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Paper text:
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 .
her blood pressure ( bp ) on presentation was 200/150 mmhg and pulse rate was 64/min .
she had a visual acuity ( va ) re of count fingers at 1 m , and va left eye ( le ) was 20/25 on snellen .
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 .
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 .
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 .
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 .
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 ,
proptosis , swelling of lids along with chemosis and congestion of conjunctiva limited abduction in left eye on levoversion ( a ) on susceptibility - weighted imaging showing bleeds in the right frontal lobe and parietal lobe due to rupture of branches of middle cerebral artery .
( b ) t2-weighted imaging revealing bleed in frontal and parietal lobe along with surrounding edema ( a ) time - of - flight magnetic resonance angiogram revealing dilated superior ophthalmic vein of the right side .
( b ) t2-weighted imaging in coronal view revealing edema of extraocular muscles ( a ) time - of - flight magnetic resonance angiogram revealing right internal carotid artery aneurysm communicating with cavernous sinus .
( b ) on three - dimensional reconstruction , magnetic resonance imaging angiogram showing aneurysm of internal carotid artery at junction of middle cerebral artery and dilated right superior ophthalmic vein right small contracted kidney
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 .
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 .
we report a case of a 20-year - old female having systemic hypertension who presented with right - sided proptosis , chemosis , and diminished vision , preceded by an acute episode of unilateral throbbing headache . 
 imaging studies revealed a right - sided direct , spontaneous carotid - cavernous fistula ( ccf ) , aneurysm of internal carotid artery , bleed in the parieto - frontal lobe , and swelling of extraocular muscles . 
 abdominal ultrasound revealed a small contracted right kidney measuring 64 mm  27 mm . 
 a direct spontaneous ccf can occur spontaneously following rupture of intracranial aneurysm without any history of trauma or connective tissue disorder . 
 prompt diagnosis of intravascular malformations at initial presentation can prevent neurological complications and vision loss . 
 a team approach including emergency physicians , neurosurgeons , and ophthalmologists is needed for the proper management of such patients .