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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 2  |  Page : 289-291

Radius–Maumenee syndrome: An unusual cause of unilateral glaucoma


Ophthalmology Service, Moulay Ismail Military Hospital, Meknes, Hassan II University Hospital, Faculty of Medicine and Pharmacy, Sidi Mohamed Ben Abdellah university, Fez, Morocco

Date of Submission04-Aug-2020
Date of Acceptance19-Nov-2020
Date of Web Publication01-Apr-2021

Correspondence Address:
Dr. Fatine El Alami
N 13 Avenue Errazi Cooperative Ouiame Champs de Course Fes Maroc
Morocco
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2520_20

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  Abstract 


Radius–Maumenee syndrome (SRM) is an idiopathic uncommon disorder that occurs with a dilation of the episcleral vessels and an increase in intraocular pressure (IOP). Most cases of dilated episcleral vessels can be attributed to carotid-cavernous-sinus fistula, cavernous sinus thrombosis, dural arteriovenous shunt, superior vena cava syndrome, Sturge–Weber syndrome, or Obstructive orbital lesions. There are rare cases without an underlying cause. It is a syndrome that constitutes a diagnostic and therapeutic challenge. The aim of our work was to report a case of Radius–Maumenee syndrome and to describe the diagnostic procedure for this relatively rare condition.

Keywords: Elevated episcleral venous pressure, glaucoma open-angle, Radius–Maumenee syndrome, unilateral glaucoma, venous pressure


How to cite this article:
El Alami F, El Ouafi A, Bouassel A, Iferkhass S, Laktaoui A. Radius–Maumenee syndrome: An unusual cause of unilateral glaucoma. Indian J Ophthalmol Case Rep 2021;1:289-91

How to cite this URL:
El Alami F, El Ouafi A, Bouassel A, Iferkhass S, Laktaoui A. Radius–Maumenee syndrome: An unusual cause of unilateral glaucoma. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Aug 3];1:289-91. Available from: https://www.ijoreports.in/text.asp?2021/1/2/289/312383



Radius–Maumenee syndrome was identified for the first time in 1978 by the two ophthalmologists who gave their names to the disease.[1] The characteristic clinical picture of this syndrome is idiopathic dilated episcleral vessels associated with increased IOP.[1] Increased IOP can be the result of the increased pressure of these vessels.[2] Several pathologies are known to cause episcleral venous pressure (EVP) increase with consequent increase in intraocular pressure (IOP). Idiopathic elevated EVP or Radius–Maumenee syndrome is a rare clinical entity with a characteristic clinical presentation, and therefore, it should be included among diagnostic possibilities. The finding of episcleral vessel dilation in non-inflamed eyes constitutes a warning to the ophthalmologist of possible glaucoma


  Case Report Top


We report a case of a 63-year-old man, who was referred to our department for unilateral glaucoma in his right eye. The patient had a history of type 2 diabetes and high blood pressure without any other ocular conditions or significant past medical history, but had chronic unilateral conjunctival hyperemia.

His first clinical assessment showed a best-corrected visual acuity at 6/6 for the right eye, 6/15 for the left eye, preserved and symmetric pupillary reactions, full ocular motility with no evidence of proptosis, ptosis or eye misalignment. Biomicroscopy presented dilated and tortuous episcleral vessels in the right eye [Figure 1], corneal scar on the left eye, clear anterior chambers, no iris alterations and clear lenses. IOP measured by Goldmann applanation tonometry was 27 mm Hg OD and 13 mm Hg OS. Funduscopy revealed a cup-to-disc asymmetry: 0.4 OD and 0.1 OS [Figure 2]. with otherwise normal posterior segment for both eyes. Gonioscopy revealed open iridocorneal angles and blood in Schlemm canal in 360 degrees of the right eye [Figure 3].
Figure 1: Clinical appearance of the eyes showing episcleral dilated veins in the right eye (a), normal appearance of the left eye (b)

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Figure 2: Cup-to-disc asymmetry 0.4 OD (a) and 0.1 OS (b)

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Figure 3: (a and b) Gonioscopy: open iridocorneal angle and blood in Schlemm canal in the right eye

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Optical coherence tomography of the peripapillary nerve fiber layer and automated perimetry were compatible with a glaucomatous damage on the right eye [Figure 4], whereas the left visual field was normal and intact. The patient's right eye was treated with a fixed combination of dorzolamide and timolol. The IOP has since been controlled in the low teens. Orbito-cerebral magnetic resonance angiography and catether angiography were used to detect the presence of arteriovenous fistulas, which are the most common cause of dilated episcleral vessels[3], but the result was normal. Other tests, including heart check-up, thyroid function tests, doppler ultrasound of the supra-aortic trunks, and orbit scan All were negative so the exclusion diagnosis of Radius–Maumenee syndrome was confirmed in the right eye.
Figure 4: Optical coherence tomography of the peripapillary nerve fibre layer (a) showing superior temporal RNFL thinning and automated perimetry (b) objectifying a nasal step, compatible with a glaucomatous damage on the right eye

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  Discussion Top


The intraocular pressure (IOP) depends on the rate of production of aqueous humor, the ease of its drainage and episcleral venous pressure.[4] Normal episcleral venous pressure ranges from 8 to 10 mm Hg, and for every 1 mm Hg of elevation in EVP, the mean IOP is thought to rise 1 mm Hg.[3] The most common sign of elevated EVP is dilation episcleral and conjunctival vessels. Depending on the underlying cause of the increased EVP, additional findings may include proptosis, chemosis, and orbital bruit [Table 1].[5] Gonioscopy reveals an open angle; in some cases, Schlemm canal may contain blood, thought to be present as a result of blood reflux from the episcleral veins. The IOP is elevated, and depending on the chronicity and severity of this increase, there may be evidence of glaucomatous optic neuropathy.
Table 1: Criteria of differentiating between elevated EVP and arteriovenous fistula

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It's important to identify any underlying causes of elevated EVP. Issues to explore include a history of thyroid disease, amyloidosis, superior vena cava syndrome, Sturge– Weber syndrome More Details, or carotid-cavernous sinus fistula, a mechanism made more likely by a history of trauma. Additional diagnostic workup for EVP elevation causes may include orbital ultrasound, computed tomography scan, and/or magnetic resonance imaging to rule out intraorbital or intra-cranial etiologies. Cases are only considered idiopathic when no underlying etiology is identified. Accordingly, the diagnosis of radius maumenee syndrome is made based on clinical findings[6]

Causes of elevated EVP can be divided into three categories: venous congestion, arteriovenous abnormalities, and idiopathic [Figure 5].
Figure 5: Causes of elevated EVP

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Although the pathogenesis of this condition remains obscure, it is believed that the presence of abnormal pressure in blood drainage could explain the emergence of the condition.[7] One hypothesis to explain the difficulty of venous return involves the hyalinization of Schlemm's canal.[8] Perhaps abnormal vasculature, present at birth, undergoes progressive dilatation until it's clinically apparent.

Initial management is directed at reducing IOP, because aqueous outflow is compromised, aqueous suppressants are more effective for the medical management of IOP resulting from elevated EVP. Apraclonidine has been shown to be effective for lowering IOP in cases of elevated EVP, because it causes arterial vasoconstriction, which decreases the amount of blood flow to the eye.[9] If the IOP remains uncontrolled despite medical management, then surgical options are considered. Several case reports point out that trabeculectomy in these eyes can be complicated by intraoperative or postoperative uveal effusions. Some physicians have recommended “tight” trabeculectomies, with multiple sutures surrounding the scleral flap, to allow gradual titration of the IOP in the postoperative period.


  Conclusion Top


Radius–Maumenee syndrome is a rare cause of secondary open-angle glaucoma, this syndrome leads to chronically increased IOP and glaucomatous damage of the optic nerve, it's a diagnosis of exclusion that requires exclusion of entities that raise episcleral venous pressure, Its management is a challenge, since there is not enough experience nor evidence.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Radius RL, Maumenee E. Dilated episcleral vessels and open-angle glaucoma. Am J Ophthalmol 1978;86:31-5.  Back to cited text no. 1
    
2.
Jyothi PT, Bindus S, Shimna I. Radius-Maumenee syndrome : A case report. Kerala J Ophthalmol 2011;23:152-4.  Back to cited text no. 2
    
3.
Allingham RR, Damji KF, Shields MB. Shields Textbook of Glaucoma. 6th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2011.  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;87:652-3.  Back to cited text no. 4
    
5.
Phelps CD, Armaly MF. Measurement of episcleral venous pressure. Am J Ophthalmol 1978;85:35-42.  Back to cited text no. 5
    
6.
Wen JC, Johnstone M. Increased episcleral venous pressure as an etiology of glaucoma. Glaucoma Today May/June 2017;53-4.  Back to cited text no. 6
    
7.
Grieshaber MC, Dubler B, Knodel C, Killer HE, Flammer J, Orgül S, et al. Retrobulbar blood flow in idiopathic dilated episcleral veins and glaucoma. Klin Monbl Augenheilkd 2007;224;320-3.  Back to cited text no. 7
    
8.
Lanzl IM, Welge-Luessen U, Spaeth GL. Unilateral open-angle glaucoma secondary to idiopathic episcleral veins dilated. Am J Ophthalmol 1996;121:587-9.  Back to cited text no. 8
    
9.
Mantzioros N, Weinreb R. Apraclonidine reduces intraocular pressure in eyes with increased episcleral venous pressure. J Glaucoma 1992;1:42-3.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1]



 

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