|Year : 2021 | Volume
| Issue : 3 | Page : 506-508
Familial exudative vitreoretinopathy presenting as bilateral secondary angle-closure
Gaurav Gupta, Raghulnadhan Ramanadhane, Deepika Dhingra, Surinder Singh Pandav, Sushmita Kaushik, Faisal Thattaruthody, Mohit Dogra
Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India
|Date of Submission||27-Jun-2020|
|Date of Acceptance||09-Feb-2021|
|Date of Web Publication||02-Jul-2021|
Dr. Mohit Dogra
Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
Source of Support: None, Conflict of Interest: None
A 24-year-old man presented with sudden-onset bilateral simultaneous angle closure with intraocular pressure (IOP) of 30 and 52 mm Hg in the right and left eye, respectively. Ultrasound biomicroscopy detected the presence of bilateral supraciliary fluid, whereas fundus appearance was suggestive of familial exudative vitreoretinopathy (FEVR). He was managed with cycloplegics, topical steroids, and anti-glaucoma medications (AGM) followed by cryotherapy to peripheral leaking retinal vessels of both eyes, once IOP was controlled and supraciliary fluid resolved. Any patient with bilateral simultaneous angle closure must alert one to look beyond the diagnosis of primary angle closure and search for secondary causes.
Keywords: Bilateral acute angle closure, familial exudative vitreoretinopathy, secondary angle closure, supraciliary effusion
|How to cite this article:|
Gupta G, Ramanadhane R, Dhingra D, Pandav SS, Kaushik S, Thattaruthody F, Dogra M. Familial exudative vitreoretinopathy presenting as bilateral secondary angle-closure. Indian J Ophthalmol Case Rep 2021;1:506-8
|How to cite this URL:|
Gupta G, Ramanadhane R, Dhingra D, Pandav SS, Kaushik S, Thattaruthody F, Dogra M. Familial exudative vitreoretinopathy presenting as bilateral secondary angle-closure. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:506-8. Available from: https://www.ijoreports.in/text.asp?2021/1/3/506/319989
Acute angle-closure (AAC) very seldom presents simultaneously in both eyes as part of primary angle-closure disease. Bilateral AAC is almost always secondary to systemic/ocular conditions, namely: topical mydriatic use in predisposed individuals, anticholinergic drugs, anticonvulsants like topiramate, following general anesthesia, and spherophakia.,,
Familial exudative vitreoretinopathy (FEVR) is a bilateral heritable vitreoretinopathy characterized by avascular peripheral retina leading to neovascularization, exudation, hemorrhage, and retinal detachment. We report a patient who presented with bilateral simultaneous AAC and was subsequently diagnosed as FEVR. To the best of our knowledge, this is the first reported case of FEVR presenting as bilateral AAC.
| Case Report|| |
A 24-year-old man presented with a 1-month history of blurred vision in both eyes (BE) along with frontal headache. Blurring of vision was insidious in onset and progressively increasing. His best-corrected visual acuity (BCVA) was 20/120 and 20/80 in the right eye (RE) and left eye (LE), respectively. Intraocular pressure (IOP), on Goldmann applanation tomometry, was 30 mm Hg and 52 mm Hg in RE and LE respectively. Slit-lamp examination revealed mild conjunctival congestion, clear cornea and crystalline lens, uniformly shallow anterior chamber (AC) in BE [Figure 1]a and [Figure 1]b and presence of a left relative afferent pupillary defect. Indentation gonioscopy revealed complete angle closure in all four quadrants of BE [Figure 1]c and [Figure 1]d. Fundus examination of RE showed an obliquely tilted disc with peripapillary atrophy (PPA), cup-disc ratio (CDR) of 0.3, narrow and supero-temporally (ST) dragged vascular arcade and presence of exudation and fibrosis in the super-temporal retinal periphery extending into the pars plana [Figure 2]a. LE had an obliquely tilted disc with infero-nasal (IN) PPA, CDR of 0.7/0.8 and presence of exudation and fibrosis in the IN retinal periphery [Figure 2]b.
|Figure 1: Anterior Segment photograph of Right (a) and Left (b) eye showing shallow anterior chamber; Gonioscopy of right (c) and left (d) eye revealed complete angle closure in all four quadrants|
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|Figure 2: Fundus photograph of right eye (a) showing supero-temporally dragged vascular arcade and presence of exudation and fibrosis in the super-temporal retinal periphery extending into the pars plana. Left eye (b) showing presence of exudation and fibrosis in the inferonasal retinal periphery. Fundus fluorescein angiography of right (c) and left (d) eye showing patchy hyper-fluorescence with late leakage of the corresponding quadrants|
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Father of the patient, though asymptomatic, had peripheral retinal exudation and fibrosis in the same quadrants as the patient. Fluorescein angiography of BE showed early patchy hyper-fluorescence with late leakage of the ST and IN quadrants of the RE and LE [Figure 2]c and [Figure 2]d. Ultrasound biomicroscopy (UBM) revealed the presence of bilateral supraciliary effusion, more in the nasal quadrant in the LE and temporal quadrant in the RE [Figure 3]a and [Figure 3]b. Diagnosis of FEVR stage 3B with bilateral AAC with supraciliary effusion was made and the patient was started on 40 mg prednisolone, tablet acetazolamide, betamethasone, and atropine eyedrops along with brimonidine + timolol fixed drug combination and brinzolamide eye drops. At 1-month follow-up, IOP improved to 24 and 34 mm Hg in RE and LE respectively, AC depth improved but still shallow, and gonioscopy revealed narrow angles. Steroid tapering was started but anti-glaucoma medications were continued. Over the following two months, the patient's symptoms improved, AC depth increased, IOP normalized (on topical drugs) (16 and 20 in RE and LE respectively), angles opened on gonioscopy and supraciliary effusion decreased. Oral steroids were tapered and acetazolamide was stopped. At this juncture, cryotherapy was applied to the ST and IN quadrants of the RE and LE, respectively, to manage the exudation secondary to FEVR. At one month follow-up post cryotherapy, IOP was 16 and 18 in RE and LE respectively, angle remained open on gonioscopy, UBM showed minimal supraciliary fluid. Tapering of topical anti-glaucoma medication was started. At 3 months follow up, IOP remained normal, AC depth was normal with open angle on gonioscopy and UBM showed no supraciliary fluid. So, tapering of topical anti-glaucoma medications was continued. At one year of follow-up, the patient had a BCVA of 20/80 in BE, IOP of 16 mm Hg and 10 mm Hg in RE and LE respectively (off treatment), normal AC depth, and absence of supraciliary effusion on UBM.
|Figure 3: Ultrasound biomicroscopy of right (a) and left (b) eye showing presence of supraciliary effusion (yellow arrows)|
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| Discussion|| |
Primary angle closure occurs due to an anatomic configuration of the AC in which there is mechanical blockage of the trabecular meshwork by the peripheral iris., Secondary angle closure (SAC), unlike its primary counterpart, has an underlying causative factor for obstruction of aqueous flow which is usually unrelieved by iridotomy. The treatment of SAC involves use of cycloplegics, steroids and instituting appropriate management of the underlying etiology. One of the mechanisms of SAC is uveal effusion with supraciliary fluid leading to anterior displacement of the lens-iris diaphragm., Our patient had bilateral supraciliary effusion (confirmed on UBM) which led to SAC with subsequent raised IOP in BE.
FEVR is a hereditary dominant exudative vitreoretinopathy that asymmetrically afflicts BE of the patient. Presence of temporal post-equatorial capillary non-perfusion areas leads to peripheral neovascularization, intra- and sub-retinal exudation, vitreous hemorrhage, fibrosis, and tractional/.combined retinal detachment., Although presence of temporal retinal peripheral changes is commonly seen, ST and IN quadrant involvement in FEVR is also seen. Our patient had ST quadrant involvement in the RE and IN quadrant involvement in the LE. UBM confirmed that supraciliary fluid was most pronounced in the ST and IN quadrant in the RE and LE, respectively. We hypothesize that exudation and leakage of fluid, which usually occurs posteriorly in FEVR, happened both posteriorly as well as anteriorly in our patient. The involvement of the pars plana by exudation and fibrosis could be clearly made out on scleral depression of the ST quadrant of the RE and IN quadrant of the LE during indirect ophthalmoscopy. This hypothesis is further strengthened by the fact that the patient had no recurrence of supraciliary effusion after cryotherapy was applied to the afflicted retinal periphery in BE.
This case has multiple points of learning. Firstly, it highlights the importance of detailed fundus examination in a case of bilateral simultaneous AAC. Secondly, patients of bilateral AAC secondary to uveal effusion do not need laser iridotomy but need to be managed with cycloplegics, steroids, and AGM. Thirdly, UBM is an indispensable imaging modality in patients suspected of SAC as it not only helps to detect the mechanism of angle closure but also aids in monitoring response to therapy in these patients.
| Conclusion|| |
Presentation of bilateral simultaneous AAC in a patient must alert one to look beyond the common diagnosis of primary angle closure. FEVR may present with supraciliary effusion with SAC glaucoma. Oral steroids, cycloplegics, and AGM are the mainstay of treatment supplemented by ablation of the peripheral non-perfused retina by cryotherapy or laser photocoagulation.
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
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]