AN UNUSUAL CASE OF GLAUCOMA

DR. AAYUSH TANDON M.S Ophthalmology

CASEPresentationA 21 year old male patient presented withMild blurring of vision in right eyeRedness in right eye

3 months

Scar Mark

Past History•Road traffic accident one year back, resulting in fracture of right maxillary bone & right zygomatic bone.

•Operated by maxillo-facial surgeon by applying titanium plates for fixation of fractures.

Medical History: No history of headache, hypertension or diabetes.

Personal History: Sleep, appetite,bowel and bladder habits unaltered.

General Examination:•Conscious, oriented•Averagely built and well nourished•BP- 118/74 mm of Hg, Pulse- 84/min

Systemic Examination:•CVS: S1S2 heard, no murmurs.

•Lungs: Equal air entry and symmetrical expansion bilaterally.

•Abdomen: Soft, Non tender.

•Neurological Examination: Alert and oriented.

Ophthalmic Examination

Ophthalmic Examination

MANAGEMENT

• Patient underwent Perimetry – Visual Fields were normal

• Timolol Maleate 0.5% e/d BD in right eye.• Frequent follow up was advised for IOP

monitoring and to gauge treatment response.

1st FOLLOW UP (1 Month)

• Visual acuity remined the same in both eyes• Dilated and tortous episcleral veins were seen in the RE • No disc odema was seen on fundus examination• Perimetry was repeated - Fields were still normal • Brimonidine 0.2% e/d BD started in the right eye over and above timolol 0.5% e/d to

control the IOP• Patient was advised frequent follow to gauge the treatment response

2nd FOLLOW UP (3 months)

• Mild proptosis seen in both eyes (R>>L)

• More dilated episcleral vessels seen in the right eye

• Gonioscopy was repeated – Both eyes showed open angle

• No disc oedema seen on fundus examination

IOP still rising ???

• Taking in consideration proptosis, dilated episcleral vessels and past history of trauma further evaluation was done which revealed significant findings.

1) On palpation, arterial pulsations were felt on both sides

2) On auscultation of the orbit, bruit was heard

Provisional diagnosis of CAROTID CAVERNOUS FISTULA was made

• Dorzolamide 2% e/d BD was started in the right eye over and above Timolol and Brimonidine e/d

• Timolol 0.5% eye drop BD started in the left eye also

• Patient was advised further investigations to confirm the diagnosis

COLOUR DOPPLER

Dilated and tortuous superior ophthalmic veins with reversal of flow in both eyes

MAGNETIC RESONANCE IMAGING (MRI) BRAIN AND ORBIT (CONTRAST STUDY) WITH MAGNETIC

RESONANCE ANGIOGRAPHY (MRA)

Congestion of both superior ophthalmic veins and cavernous sinuses

Clinical diagnosis of‘Carotid Cavernous Fistula’

was confirmed.

• Patient was referred to higher centre for further management to be done by Neurologist and Interventional Radiologist

• He was advised to get a Cerebral Angiography(GOLD STANDARD) done to know to exact type

and location of the fistula.

TREATMENT• Patient was treated by interventional embolization

by coiling of fistula.• Patient followed up to us regurlarly after

embolization • His signs and symptoms started reducing gradually• Anti glaucoma medication was gradually tapered.

• IOP in both eyes came down to 18 mm of Hg over a period of 12 weeks

CAROTID CAVERNOUS FISTULA

• Type of arteriovenous fistula

• Abnormal communication between the internal or external carotid arteries and the cavernous sinus

Direct carotid-cavernous sinus fistulae2 types1

Dural carotid-cavernous sinus fistulae

TYPES

(Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT: Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 62:248–256, 1985)

TYPES

OPHTHALMIC MANIFESTATIONS

OPHTHALMIC MANIFESTATIONS

GLAUCOMA IN CAROTID-CAVERNOUS FISTULA

Other Factors•Elevation of orbital pressure secondary to venous stasis and edema•Anterior segment neovascularization•In extreme cases Secondary angle closure occurs due to congestion of the choroid and ciliary body and a forward shift of iris diaphragm

TREATMENT“Completely occlude the fistula while preserving

the normal flow of blood through the ICA”

(I)Endovascular Intervention(I) Trans arterial Embolization(II) Trans venous Embolization

(II)Surgical Intervention

(III)Radiosurgical Intervention

Summary– CAROTID CAVERNOUS FISTULA occurs in just 0.2%

of patients with cranio-cerebral trauma

– Commonly seen in young male patients

– Bilateral involvement is seen in 1% – 2% of patients with post traumatic fistulas.

- Life threatening sequelae (Intracranial hemorrhage, SAH etc) if not diagnosed and treated in time.

• A patient having CAROTID CAVERNOUS FISTULA may rarely present to an OPHTHALMOLOGIST.

• Because the symptoms and signs of a Carotid Cavernous Fistula often are mild in the initial stages (specially with Indirect/Dural fistulas), this lesion is difficult to diagnose and is often misdiagnosed as chronic conjunctivitis, blepharoconjunctivitis or POAG

• Therefore a high index of suspicion is required for the correct diagnosis and management

CONCLUSION

Our case was Rarest of rare

Complete resolution of Intractable glaucoma, proptosis and congestion occurred after correct treatment

Life threatening complications could be avoided

Why have we presented this case ?

REFERENCES1. Jason A , Hannah G, Sande P, Philip M. Carotid-cavernous fistulas.

Neurosurg Focus. 32 (5):E9, 2012, 314-23.

2. Borba LAB, Al-Mefty O: Normal anatomy of the cavernous sinus, in Eisenberg MB, Al-Mefty O (eds): The Cavernous Sinus: A Comprehensive Text. Philadelphia: Lippincott Williams & Wilkins, 2000, pg 21–34.

3. Pedersen RA, Troost BT, Schramm VL: Carotid-cavernous sinus fistula after external ethmoid-sphenoid surgery. Clinical course and management. Arch Otolaryngol 107:307–309,1981.

4. Isamat F, Twose J, Conesa G: Surgical management of cavernous-carotid fistulas, in Eisenberg MB, Al-Mefty O (eds): The Cavernous Sinus: a Comprehensive Text. Philadelphia:Lippincott Williams & Wilkins, 2000, pp 201–208.

5. Hirai T, Korogi Y, Goto K, Ogata N, Sakamoto Y, Takahashi M: Carotid-cavernous sinus fistula and aneurysmal rupture associated with fibromuscular dysplasia. A case report. Acta Radiol 37:49–51, 1996.

REFERENCES6. De Keizer R: Carotid-cavernous and orbital arteriovenous fistulas:

ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity. Orbit; 22:121–142, 2003.

7. Lewis AI, Tomsick TA, Tew JM Jr: Management of 100 consecutive direct carotid-cavernous fistulas: results of treatment with detachable balloons. Neurosurgery; 36:239–245, 1995.

8. Schroth G, Lovblad O, Ozdoba C, Remonda L. Non-traumatic neurological emergencies: emergency neuroradiological interventions. Eur Radiol 2002;12:1648–1662.

9. Uchino A, Hasuo K, Matsumoto S, Masuda K. MRI of dural carotid-cavernous fistulas. Comparisons with postcontrast CT. Clin Imaging. Oct-Dec 1992;16(4):263-8.

10. Elster AD, Chen MY, Richardson DN, Yeatts PR. Dilated intercavernous sinuses: an MR sign of carotid-cavernous and carotid-dural fistulas. AJNR Am J Neuroradiol. Jul-Aug 1991;12(4):641-5.

THANK YOU

CAROTID CAVERNOUS FISTULA patient may present with raised intraocular pressure and the etiology may remained undiagnosed unless thorough evaluation is done