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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 59  |  Issue : 3  |  Page : 273-276

Radius maumenee syndrome - Idiopathic elevated episcleral venous pressure


Department of Glaucoma, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India

Date of Submission14-May-2020
Date of Decision12-Jun-2020
Date of Acceptance20-Jul-2020
Date of Web Publication09-Sep-2021

Correspondence Address:
Dr. Vijayalakshmi A Senthilkumar
Department of Glaucoma, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_53_20

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  Abstract 


A 42-year-old female patient has been referred for high intraocular pressure (IOP) in both eyes (BE). Examination revealed dilated, tortuous conjunctival, and episcleral vessels in BE with no apparent cause, also the patient had advanced glaucomatous damage in the left eye (LE). A diagnosis of bilateral idiopathic elevated episcleral venous pressure with secondary open-angle glaucoma (Radius Maumenee syndrome) was made. She was treated with aqueous suppressants in BE and underwent Partial diode cyclophotocoagulation in LE for control of high IOP. Nearly 50–55 cases have been reported till now. We publish here as this is a rare entity; also we discuss certain management controversies and provide literature review about this entity.

Keywords: Blood in Schlemm's canal, dilated episcleral vessels, diode cyclophotocoagulation, idiopathic, secondary glaucoma


How to cite this article:
Senthilkumar VA, Rajendrababu S, Krishnadas R. Radius maumenee syndrome - Idiopathic elevated episcleral venous pressure. TNOA J Ophthalmic Sci Res 2021;59:273-6

How to cite this URL:
Senthilkumar VA, Rajendrababu S, Krishnadas R. Radius maumenee syndrome - Idiopathic elevated episcleral venous pressure. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Sep 26];59:273-6. Available from: https://www.tnoajosr.com/text.asp?2021/59/3/273/325737




  Introduction Top


Regulation of intraocular pressure (IOP) is a function of secretion of aqueous humor, resistance to aqueous outflow at the anterior chamber angles, and episcleral venous pressure (EVP). When the episcleral veins are dilated, pressure in these veins becomes elevated. Normal EVP is 8–10 mm Hg. Prolonged elevation of EVP often causes a reduction in aqueous outflow facility, and this consequently causes secondary open-angle glaucoma (SOAG). The precise interrelationship between EVP and aqueous humor dynamics is complex and not well understood.[1] Most cases of dilated episcleral vessels with increased IOP can be attributed to carotid-cavernous-sinus fistula, cavernous sinus thrombosis, dural arteriovenous shunt, superior vena cava syndrome, Sturge weber syndrome, thyroid ophthalmopathy, orbital obstructive lesions, orbital varices. Arteriovenous fistula is the most common cause of elevated EVP. There are rare cases of SOAG without any underlying cause. This condition known as idiopathic elevated EVP (IEEVP) was first described by Minas and Podos.[2] Later, this entity was named as Radius-Maumenee syndrome.[3]


  Case Report Top


A 42-year-old female was referred for the evaluation of high IOP in both eyes (BE). There was no antecedent history of trauma to the orbit, head or neck, history of proptosis, anterior neck swelling, systemic illness, or family history of ocular disease. On examination, best-corrected-visual-acuity (BCVA) was 6/6 in the right eye (RE) and 6/60 in the left eye (LE). Anterior segment examination revealed bilateral hyperaemic conjunctiva with dilated and tortuous episcleral vessels [Figure 1] with Grade 2 relative afferent pupillary defect in LE. There was no restriction of ocular movements. IOP by applanation method was 24 mm Hg and 42 mmHg in RE and LE, respectively. Posterior pole evaluation with 90D lens was remarkable for a cup disc ratio (CDR) of 0.6 with sloping of the neuroretinal rim superiorly in RE and 0.9–0.95 CDR with diffuse retinal nerve fiber layer (RNFL) thinning in LE [Figure 2].
Figure 1: (a and b) Slit-lamp photography showing bilateral dilated and tortuous episcleral vessels

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Figure 2: (a and b) Fundus photo showing cup disc ratio of 0.6 with sloping of the neuroretinal rim superiorly in right eye and 0.9 cup-to-disc ratio with diffuse retinal nerve fiber layer thinning in left eye

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Visual field analysis by Humphrey automated perimeter revealed the fields to be normal in RE on 24-2 strategy and advanced glaucomatous visual field loss involving fixation on 10-2 strategy in LE. RNFL analysis by spectral-domain optical coherence tomography was characteristic for superior quadrant thinning in RE and diffuse thinning in LE [Figure 3]. Gonioscopy by Goldmann 3 mirror lens revealed bilateral open angles, with blood in Schlemm's canal [Figure 3]. All relevant investigations such as thyroid function tests, chest X-ray, B scan ultrasonography of the eye and orbital structures, Orbital Doppler imaging, computed tomography (CT) and magnetic resonance imaging (MRI) of brain, magnetic resonance arteriography (MRA), magnetic resonance venography (MRV) were found to be within the normal limits [Figure 4]. Invasive catheter angiography was not done in our patient as the above-mentioned investigations were found to be normal. The patient was diagnosed with Radius-Maumenee syndrome BE with advanced glaucoma LE based on the elevated IOP, optic nerve cupping, blood in Schlemm's canal, and unremarkable head and orbit imaging.
Figure 3: (a) Spectral domain optical coherence tomography showing superior retinal nerve fiber layer thinning in the right eye and diffuse thinning involving macula in the left eye. (b) Goniophoto showing blood in Schlemm's canal

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Figure 4: (a) Magnetic resonance imaging-axial T2-weighted image at the level of cavernous sinus showing normal cavernous sinus and cavernous segment of internal carotid artery. (b) Magnetic resonance arteriography-three-dimensional time-of-flight image of the normal circle of Willis. (c an d) magnetic resonance venography-three-dimensional veno inhance appears normal

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Management

The patient was initially treated with medical management with a combination of aqueous suppressants (topical dorzolamide 2%, timolol maleate 0.5%) in BE to reduce IOP. Owing to the advanced disc damage in LE with high IOP, filtering surgery was planned in LE to preserve the existing vision. Our patient was counseled regarding the risks and complications involved with invasive trabeculectomy surgery like a macular snuff-out phenomenon or wipe-out phenomenon, choroidal effusions and expulsive hemorrhage. The patient was advised to continue medical management as she was not willing to undergo trabeculectomy surgery.

At 1 month follow-up visit, her IOP in LE was found to be 38 mmHg by Goldmann applanation tonometry. Hence, we proceeded with noninvasive transscleral diode cyclophotocoagulation (IRIDEX TSCPC 810 nm Diode Laser-power-1500 mW, duration-1500 ms, 16 spots) in the inferior 180° to contain IOP and progressive glaucomatous visual loss. She was advised to continue aqueous suppressants in BE. At last available follow-up visit, 6 months since the diode laser treatment, LE ocular pressure was 18 mm Hg with aqueous suppressants (the combination of topical dorzolamide and timolol) with BCVA of 6/60. The patient has been advised to review with us periodically to closely monitor glaucomatous progression in RE.


  Discussion Top


IEEVP was first described by Minas and Podos in 1968 when they reported two cases of unilateral dilated episcleral veins, elevated EVP, and blood in the Schlemm's canal in a mother and daughter.[2] In 1978, Radius and Maumenee reported four additional cases of idiopathic dilated episcleral vessels and secondary open-angle glaucoma.[3] IEEVP is due to a congenital abnormality in the vasculature or localized venous obstruction in the region of extraocular muscles.[1],[4] The reported age of onset ranges from mid-teens to 70s, and most cases appear to be unilateral or bilateral with asymmetric involvement.[5] The youngest age of presentation reported in the literature was 15 years.[6] There is no known sex predilection, but the majority of those reported were women.[1] There is a familial predisposition in IEEVP.[2]

After the aqueous humor flows into the episcleral venous plexus, it flows first into the anterior ciliary vein, and then enters the superior ophthalmic vein. Then, above the annulus of Zinn, it enters the cavernous sinus into the superior vena cava through the internal jugular vein and finally drains into the right atrium.[5] Any obstruction of the drainage pathway may elevate EVP causing episcleral vessel dilation. Mechanistically, increased EVP can obstruct the normal aqueous outflow and lead to elevated IOP, and theoretically based on the Goldmann equation, there should be a linear relationship between EVP and IOP.[6] The measurement of EVP can be performed through direct and indirect methods, but no commercially available device is available, making it difficult to perform in the routine clinical setting. The causes of increased EVP can be divided into three groups [Table 1].
Table 1: Causes of elevated episcleral venous pressure

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As the diagnosis of IEEVP is one of exclusion, it is imperative to rule out other causes of increased EVP, such as carotid-cavernous sinus fistulas that lead to arteriovenous communication and venous obstructive disorders such as thyroid eye disease.[4] CT or MRI can help to identify any intraorbital or intracranial pathology, and CT or MR angiography is necessary to exclude intracranial vascular abnormalities. If identified, management of the primary neurovascular disorders usually results in stabilization of IOP.[7],[8] Our patient had a negative MRI/MRA ruling out a neurovascular abnormality.

The diagnosis of IEEVP is exclusively based on clinical findings such as dilated episcleral veins, open-angle, and elevated IOP with corresponding optic nerve and visual field findings consistent with glaucoma.[2],[5] Gonioscopy typically shows an open angle with blood in Schlemm's canal, but blood in Schlemm's canal can be seen in 20% of normal eyes also.[2] Our patient presented with bilateral dilated episcleral veins and blood in Schlemm's canal, suggesting bilateral IEEVP, which is sparsely reported in literature. Besides, our patient had early glaucomatous damage in RE controlled by medical management, whereas advanced glaucomatous damage in LE with very high IOP necessitating the need for Diode laser treatment, thus explaining the asymmetric nature of the disease.

Treatment can be challenging in these cases. Typically, topical medications are started, but most patients in the literature required maximum medical therapy, and few were stable only on medications. The efficacy of laser trabeculoplasty in these patients has not been well studied. There are reports of argon laser trabeculoplasty and micropulse diode laser trabeculoplasty having minimal effect on IOP in IEEVP.[5],[6] If there is a continued progression on maximum-tolerated medical therapy, glaucoma surgery should be performed. Most cases of IEEVP were managed with filtration procedures, including trabeculectomy, penetrating cyclodiathermy, sinusotomy, nonpenetrating deep sclerectomy and viscocanalostomy, with favorable outcomes reported in the literature.[5],[6],[9]

Certain intraoperative complications such as choroidal effusion, expulsive hemorrhage and flat anterior chamber are more likely to occur during trabeculectomy.[9],[10] Preoperative mannitol is mandatory. Other precautions like slow decompression, use of intracameral viscoelastics, tight scleral flap sutures may be considered to reduce the risk of intraoperative complications. Prophylactic sclerectomies could be considered in high-risk patients at the time of filtration surgery to prevent intraoperative and postoperative serous choroidal detachments.[5],[9],[10] In eyes with advanced glaucoma and limited visual potential, one may consider partial diode cyclophotocoagulation, micropulse G6 diode cyclophotocoagulation, or tube shunt surgery though the latter does not completely eliminate the risk of complications due to late hypotony.


  Conclusion Top


In patients presenting with elevated IOP and blood in Schlemm's canal, it is important to rule out the vision and life-threatening causes of increased EVP, before arriving at the diagnosis of IEEVP. We publish this case report to emphasize that in eyes with advanced glaucomatous disc damage with limited visual potential, where risks of complications like choroidal effusion and expulsive hemorrhage are significant after invasive glaucoma surgeries, then noninvasive partial diode laser cyclophotocoagulation with ancillary medical management is a viable option.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Jørgensen JS, Guthoff R. The role of episcleral venous pressure in the development of secondary glaucomas. Klin Monbl Augenheilkd 1988;193:471-5.  Back to cited text no. 1
    
2.
Minas TF, Podos SM. Familial glaucoma associated with elevated episcleral venous pressure. Arch Ophthalmol 1968;80:202-8.  Back to cited text no. 2
    
3.
Radius RL, Maumenee AE. Dilated episcleral vessels and open-angle glaucoma. Am J Ophthalmol 1978;86:31-5.  Back to cited text no. 3
    
4.
Foroozan R, Buono LM, Savino PJ, Sergott RC. Idiopathic dilated episcleral veins and increased intraocular pressure. Br J Ophthalmol 2003;87:652-4.  Back to cited text no. 4
    
5.
Rhee DJ, Gupta M, Moncavage MB, Moster ML, Moster MR. Idiopathic elevated episcleral venous pressure and open-angle glaucoma. Br J Ophthalmol 2009;93:231-4.  Back to cited text no. 5
    
6.
Sun CQ, Medert CM, Chang TC. Idiopathic elevated episcleral venous pressure in a teenager. Am J Ophthalmol Case Rep 2020;18:100712.  Back to cited text no. 6
    
7.
Horowitz ER, Forbes M, Podos MS, Tsai JC. Episodic elevation of intraocular pressure associated with blood in the Schlemm's canal. Arch Ophthalmol 2004;122:1230-2.  Back to cited text no. 7
    
8.
Lieb WE, Merton DA, Shields JA, Cohen SM, Mitchell DD, Goldberg BB. Colour Doppler imaging in the demonstration of an orbital varix. Br J Ophthalmol 1990;74:305-8.  Back to cited text no. 8
    
9.
Bhagat N, Lim JI, Minckler DS, Green RL. Posterior uveal effusion syndrome after trabeculectomy in an eye with ocular venous congestion. Br J Ophthalmol 2004;88:153-4.  Back to cited text no. 9
    
10.
Bellows RA, Chylack LT, Epstein DL, Hutchinson BT. Choroidal effusion during glaucoma surgery in patient with prominent episcleral vessels. Arch Ophthalmol 1979;97:493-7.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
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