Elevated Episcleral Venous Pressure (EVP)

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Elevated Episcleral Venous Pressure (EVP)
Classification and external resources
ICD-10 H40.8
OMIM 137700


Disease Entity

Elevated episcleral venous pressure (EVP) is a clinical finding which may be observed in a variety of primary conditions. It can also be idiopathic, although this is a diagnosis of exclusion. In either case, elevated EVP may be associated with elevated intraocular pressure (IOP) and glaucoma. Idiopathic elevated EVP leading to secondary open angle glaucoma is also known as Radius-Maumenee syndrome in German literature.[1]

Disease

Average EVP ranges from 8-10 mmHg, although it can transiently elevate with downward displacement of the head.[2] Persistently elevated EVP is a known cause of open-angle glaucoma as it can lead to obstruction of the aqueous drainage into the orbital venous system.[3] If not caught early it can lead to an insidious onset of glaucoma and subsequent vision loss.[4] Idiopathic elevated EVP was first described in 1968 by Thomas Minas and Steven Podos in a case report of a family with two members found to have the condition after ruling out primary entities known to cause secondary elevated EVP.[5]

Etiology

The Goldmann equation describes intraocular pressure (IOP) as the rate of aqueous humor production divided by the facility of outflow plus EVP.[6] In the acute setting, IOP rises 1 mmHg for every 1 mmHg increase in EVP.[7] However, the relationship between IOP and elevated EVP in chronic conditions is less well understood. Chronically elevated EVP can cause blood to reflux into Schlemm’s canal, which can decrease the facility of outflow of aqueous into the trabecular meshwork and subsequently raise the IOP.

Etiologies leading to elevated EVP are numerous and can be divided into several subcategories including venous obstruction, arteriovenous anomalies, and idiopathic pathologies.[8][9][10] Patients may endorse a distant history of craniofacial trauma that might suggest the cause of a carotid cavernous sinus, dural fistula, or other arteriovenous anomaly.[7] Potential causes of elevated EV include:

  • Venous Obstruction
    • Retrobulbar tumor
    • Orbital amyloidosis
    • Thyroid ophthalmopathy
    • Jugular vein obstruction
    • Congestive heart failure
    • Thrombosis of cavernous sinus or orbital vein
    • Vasculitis involving episcleral vein or orbital vein
    • Superior vena cava syndrome (mediastinal tumor)
  • Arteriovenous Anomalies
    • Carotid-cavernous sinus fistula
    • Orbital varix
    • Sturge–Weber syndrome
    • Orbital–meningeal shunts
    • Carotid–jugular venous shunts
    • Intraocular vascular shunts
  • Idiopathic
    • Sporadic
    • Familial

Risk Factors

Any history of head trauma is a risk factor for developing a carotid cavernous sinus, dural fistula or other arteriovenous anomaly which can lead to the development of elevated EVP.[7]

Pathophysiology

Branches of the anterior ciliary venous circulation system anastomose with efferent channels of the canal of Schlemm before converging with the episcleral venous plexus of Tenon's capsule and the conjunctiva. With very few capillary networks present in the angioarchitecture of the episcleral vessels, arteriovenous vasculature anastomoses predominate.[6] The episcleral venous plexus will ultimately drain into the ophthalmic veins, which then drain into the cavernous sinus.[8]

Increased venous pressure downstream to the episcleral anastomoses leads to decreased outflow of venous blood from the orbit and decreased outflow of aqueous. A subsequent pressure difference across the trabecular meshwork occurs, lowering aqueous egress. Elevated EVP resulting in chronic IOP elevations can then lead to glaucomatous damage and the eventual diagnosis of secondary open angle glaucoma.[5]

Diagnosis

History

Patients with elevated EVP may be entirely unaware of their condition or the underlying cause. They generally present without typical glaucomatous signs or symptoms early in their disease. Patients may endorse a distant history of craniofacial trauma that might suggest the cause of a carotid cavernous sinus, dural fistula or any other arteriovenous anomaly.[7] It is important to review the patient’s medical history, particularly for conditions such as amyloidosis, hyperthyroidism, congestive heart failure, hypercoagulable states, vasculitis, superior vena cava syndrome, Sturge–Weber syndrome, or other arteriovenous anomalies which may suggest an underlying cause for the elevated EVP.

Physical examination

A clinical diagnosis of elevated EVP can be suspected when a patient presents with chronically injected episcleral vessels without a history of pain or irritation (Fig. 1).[7] Elevated EVP can lead to small concentrations of blood reflux seen in Schlemm’s canal on gonioscopy.[11] Hyalinization of the inner wall of Schlemm’s canal can occur secondary to this chronic accumulation of blood in the canal.[11]Additionally, if ischemia has occurred, neovascularization of the iris can be present.[12] It should be noted that blood in Schlemm’s canal, hyalinization of the inner wall, and neovascularization of the iris are all not specific for elevated EVP, but one should have raised suspicions of elevated EVP should they occur. It is of particular importance to be suspicious of elevated EVP in patients with unilateral dilated episcleral vessels.[13] Elevated EVP can be present bilaterally, but most commonly presents unilaterally as the condition is commonly a sequela of ipsilateral localized anatomical variants.[3]

Other physical exam findings include chemosis, proptosis, and an orbital bruit. However, these are less reliable findings and their presence can depend on the underlying conditions leading to elevated EVP.[14] Pulsatile exophthalmos may be seen when elevated EVP is associated with a carotid-cavernous fistula.[15] In elevated EVP, glaucomatous optic atrophy and visual field loss can be more insidious than other acute forms of glaucoma.

Fig 1. Corkscrew vessels in elevated episcleral venous pressure.

Diagnostic procedures

Elevated EVP is often the result of another underlying disorder. A complete workup of the patient with elevated EVP to rule out underlying causes may include ultrasound biomicroscopy, orbital ultrasonography, computerized axial tomography, magnetic resonance imaging, chest x-ray and angiography.[6][16] Only after this workup is completed and negative may the patient be considered to have idiopathic elevated EVP.

There are several different diagnostic modalities which may be used to measure the EVP, though many of these are not routinely performed clinically. These include one direct method (cannulation), and three indirect methods (venomanometer pressure chamber, torsion balance, and air jet). The most accurate of these is direct cannulation, and the most accurate form of the indirect method is venomanometer.[3][17] Other investigators have also used color-coded Doppler sonography to measure blood pulsation from the ophthalmic veins.[18]

Direct Cannulation. This method includes insertion of a cannula into an episcleral vein. These veins range in diameter from 50-100 micrometers, and investigators have used two different cannulation methods: complete and partial vessel occlusion. In the complete occlusion method, plastic tubing is employed to occlude an episcleral vessel completely. In the partial vessel occlusion method, a glass pipette with a beveled 2-5 micrometer tip is placed in the episcleral vessel. For best measurements, the cannula must face downstream.[19]

Venomanometer Pressure Chamber. In short, the venomanometer pressure chamber method requires a particular apparatus to be attached to the slit lamp with a chamber consisting of an encased, highly distensible and transparent membrane, which is applied to the conjunctiva at a preselected episcleral vessel. The pressure within the chamber is slowly increased until a subjective endpoint of a collapsed episcleral vein is observed.[17] Several membranes have been utilized in this technique including latex, serosal membrane, frog pericardium, and silicone rubber. This is the most common non-invasive method for determining EVP in part due to its commercial availability (marketed as Episcleral Venomanometer, Model EV-310, EyeTech Ltd, Morton Grove, IL).[19]

Torsion Balance. This method requires a particular torsion balance instrument (very similar to the Goldmann applanation tonometer) to be mounted to the slit lamp. The applanation surface of the instrument has a diameter of 300 micrometers and is placed directly on the conjunctiva.[17] Magnification is set at 25 to 40X and force is applied until a subjective endpoint of a collapsed episcleral vein is observed. Being that the area of the applanation surface is fixed, EVP can be determined by the force required to collapse the vessel.[19]

Air Jet. Similar to the venomanometer and torsion balance methods, the air jet also utilizes a subjective endpoint of a collapsed episcleral vein by an applied pressure in this case administered via an air jet.[19]

Laboratory test

There is currently no laboratory test routinely used to suggest a patient has elevated EVP. Lab testing may be used as needed to evaluate for any underlying conditions.

Differential diagnosis

The differential diagnosis for elevated EVP includes entities that present with prominent ocular vessels. Elevated EVP tends to present with larger caliber, more tortuous vessels than many of the following etiologies. Other differential diagnosis for a chronically injected eye includes:

  • Ataxia telangiectasia
  • Acute closed angle glaucoma
  • Scleritis
  • Episcleritis
  • Conjunctivitis
  • Rosacea
  • Episcleral nodule
  • Cornea lesion near the limbus
  • Foreign body
  • Herpetic keratitis
  • Uveal neoplasm
  • Polycythemia vera
  • Leukemia[3][20]

Management

The management of elevated EVP can be medical or surgical. Care should be taken first to address any primary medical condition that might have caused the elevated EVP and would require specific treatment.

Medical therapy

Medical management of elevated IOP and secondary glaucoma due to elevatedEVP includes inhibiting aqueous production via beta-blockers and carbonic anhydrase inhibitor. Apraclonidine may also be considered due to its arterial vasoconstriction effects leading to less blood flow to the eye.[21]

Surgery

Surgical intervention should be considered in cases that have not responded first to more conservative efforts. According to several case reports in the literature, uveal effusion-like syndromes have complicated trabeculectomies in elevated EVP and some authors recommend sclerotomies with filtering surgery.[4][22] Laser trabeculoplasty has shown not to be beneficial.[3] One case report recommends performing a "tight" trabeculectomy with multiple releasable sutures to titrate the IOP gradually and prevent anterior chamber shallowing during surgery.[23]

Follow up

Follow up of elevated EVP may depend upon the underlying cause, but otherwise is similar to that of other glaucomatous conditions. Patients should undergo routine follow-up assessing visual acuity, IOP, gonioscopy, visual fields, OCT, and DFE and EVP with appropriate referral as necessary for any additional underlying condition.

Complications

A major complication of untreated elevated EVP is secondary open angle glaucoma. Elevated EVP can also lead to acute angle-closure glaucoma because venous stasis in the vortex veins can cause serous choroidal detachment or suprachoroidal hemorrhage leading to the forward displacement of the lens-iris diaphragm.[24][25] Neovascular glaucoma has also been reported as a complication of elevated EVP once arterial blood flow has met enough IOP resistance to cause ocular ischemia.[26]

Additional Resources

References

  1. Rhee DJ, Gupta M, Moncavage MB, Moster ML, Moster MR. Idiopathic elevated episcleral venous pressure and open-angle glaucoma. British Journal of Ophthalmology. 2009 Feb 1;93(2):231-4.
  2. Allingham RR, Damji KF, Freedman SF, Moroi SE, Rhee DJ, Shields MB. Shields textbook of glaucoma. Lippincott Williams & Wilkins; 2012 Mar 28.
  3. 3.0 3.1 3.2 3.3 3.4 Craven ER. Raised episcleral venous pressure. Ophthalmology. 2008.
  4. 4.0 4.1 Parikh RS, Desai S, Kothari K. Dilated episcleral veins with secondary open-angle glaucoma. Indian journal ofophthalmology. 2011 Mar;59(2):153.
  5. 5.0 5.1 Minas TF, Podos SM. Familial glaucoma associated with elevated episcleral venous pressure. Archives of Ophthalmology. 1968 Aug 1;80(2):202-8.    
  6. 6.0 6.1 6.2 Moster M, Ichpujani P. Episcleral venous pressure, and glaucoma. Journal of Current Glaucoma Practice. 1996;3(1):1143-55.    
  7. 7.0 7.1 7.2 7.3 7.4 Cioffi GA, Durcan FJ, Girkin CA. Basic and Clinical Science Course: Glaucoma. San Francisco: American Academy of Ophthalmology. 2013:26.    
  8. 8.0 8.1 Higginbotham EJ. Glaucoma associated with increased episcleral venous pressure. Principles and practice of ophthalmology. WB Saunders, Philadelphia. 2000:2781-92. 
  9. Weinreb RN, Jeng S, Goldstick BJ: Glaucoma secondary to elevated episcleral venous pressure. In: Ritch R, Shields MB, Krupin T, (eds): The glaucomas. St. Louis: CV Mosby; 1989: p 1130.      
  10. Nelson GA, Edward DP, Wilensky JT. Ocular amyloidosis and secondary glaucoma. Ophthalmology. 1999 Jul 1;106(7):1363-6. 
  11. 11.0 11.1 Roll P, Benedikt O. Dilatation and tortuosity of episcleral vessels in open angle glaucoma. II. Electron microscopy study of the trabecular meshwork [in German]. Klin Monatsbl Augenheilkd. 1980;176:297-301.
 
  12. Harris MJ, Fine SL, Miller NR. Photocoagulation treatment of proliferative retinopathy secondary to a carotid-cavernous fistula. American Journal of Ophthalmology. 1980;90(4):515-8.
  13. Lanzl IM, Welge-Luessen U, Spaeth GL. Unilateral open-angle glaucoma secondary to idiopathic dilated episcleral veins. American Journal of Ophthalmology. 1996 May 31;121(5):587-9.    
  14. Allingham RR, Damji KF, Freedman SF, Moroi SE, Rhee DJ, Shields MB. Shields Textbook of Glaucoma. Lippincott Williams & Wilkins; 2012 Mar 28.    
  15. Heichel J, Hammer T, Solymosi L, Brandt S, Winter I. Pressure-Lowering Effect of Fistula Occlusion in a Patient with Secondary Glaucoma Due to an Intracranial Arteriovenous Fistula. Ophthalmology and therapy. 2015 Dec 1;4(2):135-41.    
  16. Kranemann CF, Pavlin CJ, Trope GE. Ultrasound biomicroscopy in Sturge-Weber-associated glaucoma. American Journal of Ophthalmology. 1998 Jan 31;125(1):119-21.    
  17. 17.0 17.1 17.2 Brubaker RF. Determination of episcleral venous pressure in the eye: a comparison of three methods. Archives of Ophthalmology. 1967 Jan 1;77(1):110-4.
  18. Cymbor M, Knapp E, Carlin F. Idiopathic Elevated Episcleral Venous Pressure with Secondary Glaucoma. Optometry & Vision Science. 2013 Jul 1;90(7):e213-7.    
  19. 19.0 19.1 19.2 19.3 Sit AJ, McLaren JW. Measurement of episcleral venous pressure. Experimental eye research. 2011 Sep 30;93(3):291-8.    
  20. Bagheri N, Wajda B, Calvo C, Durrani A. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. Lippincott Williams & Wilkins; 2016 Apr 19.    
  21. Mantzioros N, Weinreb RN. Apraclonidine reduces intraocular pressure in eyes with increased episcleral venous pressure. Journal of Glaucoma. 1992 Apr 1(1):42-3.    
  22. Bhagat N, Lim JI, Minckler DS, Green RL. Posterior uveal effusion syndrome after trabeculectomy in an eye with ocular venous congestion. British Journal of Ophthalmology. 2004 Jan 1;88(1):153-4.    
  23. Pradhan ZS, Kuruvilla A, Jacob P. Surgical management of glaucoma secondary to idiopathic elevated episcleral venous pressure. Oman Journal of Ophthalmology. 2015 May;8(2):120.
  24. Buus DR, David TT, Parrish RK. Spontaneous carotid cavernous fistula presenting with acute angle closure glaucoma. Archives of Ophthalmology. 1989 Apr 1;107(4):596-7.    
  25. Fourman S. Acute closed-angle glaucoma after arteriovenous fistulas. American Journal of Ophthalmology. 1989 Feb 28;107(2):156-9.    
  26. Spencer WH, Thompson HS, Hoyt WF. Ischemic ocular necrosis from carotid-cavernous fistula. Pathology of stagnant anoxic inflammation in orbital and ocular tissues. The British Journal of Ophthalmology. 1973 Mar;57(3):145.