Carotid-cavernous fistulas (CCFs) are spontaneous or acquired abnormal connections between the carotid artery system and the cavernous sinus (CS). Barrow classified CCFs angiographically into four types: Type-A involve a direct shunt between the internal carotid artery (ICA) and the CS; the other three are dural shunts connecting the CS to the meningeal branches of the ICA in Type-B, the external carotid artery in Type-C, or both in Type-D. Direct fistulas involve high flow and are usually posttraumatic while indirect fistulas are low flow, developing spontaneously or with atherosclerosis, hypertension, collagen vascular disease, or intra- or post-partum. Nukui found only one case of direct type among 21 cases of spontaneous variety. In Peeters series, all patients of direct type had a history of trauma. Halbach reported 3.1% intracerebral hemorrhage among 127 patients of the direct type. Based on pathology studies, the incidence of saccular aneurysms is estimated at 9%. We report a rare case having spontaneous direct CCF with intracerebral hemorrhage associated with an intracranial aneurysm, secondary hypertension, and unilateral small contracted kidney.
A 20-year-old female presented to our emergency department with complaints of headache for the last 7 days and progressive swelling over the right eye (RE), along with redness and diminution of vision (DOV) for the last 5 days. An initial headache was acute in onset, throbbing, confined mainly to the right half of the head, and relieved on analgesics. After 2 days, she experienced a severe episode of headache, along with fever, orbital swelling, and DOV of RE. A local ophthalmologist prescribed topical and oral steroids with antibiotics, nonsteroidal anti-inflammatory drugs, and lubricants. Despite this, her condition worsened and she was referred to our hospital. There was no history of trauma, previous surgery, abnormal movements, or unconsciousness. She denied being a diabetic or hypertensive. She was oriented to time, place, and person but drowsy and irritable. Her blood pressure (BP) on presentation was 200/150 mmHg and pulse rate was 64/min. Electrocardiogram suggested a left ventricular hypertrophy. She had a visual acuity (VA) RE of count fingers at 1 m, and VA left eye (LE) was 20/25 on Snellen. Undilated retinoscopy was +1.50 DS RE and +0.50 DS LE. RE revealed swollen lids with discharge and crusting, along with axial proptosis of 11 mm, chemosis, and conjunctival congestion. She had a clear cornea with sluggishly reacting pupil and restricted ocular movements in all directions of gaze [Fig. 1]. Ophthalmoscopy revealed edematous and hyperemic discs, somewhat more in the RE, with pulsatile veins. Bruit was audible over the RE. LE was otherwise unremarkable except for an abduction deficit [Fig. 2]. Magnetic resonance imaging (MRI) of the brain showed a bleed in the right frontal lobe of size 24 mm × 32 mm × 20 mm, along with extensive edema surrounding the lesion involving parieto-frontal lobe, associated with midline shift of 0.45 cm and effacement of ipsilateral lateral ventricle [Fig. 3]. MRI orbit displayed marked prominent superior ophthalmic vein with flow reversal, with edema of the extraconal space and bulky extraocular muscles (EOMs) [Fig. 4]. MR angiogram exhibited dilated ICA communicating with the right CS (CCF: direct type) and aneurysm of the right ICA (6 mm × 8 mm × 7 mm) seen as an outpouching at the junction of the right middle cerebral artery (MCA) along with dilated right superior ophthalmic vein [Fig. 5]. Abdominal ultrasound revealed a small contracted right kidney of size 64 mm × 27 mm (left kidney of size 119 mm × 54 mm) with altered contour while mildly elevated blood urea (43 mg/dl) and serum creatinine (1.2 mg/dl) suggested some deranged renal functions [Fig. 6]. The patient was conservatively managed on antihypertensives and antiepileptic and recommended for endovascular intervention for treatment of the CCF and the aneurysm, which she declined. On medication, her BP had reduced to 170/100 mmHg.
The diagnosis of the direct, spontaneous variety of CCF is usually made due to a high index of clinical suspicion and confirmed radiologically although sometime it could be mistaken for orbital cellulitis or CS thrombosis. Direct CCFs usually present as proptosis, generalized ophthalmoplegia, chemosis and conjunctival injection, visual loss, and sometimes an audible bruit. CCFs of the direct, spontaneous type have been reported following surgical procedures and spontaneous rupture of intracranial aneurysms. Most ICAs (90%) remain asymptomatic before rupture while only 10% usually present with symptoms of mass effect, in the form of headaches. Aneurysms of ≥ 25 mm usually cause headaches and are more prone to rupture. Leonard suggested that in CCFs, the hypoxic, congested EOMs are responsible for ophthalmoplegia. Remzi reported a rare case of indirect CCF with unilateral proptosis and bilateral abducens palsies in the absence of EOMs swelling. In our case, the initial presentation of headache is likely due to the mass effect of the ICA, which may have formed due to secondary hypertension, perhaps, due to focal destruction of the internal elastic membrane by hemodynamic forces at the apices of the bifurcations: Which subsequently ruptured following the hypertensive episode. There is always the possibility that in this young female, the unilateral contracted kidney and consequent secondary hypertension with left ventricular hypertrophy can all be on account of the evident unilateral renal artery stenosis, as documented elsewhere too. The intracerebral bleed in our patient followed a rupture of the MCA as a possible consequence of a sudden rise in intra-arterial BP. The bilateral abduction deficits indicate the sixth nerves palsy on account of a raised intracranial pressure, further complicated by the swelling of the EOMs in the RE. Since patients of intracranial vascular malformations may often first present to ophthalmologists, an early diagnosis can prevent neurological complications and lead to a better outcome.
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