer or provider of services discussed in this article. The authors do not discuss the use of off-label products, which includes unlabeled, unapproved, or investigative products or devices. The authors report a case of traumatic internal carotid artery dissection and review relevant aspects of presentation, investigation, and treatment. Submitted for publication: 3/2/07. Accepted: 6/29/07. Annals of Ophthalmology, vol. 39, no. 4, Winter 2007 Ó Copyright 2007 by ASCO All rights of any nature whatsoever reserved. 1530-4086/07/39:334–336/$30.00. ISSN 1558-9951 (Online) ANN OPHTHALMOL. 2007;39 (4) ..............................................334 A 29-year-old woman was struck on the side of her head by a surfboard. Her neck was twisted to the side and backward and she immediately developed pain in the right side of her neck radiating into the right temporoparietal region and forehead. Six to eight hours later she developed sudden left-sided weakness affecting her face, arm, and leg, with slurred speech. She had two generalized seizures and was admitted to the hospital. Her past medical history included having anorexia and bulimia for 5 years. She was taking only depot medroxyprogesterone acetate (DMPA, Depo ProveraÒ, Pfizer, New York, NY) for contraception. She reported drinking alcohol occasionally and but not smoking. On examination, her visual acuities were VR 6/9 and VL 6/18–2. She had a left homonymous hemianopia (Fig. 1). There was no Horner’s syndrome or relative afferent pupillary defect. Anterior and posterior segments were normal. She also had a left Figure 1—Humphrey visual fields showing the total deviation map. Note the left homonymous hemianopia (abnormal responses to the stimulus are shown as black or shaded squares). Figure 2—(A) Axial computed tomographic (CT) angiogram image through the cervical carotid arteries above the carotid bifurcation, showing loss of the right internal carotid artery lumen (block arrow) suggesting dissection. (B) Three-dimensional helical CT reconstruction image showing rapid tapering of the lumen of the right internal carotid artery termination in occlusion (the ‘‘rat tail’’ sign: thin arrow). upper motor neuron VII palsy, a slurring dysarthria, severe left upper limb weakness with only a flicker of movement distally and mild pyramidal weakness of the left lower limb. She had left-sided hyperreflexia with an extensor plantar. Axial computed tomographic (CT) angiography of this patient showed abrupt narrowing of the internal carotid artery lumen at C3 level (the so-called ‘‘rat tail’’ sign), which is characteristic of dissection. The distal cervical and intracranial segments of right internal carotid were completely occluded (Fig. 2). An unenhanced CT scan of the brain showed infarction in the right middle cerebral artery territory affecting the right lentiform nucleus and fronto-parietal cortex (Fig. 3). DISCUSSION Internal carotid artery (ICA) dissections can have neurological manifestations as a result of local effects Figure 3—Axial computed axial tomographic (CT) showing low attenuation, compatible with infarction in the right fronto-parietal region in the carotid arterial territory. of the dissection via pressure or interruption of blood supply to neural structures. These local effects may include a painful postganglionic Horner’s syndrome, lower cranial nerve palsies (particularly XII), and neck tenderness or swelling. Symptoms due to thromboembolism include any focal disturbance in the territory of the carotid artery including transient monocular blindness or hemiparesis with or without other cortical signs including hemianopia or language disturbance (2). A study on 146 patients with internal carotid artery dissections revealed that 62% (91/146) of the patients had ophthalmologic symptoms and signs with 44% (65/146) with painful Horner syndrome, 28% (41/146) with transient monocular visual loss and four patients with ischemic optic neuropathy (3). ANN OPHTHALMOL. 2007;39 (4) ..............................................335 Another study showed that 12% (23 of 190 patients) have cranial nerve palsies with cranial nerve (CN) XII being most frequently involved (10 of 23 patients) and CN V being the second most frequently involved (7 of 23 patients). Headache was present in all but four patients (83%) and oculosympathetic palsy noted in 13 patients (56%). Reasons for cranial nerves palsies include compression or stretching of the cranial nerves by the expanded artery and compromised nutrient arterial supply to the nerves involved (4). Traumatic causes of internal carotid artery dissection includes include head turning, hairdressing (‘‘barber chair syndrome’’), sports injuries, child abuse, inappropriate chiropractic manipulation, and blunt trauma. Nontraumatic causes include smoking, arteriosclerosis, familial (probably related to collagen gene mutations), Marfan’s syndrome, Ehler’s Danlos syndrome, infection, e.g., syphilis, oral contraceptives, and fibromuscular dysplasia. Dissection occurs when blood penetrates a primary intimal tear into the arterial wall. Intramural hematoma surrounding the tear narrows the true arterial lumen causing stenosis or occlusion. Emboli from the thrombus can travel to the brain, causing cerebral infarction (5). Enlargement of the artery can also compress the surrounding structures, causing other manifestations. The internal carotid artery dissection usually begins 2–3 cm distal to the carotid bifurcation, which is different from the typical arteriosclerotic changes, which is at the carotid bifurcation. It usually terminates before the petrous part of the temporal bone whereby the rigid bony canal limits distal progression. The gold standard for diagnosis of internal carotid artery dissection is carotid angiography, which shows segmental narrowing or occlusion of the extracranial internal carotid artery. Other noninvasive investigations include magnetic resonance, magnetic resonance angiography, and Doppler ultrasound (6). Onset of stroke after internal carotid artery dissection can occur within a few minutes to one month after initial symptoms. Preventive treatment should be initiated as soon as possible and this included ANN OPHTHALMOL. 2007;39 (4) ..............................................336 anticoagulation, antiplatelet, and even surgical intervention such as carotid artery ligation. Although randomized trial data are lacking, anticoagulants should theoretically help prevent thromboembolic stroke, and most stroke physicians recommend anticoagulation with heparin and then warfarin for 3–6 months, particularly if there is evidence of significant arterial stenosis or thromboembolism into the carotid territory. There are few data to support this treatment, and a randomized trial is currently planned. Cerebral blood flow should be optimized by maintaining systemic blood pressure, or altering the patient’s posture as appropriate. The available natural history studies suggest that the prognosis for extracranial carotid artery dissection is good, with a low annual recurrence risk. Intracranial dissection is much less common, but carries a worse prognosis. Anticoagulation may be more hazardous in intracranial dissection, which carries a risk of subarachnoid hemorrhage (7). Treating doctors should have a high index of suspicion for ICA dissection in patients with neurological deficits, headache, and Horner’s syndrome. Instituting early treatment can prevent cerebrovascular stroke and significant morbidity. REFERENCES 1. Lee VH, Brown RD Jr, Mandrekar JN, et al. Incidence and outcome of cervical artery dissection: a population-based study. Neurology 2006;67:1809–1812. 2. Kerty E. The ophthalmology of internal carotid artery dissection. Acta Ophthalmol Scand 1999;77:418–421. 3. Biousse V, Touboul PJ, D’Anglejan-Chatillon J, et al. Ophthalmologic manifestations of internal carotid artery dissection. Am J Ophthalmol 1998;126:565–577. 4. Mokri B, Silbert PL, Schievink WI, et al. Cranial nerve palsy in spontaneous dissection of the extracranial internal carotid artery. Neurology 1996;46:356–359. 5. Cucchiara BL, Kasner SE. Carotid dissection causing occipital lobe infarction. Neurology 2005;65:1408. 6. Flis CM, Jager HR, Sidhu PS. 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