An 86 year old woman with headache and limited eye movements
Digital Journal of Ophthalmology 2005
Volume 11, Number 10
April 12, 2005
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Damian Lake | St. George's Hospital
Kashif Qureshi | Kingston Hospital
Nabil Malik | Kingston Hospital


History
An 86 year old Asian female presented with a 12 month history of indolent right sided headache, and a sensation described as “difficulty moving my eyes”, but not diplopia. The headache was localized to the right frontal area, stopping at the midline. It was constant and described as burning in nature. She complained of no problem with jaw claudication, facial weakness, dysarthria, limb girdle weakness, diurnal variation or respiratory difficulty. She had a past medical history of hypertension and type 2 diabetes mellitus controlled with diet. There was no significant past ocular history.

Examination
On examination visual acuity was 6/12 corrected. She had bilateral ptosis, which was worse on the right side. Hertel exophthalmometry was 17 mm bilaterally, no orbital mass could be palpated, no orbital bruit present and there was no resistance to retropulsion. A right face turn and chin-up head posture was noticed, with a 25 prism diopter right esotropia and an 8 prism diopter right hypertropia. There were limitations of upgaze, abduction, downgaze and adduction bilaterally. On attempted down gaze, incyclotorsion was elicited bilaterally. Sensation was equal bilaterally to light touch and pain on the face and the corneal reflex was brisk and equal on both sides. Cranial nerve and general neurological examination was otherwise unremarkable. The pupils were 4mm in diameter in room light conditions, reacting equally to light and accommodation. There was no relative afferent pupillary defect, or change in pupil diameter on versions. Colour vision was measured with Isihara’s pseudoisochromatic plates; all plates were identified correctly bilaterally. Humphrey neurological visual field examination was unremarkable. The ocular exam was normal; specifically there was no evidence of optic atrophy or a pigmented retinopathy.

Bilateral cavernous carotid aneurysyms
 Video clip of eye movements demonstrating reduction in abduction, adduction, upgaze and downgaze in both eyes

Ancillary Testing

Axial T1 weighted MRI
 MRI demonstrating bilateral carotid aneursyms within the cavernous sinus

Treatment
This lady opted to not undergo any treatment and presently remains unchanged.

Differential Diagnosis
Thyroid eye disease
Myasthenia gravis
Chronic progressive external ophthalmolplegia
Neoplastic infiltration
Space occupying lesion within the cavernous sinus

Diagnosis and Discussion
Intracavernous carotid aneurysms comprise 2% of all intracranial cerebral aneurysms but bilateral cavernous carotid aneurysms are rare.
Intracavernous carotid aneurysms occur predominantly in women over the age of 50, and behave differently from other intracranial aneurysms as they rarely hemorrhage into the subarachnoid space because of their extradural location. If the aneurysm ruptures, carotico- cavernous fistula is the most likely outcome, producing symptoms of complete ophthalmoplegia, proptosis, conjunctival chemosis, orbital congestion, optic neuropathy, optic disc oedema, and retinal haemorrhages.
Intracavernous carotid aneurysms may slowly enlarge without rupture or producing symptoms. The commonest neuro-ophthalmic manifestation is diplopia, due to compression of one or more of the oculomotor nerves. The Abducens nerve is commonly affected as it lies within the cavernous sinus, adjacent and lateral to the carotid artery. The Oculomotor nerve lies superiorly within the dura mater that forms the lateral wall of the cavernous sinus. Movement of the Oculomotor nerve is restricted when compressed, as the petroclinoid and interclinoid ligaments tether the nerve, producing symptoms of superior, medial, inferior and inferior oblique paresis. Trochlear nerve paresis as an isolated finding is rare. When an Oculomotor palsy is already present, the function of superior oblique can be demonstrated clinically by eliciting incyclotorsion on attempted down gaze, in our case this appeared to be present bilaterally, and may be explained by the relative laxity in the lateral wall of the cavernous sinus at this point, sparing the Trochlear nerve.
Aneursymal growth may be so mild or slow growing that, patients present without diplopia but signs of oculomotor synkinesis such as pseudo-Graefe’s sign, miosis on attempted adduction and light near dissociation of the pupils.
Facial pain may also be a presenting complaint, as the ophthalmic and maxillary divisions of the Trigeminal nerve are located in the lateral wall of the cavernous sinus. Trigeminal sensory loss is rarely elicited on examination. The combination of pain in the distribution of the ophthalmic division of the Trigeminal nerve and a post ganglionic Horner’s syndrome (due to the relationship of the sympathetic plexus to the internal carotid artery) is referred to as Raeder’s syndrome. Rarely carotid cavernous aneurysms can compress the pituitary gland medial to the cavernous sinus producing symptoms of headache, amenorrhea, decreased libido, hypothyroidism, or diabetes insipidus.
The treatment goal is to occlude the aneurysm before rupture, and minimize medical and neurological complications. Due to the inaccessible position of the intracavernous carotid aneurysm, direct surgical clipping may be difficult, and associated with over 3% mortatility and 7% morbidity. Those patients over 70 years have an increased risk due to increased medical complications and more friable vessels. In these situations a GDC (Gugliemi Detachable Coil) may be safer. The GDC coil is placed into the neck of the aneurysm percutaneously through a micro catheter. The soft platinum coil is packed into the neck and sac of the aneurysm until the aneurysm is separated from the circulation, a small electric current then allows separation of the coil from the steel guide wire. The morbidity and mortality after GDC embolisation varies from 3 to 6%. The main complications are embolism and Aneursymal rupture. Aneurysm occlusion rates are related to size, position and the neck of the aneurysm. Those aneurysms less than 10 millimetres diameter have a 75% chance of complete occlusion but for those over 10 millimetres the success falls to 40%. Long-term recurrence rate is between 5 and 10 %. In our case the patient decided not to proceed with intervention due to her age and the fusiform character of the aneurysms.
Bilateral carotid cavernous aneurysms have previously been described, occasionally mimicking other conditions. Bilateral ophthalmoplegia caused by bilateral cavernous carotid aneurysms are rare but should be included in the differential diagnosis.

References
References

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