Delhi Journal of Ophthalmology

Microincision vitrectomy for secondary epiretinal membrane in adult-onset Coats’ disease

Manmath Das1, Priyanka Chaudhary2, Abhishek Varshney3
1Consultant Retina Surgeon, K.I.M.S, KIIT Campus, Bhubaneshwar, Odisha, India
2Vitreoretina Services, Maharaja Agarsen Medical College, Agroha, Haryana, India
3Vitreoretina services, C.L. Gupta Eye Institute, Moradabad, Uttar Pradesh, India

Corresponding Author:

Abhishek Varshney MS
Consultant, Vitreoretina
C L Gupta Eye Institute, Ram Ganga Vihar
Phase 2 (Ext.), Moradabad (Uttar Pradesh)
PIN -244001, India
Email id: doctorabhishekvarshney@gmail.com

Received: 16-MAY-2020

Accepted: 16-AUG-2020

Published Online: 24-DEC-2019

DOI:http://dx.doi.org/10.7869/djo.480

Abstract
We report a case of a 20-year-old male who presented with a gradual painless decrease of vision in his left eye. Best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/200 in the left eye. Anterior segment examination was normal for both eyes. Left eye indirect ophthalmoscopy revealed the presence of a thick epiretinal membrane (ERM) along with telangiectatic vessels and subretinal lipid deposits in inferotemporal quadrant which became more obvious on Fluorescein angiography. Spectral-domain optical coherence tomography (SD-OCT) of left macula revealed a thick ERM with tangential contraction. The patient was diagnosed as adult-onset Coats’ disease (Stage 2A) with ERM. The patient underwent standard 25 gauge pars plana vitrectomy with ERM peeling followed by cryotherapy of the telangiectatic vessels. Postoperatively there was a decrease in the amount of subretinal lipid deposition with scarring and obliteration of telangiectatic vessels and BCVA improved to 20/20, N6 which remained stable throughout follow-up.

Keywords :Adult-onset Coats’ disease, Epiretinal Membrane (ERM), Pars plana Vitrectomy, Cryotherapy

Introduction

Coats’ disease is a sporadic, non-hereditary unilateral exudative retinopathy typically occurring in young males. Clinical manifestations include idiopathic retinal telangiectasia with intraretinal and/or subretinal exudation without appreciable retinal or vitreal traction.1 It can result in serious complications including neovascular glaucoma, amblyopia, and anterior segment abnormalities such as anterior chamber cholesterolosis, cataract, persistent subfoveal ?uid or exudates eventually leading to macular ?brosis.
Adult-onset Coats’ disease has less extensive involvement, hemorrhage near larger vascular dilatations, more benign natural course, and a more favorable treatment outcome as against the childhood-onset disease.2
Epiretinal membrane (ERM) is rare in Coats’ disease1,3 but can cause visual impairment in affected patients.4,5,6 It usually develops iatrogenically after excessive treatment (treated coats’ disease) like retinal photocoagulation or cryoretinopexy7 but rarely may arise de novo (untreated Coats’ disease).4,5,6 In later cases, only a few cases of ERM have been published.4,5,6 Till date, only 4 cases of adult-onset treated or untreated Coats’ disease with ERM that underwent successful vitrectomy has been published [4,5,6,8]. We report a case of adult-onset Coat’s disease with an ERM which was successfully treated with 25-gauge pars plana vitrectomy (25G PPV).

Results

A 20 year-old male presented with a 2-week history of gradual and painless diminution of vision in the left eye, there was no metamorphopsia in the affected eye. Bestcorrected visual acuity (BCVA) was 20/20 in the right eye and 20/200 in the left eye. Anterior segment examination of both eyes was normal with an intraocular pressure of 14 mmHg in the right eye and 16 mmHg in the left eye by Goldmann applanation tonometry. Right eye fundus examination was unremarkable. Left eye fundoscopy revealed the presence of a thick ERM along with telangiectatic vessels and subretinal lipid deposits in inferotemporal quadrant (Figure 1A). Spectral-domain optical coherence tomography (SD-OCT) of left macula revealed a thick macular ERM with loss of foveal contour and retinal architectural distortion (Figure 2A). A diagnosis of adult-onset Coats’ disease with ERM was made. The patient underwent standard 25 gauge 3 port pars plana vitrectomy and dye assisted peeling of the ERM was done, followed by cryotherapy of the telangiectatic vessels (double freeze-thaw technique). Intra-operatively, there was no posterior vitreous detachment and was induced during surgery. After doing fluid air exchange, the ERM was negatively stained with brilliant blue G (0.05%) dye for 2 minutes. The ERM was loosely adherent to the retinal surface and no problems were encountered while removing it. The ERM came off in single piece. Air tamponade was given. After about one month, the cryotherapy was repeated and there was a decrease in the amount of subretinal lipid deposition with scarring and obliteration of telangiectatic vessels (Figure 1B). Postoperatively, BCVA in left eye improved to 20/20, N6 which remained stable during the 10 months of follow-up. However, few internal limiting membrane (ILM) folds remained on macula but the traction on the parafoveal area was removed (Figure 2B).


monly presents in adulthood with similar features as in childhood. Diagnosis of Coats’ disease should not be accepted in an adult until other causes of exudative retinopathy are clearly excluded.1 Although ERM in Coats’ disease (both juvenile and adult-onset) is a rare entity, it might cause significant visual acuity loss in these patients.1 Its exact pathogenesis is not known, but it develops secondary to excessive retinal photocoagulation or cryoretinopexy.7 Shields et al in their study of 150 patients with juvenile and adult-onset Coats’ disease found ERM in 2.5 % of treated eyes with poor visual outcomes [1], while Rishi et al reported a higher incidence of ERM (4.4%).3 Our case had no history of any ocular treatment. In untreated cases of adult-onset coats disease, ERM develops after PVD. But in our case, PVD was not present. Machemer suggested that such an ERM is due to chronic leakage from the telangiectatic vessels which leads to reactive glial proliferation on the retinal surface that causes traction on the retina and further accumulation of subretinal fluid.9
Smithen et al reported ERM in 2 out of 13 patients with adult-onset coats disease.2 Mino et al reported a case of adult-onset coat with ERM at the age of 26.5 We suggest that Coats’ disease with an ERM is rare in the early 20s as seen in our case.
Long-standing ERM with coats disease can result in submacular exudation which later can lead to submacular fibrosis and permanent loss of vision, so an early vitrectomy is indicated for better visual outcomes. Shukla et al reported that early ERM with ILM peeling and simultaneous endophotocoagulation before submacular exudation or fibrosis leads to excellent visual outcomes with a final visual acuity of 1 from 0.17 in adult-onset Coats’ disease.4 Mino et al also reported that there is a better visual outcome in adultonset Coats’ disease when vitrectomy is performed earlier on the first appearance of traction. On the other hand, Sugimoto et al reported a case of Coats’ disease with PVD induced spontaneous peeling of ERM after laser photocoagulation.7 Recently, Kumar and Kumar have also reported favorable outcomes after ERM removal and ILM peeling.6
In our case, though ILM peeling was not done, still BCVA improved to 20/20 from 20/200 after early vitrectomy and ERM removal which did not change during the 10 months follow-up period. However, few ILM folds remained on the retinal surface but parafoveal traction was completely eliminated. The patient had a good visual outcome with complete regression of telangiectatic vessels. So for better visual outcomes, we recommend early ERM removal for a thick widespread and firmly attached ERM as spontaneous peeling cannot be expected in such cases.

Conclusions

25 G PPV combined with ERM peeling along with cryoretinotherapy to telangiectatic vessels appears to be an effective treatment for ERM secondary to adult-onset Coats’ disease with good visual outcomes.


References
  1. Shields JA, Shields CL, Honavar SG, Demirci H, Cater J: Classification and management of Coats disease: the 2000 Proctor Lecture. Am J Ophthalmol, 2001;131:572-583.
  2. Smithen LM, Brown GC, Brucker AJ et al: Coats’ disease diagnosed in adulthood. Ophthalmology, 2005; 112:1072-1078.
  3. Rishi P, Rishi E, Uparkar M, Sharma T, Gopal L, Bhende P, et al. Coats’ disease: an Indian perspective. Indian J Ophthalmol, 2010; l58:119-124.
  4. Shukla D, Chakraborty S, Behera UC, Kim R. Vitrectomy for epimacular membrane secondary to adult-onset Coats’ disease. Ophthalmic Surg Lasers Imaging, 2008; 39:239-241.
  5. Mino A, Mitamura Y, Katome T, Semba K, Egawa M, Naito T, et al. Case of adult-onset coats' disease with epiretinal membrane treated with 25-gauge pars plana vitrectomy. J Med Invest, 2015; 62:85-88.
  6. Kumar P, Kumar V. Vitrectomy for epiretinal membrane in adult-onset Coats' disease. Indian J Ophthalmol, 2017;65:1046-8
  7. Sugimoto M, Sasoh M, Ito Y, Miyamura M, Uji Y, Chujo S. A case of Coats’ disease with a peeling of premacular fibrosis after photocoagulation. Acta Ophthalmol Scand, 2002; 80:96-97.
  8. Yadav NK, Vasudha K, Gupta K, Shetty KB. Vitrectomy for epiretinal membrane secondary to treatment for juvenile Coats' disease. Eye (Lond), 2013; 27:278-80.
  9. Machemer R. Epiretinal membrane formation can occur in adult Coats’ disease. Retina, 1996; 16:168-169.

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Das M, Chaudhary P, Varshney AMicroincision vitrectomy for secondary epiretinal membrane in adult-onset Coats’ disease.DJO 2019;30:49-51

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Das M, Chaudhary P, Varshney AMicroincision vitrectomy for secondary epiretinal membrane in adult-onset Coats’ disease.DJO [serial online] 2019[cited 2020 Jan 27];30:49-51. Available from: http://www.djo.org.in/articles/30/1/Microincision-vitrectomy-for-secondary-epiretinal-membrane-in-adult-onset-Coats-disease.html