Advanced glaucoma secondary to bilateral idiopathic dilated episcleral veins – a case report

Abstract

BackgroundIdiopathic elevated episcleral venous pressure (IEEVP) or idiopathic dilated episcleral veins (IDEV) is a rare abnormality, and thus therapeutic treatment for this condition rarely is discussed. We report a case of a patient with bilateral glaucoma secondary to IDEV for whom intraocular pressures (IOPs) were controlled successfully by trabeculectomy.Case presentationA 50 year-old female with a complaint of persistent red eyes for over 30 years, presented with numerous tortuous and engorged episcleral vessels in both eyes (OU), open angles OU with spontaneous blood in Schlemm’s canal 360 degrees bilaterally. Orbital color Doppler examination showed the superior ophthalmic veins to be of normal calibre, with no reversal of flow. Head MRI with contrast and cerebral angiography were negative for arteriovenous fistula. Coronary angiography, color Doppler echocardiography and chest radiographs were within normal limits. A diagnosis of secondary glaucoma and IDEV was made. Neither anti-glaucoma medications, nor laser trabeculoplasty reduced the patent’s IOP effectively. Only after trabeculectomy was performed in each eye, were IOPs successfully controlled.ConclusionsThis case serves to remind clinicians of the importance of identifying and evaluating thoroughly patients with episcleral vessel dilation in non-inflamed eyes with no known cause. A misdiagnosis or missed diagnosis of long-term elevated intraocular pressure may result in significant damage to the optic nerve. In addition, when performing filtration surgery it is crucial that the ophthalmologist control the IOP and make an effort to prevent choroidal effusion.

Bilateral Episcleral Corkscrew Vessels: Expedition into the Unknown – Case Report

“…IEEVP or Radius-Maumenee syndrome is characterized by arterialized, engorged, and tortuous episcleral veins, with raised IOP, and, almost always, open-angle glaucoma is already present at the time of diagnosis [ 3 , 6 , 7 , 13 ]. It affects individuals of any age with no gender preference.…”

“…It affects individuals of any age with no gender preference. This condition could be unilateral or bilateral; when bilateral, it may be asymmetrical [ 3 ]. IEEVP is a diagnosis of exclusion and can only be established after other conditions, some of which are life-threatening, have been excluded [ 1 ].…”

“…Other conditions that lead to venous obstruction include retrobulbar tumours, cavernous sinus thrombosis, thyroid orbitopathy, and superior vena cava syndrome [ 1 , 2 ]. Idiopathic EEVP (IEEVP) or Radius-Maumenee syndrome is a rare entity and should be considered a diagnosis of exclusion [ 3 , 4 , 5 ].…”

Congenital isolated idiopathic episcleral arteriovenous malformation

“…Secondly, it would be prudent to keep in mind the cause-effect relationship of episcleral venous congestion. Literature has numerous reports of bilateral idiopathic dilated episcleral venous congestion leading to bilateral glaucoma 5 , 6 , 7 as well as unilateral idiopathic dilated episcleral venous congestion leading to ipsilateral glaucoma. 8 , 9 Groh MJ et al 10 even reported a case of the Radius-Maumenee syndrome.…”

Tissue-Engineered Models for Glaucoma Research

“…Although there is AH fluid and ion exchange in cornea, iris and vitreoretinal interface, no significant net fluid movement is found. In the trabecular pathway, also known as the direct or conventional outflow pathway, AH flows through the multilayered TM and the inner wall of SC into the lumen of SC, where it will pass through collector channels and reenter systemic circulation via the episcleral venous plexus and the aqueous veins of Ascher [30] (Figure 2). AH in the uveoscleral pathway, also known as the indirect or unconventional outflow pathway, is taken up by the iris root and flows through the uveal meshwork, the anterior face of ciliary muscle and the connective tissues between the muscle bundles.…”