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Original Article
Carotid dissection: a common cause of Horners syndrome
Colin CK Chan MBBS(Hons), Mark Paine FRACP and Justin ODay FRACO
Department of Neuro-Ophthalmology, The Royal Victorian Eye and Ear Hospital, East Melbourne, Victoria, Australia
ABSTRACT
Purpose: To highlight that internal carotid artery dissection
is a common cause of Horners syndrome and that it is
important to diagnose dissection as anticoagulation can
prevent carotid thrombosis and embolism.
Methods: Five case reports are presented of patients with
Horners syndrome secondary to carotid dissection. One
patient had carotid dissection secondary to trauma, two
had spontaneous dissections and two had dissections in the
settings of other illness. A literature search was performed
on carotid dissection as a cause of Horners syndrome and
its diagnosis and management.
Results: The case reports and literature highlight that
dissection is under-recognized as a cause of Horners
syndrome and can be missed.The investigation of choice is
a magnetic resonance imaging and angiography scan of the
head and neck.The treatment advocated is anticoagulation
for 36 months.
Conclusion: Carotid dissection should be suspected in
patients with Horners syndrome, particularly if head or
neck pain is present.
Key words: anticoagulation, carotid dissection, headache,
Horners syndrome, magnetic resonance angiography,
magnetic resonance imaging.
INTRODUCTION
It is traditionally thought that internal carotid artery (ICA)
dissection is a serious but uncommon cause of Horners syndrome, and that dissection is usually flagged by a clear
history of neck trauma with resultant head and neck pain. It
is our experience that many dissections do not present this
CASE REPORTS
Case 1: Dissection secondary to trauma
A 47-year-old woman with hypertension presented with
pain and paraesthesiae in her right temporal and periorbital
region a few days after a fall down a flight of stairs. She fractured her left navicular bone but did not sustain any other
injuries. In particular, she did not recall having hit her head
or twisting her neck. The headache and numbness were
continuous for 12 h but then became intermittent. She then
noticed a right ptosis and miosis. She did not have any visual
disturbances but did complain of ringing in her right ear.
Investigations included a full blood count, ESR, thyroid
function tests, syphilis serology and a computed tomography (CT) brain scan, all of which were normal.
On examination, there was a moderate right ptosis with
a miotic right pupil, consistent with a right Horners syndrome (Fig. 1). Visual acuity was 6/6 in both eyes and the
visual fields were full. The remainder of the cranial nerve
examination was normal. Neurological examination revealed
absent brachioradialis and biceps jerks on the right;
however, there were no other neurological signs.
An urgent magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) head and neck confirmed a
dissection beginning just above the right internal carotid
artery origin and extending up into the skull base (Figs 2,3a).
She was admitted, heparinized and commenced on warfarin.
A repeat MRI/MRA performed 6 months later showed
Correspondence: Dr Colin Chan, Department of Neuro-Ophthalmology, The Royal Victorian Eye and Ear Hospital, 32 Gisborne Street, East Melbourne, Vic
3002, Australia. Email: chunkeng@yahoo.com
412
Chan et al.
recanalization of the right internal carotid artery. The warfarin was then ceased.
Figure 1.
Figure 3. (a) Crescent shaped hyperintense lesion representing internal carotid artery dissection on fat suppressed magnetic resonance
(MR) image (case 1). (b) Non-fat suppressed MR image showing similar hyperintensity of dissection/haematoma and high velocity carotid
flow (reproduced with permission from Dr N Trost, Consultant Radiologist, St. Vincents Hospital, Melbourne, Australia).
413
Beecham, Boronia, Victoria, Australia). He also complained
of altered sensation over the right supraorbital region,
extending back to the scalp and a recent onset right ptosis.
However, his vision was normal.
On examination, he had a right 23 mm ptosis and anisocoria of 12 mm. His visual acuity, fields and fundi were
normal. The rest of the cranial examination was also normal;
notably he did not have any evidence of trigeminal nerve
dysfunction. Both carotid pulses were present and no bruits
were heard. A MRI/MRA of the head and neck showed evidence of a right internal carotid dissection just below the
skull base, with displacement of the carotid artery and a ring
of hyperintense tissue representing haematoma surrounding
the artery posteriorly. He was commenced on warfarin. A
repeat MRI/MRA scan 3 months later showed recanalization
of the artery lumen and the anticoagulation was ceased.
Despite this, the Horners syndrome and facial pain has
persisted. Numerous membrane stabilizers and epileptic
drugs were tried with limited success.
DISCUSSION
Ophthalmologists are often the first specialists to see
patients with Horners syndrome. The aetiology of Horners
syndrome has traditionally been classified anatomically into
central, preganglionic and postganglionic lesions.1,2 Usually
postganglionic lesions can be differentiated from central and
preganglionic defects clinically. Current teaching is that
postganglionic Horners syndrome usually has a benign aetiology.35 However, much of this presumption comes from a
few prospective and retrospective studies performed before
the advent of magnetic resonance (MR). Thus these patients
did not have any screening tests for dissection unless they
had symptoms such as amaurosis fugax or neck pain. It has
been our experience that spontaneous carotid dissection is
more commonly a cause of Horners syndrome than previously recognized. This is also suggested by Miller and
Newman.3 Leira et al. further suggested that carotid dissection should be suspected in cases of transient anisocoria
also.6 There is only one prospective trial, to our knowledge,
that looks at how often Horners syndrome is caused by dissection. Digre et al. performed MRI/MRA of the head and
neck in 20 patients with postganglionic Horners syndrome.7
Three patients (15%) were found to have carotid dissection.
Interestingly, the study concluded that this was too low a
yield to make MRI/MRA useful in the management of postganglionic Horners syndrome.
As illustrated with our case reports, there are three basic
groups of patients that present with carotid dissection: (i)
patients with a clear history of preceding trauma, sometimes
as trivial as a coughing spasm (case 1); (ii) patients with no
history of trauma (cases 2 and 3) and otherwise well, the
spontaneous carotid dissection; and (iii) patients with an
intercurrent illness (cases 4 and 5).8,9 It is thus our experience that dissection may not present with the classic history
of preceding trauma, abrupt onset of neck and head pain
and Horners syndrome. However, having said this, the
414
Table 1. Symptoms and signs associated with carotid dissection
in order of decreasing frequency11,32,33
Cephalic pain/headache
Cerebral ischaemic symptoms (hemiparesis/paraesthesiae/
hemianopia)
Horners syndrome
Monocular visual loss
Photopsia (acute choroidal hypoperfusion)
Amaurosis fugax
Bruit
Dysgeusia (loss of taste)
Chan et al.
dysplasia, Marfans syndrome, cystic medial necrosis and
hypertension.1820 However, in the majority of cases no
predisposing factors are found. The mechanism of oculosympathetic palsy is thought to be enlargement of the
artery, associated with haematoma formation and localized
inflammatory response. This results in compression of the
superior cervical ganglion lying in the posterior wall of the
carotid sheath.9,19 The defect is thus postganglionic and
usually incomplete.6
It is now widely accepted that a combination of MRI and
MRA of the head and neck is the best method to diagnose
carotid dissection.9,21 Intra-arterial digital subtraction
angiography (DSA), previously considered the gold standard, is no longer used where MR is available. It is invasive,
more expensive and is less accurate than MR. Diagnosis of
dissection with DSA is by the presence of the classic string
sign which is only present in subintimal dissections where
haemorrhage into the subintimal space causes narrowing of
the vessel (Fig. 2). Some patients have subadventitial dissections that are less likely to produce stenosis and thus may be
missed on DSA.22,23 For the same reason, dissection can be
missed if the radiologist looks only at the reconstructed
images of the MRA as the reconstructed images provide
similar images to DSA. Instead it is the source images that
must be looked at as these will show high-intensity crescent
shaped haematoma in the wall of the artery (Fig. 3a).
Requests for a MRI/MRA should specifically ask for fatsuppressed T1 images. Without fat suppression, both
arterial flow and haematoma will look equally bright and a
dissection can be missed (Fig. 3b). The request should also
specify that both the head and neck images cover the entire
length of the internal carotid artery from bifurcation to skull
base (N Trost, 2001, pers. comm.).12,2430
Carotid Doppler has been proposed by a few studies as a
first line diagnostic test.8,17,31 The premise is that ultrasonography (US) may be more sensitive for detection of
early thrombus than MR. The main problem with US is that
it is unable to reliably detect dissection high in the neck,
near the skull base, where most dissections seem to occur.18
However, it may be a useful tool in monitoring recanalization of the artery and guiding anticoagulation therapy after
initial MR scans to diagnose the lesion.
Treatment of dissection should be with an antiplatelet
drug such as aspirin or anticoagulation with heparin or warfarin, but this is controversial and there is limited supportive
data.8,9,11,17 Admittedly, it would be difficult to justify a
prospective trial looking at this issue but more retrospective
data may clarify this.
In summary, carotid dissection is probably underrecognized as a cause for Horners syndrome. It should
probably be excluded by MRI/MRA in all patients with
painful postganglionic Horners syndrome and maybe
also patients without headache and neck pain because of
the potential complications. The treatment advocated is
36 months of anticoagulation. More studies are necessary
to clarify the management of Horners syndrome and
dissection.
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