A 68-year-old Filipino man

[yananshs]

Еще одна задачка пришла в рассылке (они меня принимают за кого-то другого):

BACKGROUND
A 68-year-old Filipino man presents to the emergency department with swelling and redness of his right eye for 4 days. He denies any pain, visual blurring, or double vision in the affected eye. Both the swelling and the redness have progressively worsened since he first noticed them. He has no history of trauma or weakness and no strange sensation on his face or in his extremities. He has not had vertigo, headache, or difficulty walking.

On physical examination, the patient has normal and stable vital signs, though headache may be present. Chemosis of his right eye with proptosis is observed. Visual acuity is 20/60 in the affected eye and 20/20 in the unaffected eye. No evidence of corneal abrasion or ulceration is noted, and the patient does not have any visual field deficits. The fundus appears congested with no signs of pallor. His extraocular movements are intact with no pain on movement. Gross findings on neurologic examination are within normal limits, with a specific note of intact cranial-nerve function. No bruit is noted around the affected eye.

Looking at the photo of the patient's eye (see Image), what is your diagnosis?
Hint
The patient has a history of diabetes mellitus and hypertension

[EVP]

Синдром кавернозного синуса Бонне (Bonnet)?

[Dr. W.N.]

Я ответ знаю, но не скажу, т.к. догадался не сам, видел эту задачку раньше.

[Dr. W.N.]

Яна, сказать?

[Mikhail]

Честно сказать, я в затруднении. Вроде и на кавернозный синус не очень похоже, экзофтальм то не пульсирующий... intact cranial-nerve function опять же...

Я сдаюсь.

Кстати, Яна, а в конце рассылки есть адрес чтоб на нее подписаться?

[Dr. W.N.]

Отвечу за Яну. Адрес есть, и вы его знаете ))

[yananshs]

Picture:

[yananshs]

Answer
Carotid-cavernous fistula (CCF), dural arteriovenous (AV) malformation (AVM): First reported in the 1930s and further elucidated in the 1970s, the CCF is a hole in the cavernous segment of the internal or external carotid artery that has established a direct AV fistula to the venous spaces of the cavernous sinus. The abnormal communication results in high-pressure arterial blood entering the low-pressure venous cavernous sinus, which interferes with the normal patterns of venous drainage and which compromises blood flow into the cavernous sinus and the orbit. The restriction of normal egress of blood from the cavernous sinuses may result in redirection of venous flow into cortical veins or other venous structures with development of complications such as venous hypertension of the deep venous system and venous infarcts. Different classification systems for these fistulas are based on the anatomy, etiology, or velocity of blood flow.

Of importance, direct CCFs are distinguished from dural AVMs of the cavernous sinus (ie, direct fistulous connections that most often simply travel through the cavernous sinus), which were previously called indirect fistulas. Both have similar clinical symptoms consisting of exophthalmos, ophthalmoplegia, chemosis, increased intraocular pressure, decreased visual acuity, ptosis, and possible bruit. They may even superficially appear similar on standard cerebral angiograms. Both conditions had been frequently classified as 2 subtypes of a common entity; however, they are probably 2 separate conditions, as the neurointerventional and neurosurgical literature now emphasizes.

Direct CCFs usually occur suddenly, resulting from a tear in the arterial wall connecting the internal carotid artery to the cavernous sinus. The cause is either trauma or rupture of a cavernous carotid aneurysm. The fistulas typically have a high flow rate, and symptoms develop rapidly. Compared with direct CCFs, dural AVMs of the cavernous sinus have a more gradual onset over days, weeks, or months; a less fulminant course; and generally lower flow. Even in direct CCFs, the pattern and severity of symptoms depend on the venous anatomy of the compartments of the cavernous sinuses into which they connect.

CT angiography or magnetic resonance (MR) angiography often reveals dilatation of veins and improper connections between the arterial system and the cavernous sinus. Carotid angiography is the definitive diagnostic modality and enables clear diagnosis and confirmation of the type of CCF (direct vs dural and location and direction of abnormal blood flow). This imaging also provides a therapeutic capability (see Image 1). Complete ophthalmologic workup is indicated and should include gonioscopy and direct and indirect funduscopy, as well as an assessment of visual acuity, pupillary function, and intraocular pressure.

Treatment of some CCFs and most dural AVMs can be medical. (For information on categorizing CCFs, please refer to the eMedicine references below.) As many as 20-50% of low flow lesions may close spontaneously or with carotid self-compression for 20-30 seconds 4 times per day. Patients should be instructed to compress the carotid artery on the side of the lesion by using their contralateral hand. In the acute setting of vision loss and/or paralysis of the cranial nerves, glucocorticosteroids (eg, dexamethasone) may be used as the results of definitive diagnostic studies are awaited. Indications for urgent treatment include increased proptosis and decreased visual acuity, increasing intraocular pressure, intracranial venous hypertension, elevated intracranial pressure, and rupture into the subarachnoid space. Lubrication to protect against keratopathy and pharmacologic management of glaucoma are indicated.

Endovascular surgery with balloon occlusion or coil embolization is definitive management for a CCF or dural AVM that does not close on its own or with carotid compression or that is not amenable to such closing. This patient likely had a dural AVM that was eventually treated with coil embolization (see Image 2). After the fistulous tract is delineated, the objective is to obliterate the fistulous connection and to restore normal arterial and venous flow. The prognosis for a given patient depends on the type of fistula, the progression of findings, and the duration of symptoms. If left untreated, the fistula can result in chronic glaucoma and visual loss.

For more information on CCFs, see the eMedicine articles Carotid-Cavernous Fistula (within the Radiology specialty), Fistula, Carotid Cavernous (within the Ophthalmology specialty), and Caroticocavernous Fistula (within the Internal Medicine specialty).

References
Koenigsberg RA. Carotid-cavernous fistula. eMedicine Journal [serial online]. 2005. Available at: [Ссылки доступны только зарегистрированным пользователям ].
Nosko MG. Caroticocavernous fistula. eMedicine Journal [serial online]. 2005. Available at: [Ссылки доступны только зарегистрированным пользователям ].
Purvin V. Cerebrovascular disease and the visual system. Ophthalmol Clin North Am 2004;17(3): 329-55.
Yanoff M. Ophthalmology. St Louis, MO: Mosby; 2004: 1403-4.