Transcript Direct CCF
Grand Rounds August 25, 2006 Jeffrey D. Colburn, M.D., PGY-2 Vanderbilt Eye Institute The Case… • CC: Right eye proptosis, vision loss • 81 y.o. Cauc male – – – – Presented at outside facility for HA and neck pain Noted double vision, “whooshing” in Right ear s/p Neurosurgical procedure Poor vision right eye, stable • Past Ocular Hx: Reading Glasses. No injuries/surgeries. History • Past Med/Surg Hx – – – – Hypothyroidism Hip fx with repair 1999 Dyslipidemia Recent repair R fem pseudoaneurism • Meds – Gemfibrozil – Levothyroxine – ASA • Allergies: NKDA • Social Hx – No tobacco/ETOH • ROS – Negative except as per HPI Exam • General: Awake & alert, NAD, mood/affect appropriate • • • • • VA (near, +4.00): 20/400 OD, 20/40 OS+1 IOP: 22,27 OD, 17 OS CVF: grossly full Pupils: 4 2.5 OU, + RAPD OD Motility: -2 in all gaze positions OD, Full OS • Hertel: 19 OD, 16 OS (104mm) Exam • External: Proptosis, chemosis, conj injection, resistance to retropulsion OD • Bruit heard on auscultation right orbit • PLE LLL: Crusted lashes, lagophthalmos 4mm OD Conj: 3+ chemosis, 3+ injection OD K: Clear & Quiet OU A/C: Formed & Quiet OU Iris: Intact OU Lens: NSC OU Ant Vit: Quiet OU Exam • DFE: Disks: sharp margins, no papilledema or pallor OU C/D: 0.7 OD, 0.5 OS Macula: flat OU Periphery: flat OU Vitreous: clear OU Vascular: wnl OU Differential Diagnosis Differential Diagnosis • Vascular – Carotid-cavernous fistula – Arteriovenous malformation – Cavernous sinus thrombosis • Neoplastic – – – – Cavernous sinus tumors Orbital Tumors Skull base tumors Mucocele • Trauma – Retrobulbar hemorrhage – Intraorbital foreign body • Infectious – Orbital cellulitis – Mucormycosis – Tuberculosis • Neurologic – Cranial nerve palsy • Inflammatory/Infiltrative – Thyroid eye disease – Orbital pseudotumor – Orbital vasculitis • Wegener’s granulomatosis • Polyarteritis nodosa – Intracranial sarcoidosis – Tolosa-Hunt syndrome Further History • 1/06 -- Onset double vision, “whooshing” in right ear • Diagnosed with CC-Fistula • 6/06/06 – PCI with stent – – – – Complicated by massive intracranial hemorrhage Required ventriculostomy Decreased VA OD noted after awakening in ICU Extended hospital course, then rehab • 7/21/06 – Presents to outside hospital – new HA, neck pain – CT shows “enlarged superior orbital vein consistent with CCF” Diagnosis: “Recurrent” Carotid Cavernous Sinus Fistula Our part in management… • Exposure: – Lacrilube • Increased IOP: – 2 hrs after initial eval, IOP up to 30,32 OD – Cosopt started – 3 hours later, IOP down to 22 OD – Cosopt cont BID OD, followed IOP Carotid Cavernous Sinus Fistula • Symptoms: • Signs: Carotid Cavernous Sinus Fistula • Symptoms: – Double vision – eyelid droop – facial pain/numbness • Signs: – – – – – – – – – – – – Proptosis chemosis IOP ocular pulse pressure orbital/temple bruit ptosis miosis ophthalmoplegia (most commonly CN VI) facial hypoesthesia optic disk swelling retinal venous dilatation intraretinal hemorrhage • Classic Sign: Limbal injection with arterialized conjunctival & episcleral vessels Anatomy Diagnostic Studies • Orbital color doppler U/S: – Reversed, arterialized flow in S. Ophthal v. • CT/MRI: – Enlarged S. Ophthal v. – Enlarged EOM – Proptosis • Cerebral Angiography – “Gold Standard” diagnosis – View ICA, ECA, & vertebral circulations Pathophysiology • Retrograde venous drainage into orbit – Venous HTN Enlarged EOM’s Restriction Diplopia Proptosis Exposure keratopathy Chemosis & Injection Red Eye Increased episcleral & vortex venous pressure Increased IOP Secondary Glaucoma – Venous & Arterial Stasis Decreased ocular/retinal perfusion Decreased visual acuity Anterior Segment Ischemia Decreased perfusion to intra CS cranial nerves Ophthalmoplegias Diplopia Pathophysiology • Also remember venous HTN in other directions as well. Important Example: – Retrograde cortical venous drainage • 10-55% of CCF cases Severe HA Contralateral neuro deficits 30-40% risk of intracerebral hemorrhage May be fatal Secondary Glaucoma • Neovascular: ant segment ischemia • Angle Closure: choroidal effusions • May see blood in Schlemm’s canal • Unilateral • Tx: Medical Classification • Direct CCF (High-flow) – – – – Most common (70-90%) 75% traumatic (assoc. basal skull fx) Defect in intra-cavernous ICA Spontaneous: mid-age, HTN, postmenopausal female • Indirect CCF (Low-flow) – Dural Shunt: Meningeal arteries (ICA, ECA or combo) – Can be spontaneous or traumatic also – More insidious onset of symptoms Barrow’s Classification (1985) • Type A: Direct between ICA and CS • Type B: Dural ICA branches to CS (uncommon) • Type C: Dural ECA branches to CS • Type D: Dural ICA & ECA branches to CS Prognosis • Direct CCF: – Poor visual prognosis, 90% with severe vision loss – Ocular & optic nerve damage, exposure keratopathy, sec. glaucoma, ant segment ischemia, CRVO, ischemic ON – Concern for intra-cerebral hemorrhage • Indirect CCF: – – – – Prognosis less severe Also concern for intra-cerebral hemorrhage Exacerbation & remission is the hallmark May close spontaneously (10-60%) Management – Indications for Tx: • Lack of spontaneous closure, risk to eye/vision, intolerable symptoms, “high-risk” for stroke, venous thrombosis, mental status changes – Surgical closure • Rare in last 30 years • Can be salvage option – Interventional Radiology (balloon occlusion/embolization): • Trans-arterial route directly through tear or embolization of feeding vessels • Trans-venous through S. Ophthal. v. or Inferior Petrosal sinus Endovascular Management • Meyers, et al. Am J Ophthalmology, 2002 – Retrospective interventional case series • • • • 133/135 consecutive cases had tx 121 (90%) patients were cured clinically (mean f/u 56 mos) 4% patients with moderate/severe disability 6% with symptomatic complications – Cerebral infarction, Decreased VA (2), Diabetes Insipidus, orbital eccymosis, retroperitoneal hematoma, DVT’s (2) • No operative mortality – Conclusions • High success rate • Low complication/morbidity rate • Patient’s ocular symptoms may be transiently worsened postprocedure Superior Ophthalmic Vein Approach • First proposed by Hanneken, et al. in 1989. – Direct access to cavernous sinus – Potential complications: puncture of S. ophthal v., orbital hemorrhage, infection, trochlea or other structure damage • Goldberg, et al. Arch Ophthalmol, 1996. – Retrospective clinical series • • • • • 10 consecutive cases with dilated s. ophthal v. Anterior orbitotomy with cannulation 100% with resolution of s/s, halt of visual loss 90% achieved premorbid BCVA No significant complications – Conclusions • Especially effective with significant ICA contribution to CCF • “technically straightforward, safe, and effective treatment” • Leibovitch, et al. Ophthalmology, 2006. – Present difficult cases out of 25 consecutive – Unable to isolate or cannulate 6 • Complicating factors: – – – – Small or fragile vein (3) Large posterior varix rupture & bleed (1) Clotted anterior SOV (2) SOV located inferiorly (1) • Conclusions: – SOV important & useful route – Fragile or clotted veins, deep orbital dissections should be avoided Back to our patient… • 7/24/06 – Endovascular Intervention (trans-art.) – Angiogram shows previously place stent in R ICA, but persistent feeders – Embolization of feeders from R Middle Meningeal Artery – Multiple feeders from Right Internal Maxillary Artery were not embolized Course • Hospital stay complicated by MRSA PICC line infection and UTI. • D/C home 8/2/06 • Will f/u with Ophthalmology 9/5/06 • Scheduled for repeat cerebral angiogram and embolization of AVM on 9/25/06 References • • • • • • Kanski, JJ. Clinical Ophthalmology: A systematic Approach. 5th ed. Butterworth Heinemann, New York, 2003. p243-244, 574-575. Kunimoto, DY, et al. The Wills Eye Manual: Office and emergency room diagnosis and treatment of eye disease. 4th ed. Lippincott Williams & Wilkins, Baltimore, 2004. p126-128, 213-215. Ringer AJ, Salud L, Tomsick TA. Carotid cavernous fistulas: Anatomy, classification, and treatment. Neurosurg Clin N Am 2005;16:279-295. Meyers PM, et al. Dural carotid cavernous fistula: Definitive endovascular management and long-term follow-up. Am J Ophthalmol 2002;134:85-92. Goldberg RA, et al. Management of cavernous sinus-dural fistulas: Indications and techniques for primary embolization via the superior ophthalmic vein. Arch Ophthalmol 1996;114:707-714. Leibovitch, et al. Lessons learned from difficult or unsuccessful cannulations of the superior ophthalmic vein in the treatment of cavernous sinus dural fistulas. Ophthalmology 2006;113:1220-1226.