Intravitreal Triamcinolone in Coats' Disease

Abstract

Coats' disease is a nonhereditary, usually progressive retinal vascular anomaly that predominantly affects young men and is typically unilateral.1Shields J.A. Shields C.L. Honavar S.G. Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture.Am J Ophthalmol. 2001; 131: 561-571Abstract Full Text Full Text PDF PubMed Scopus (244) Google Scholar, 2Shields J.A. Shields C.L. Honavar S.G. et al.Classification and management of Coats disease: the 2000 Proctor Lecture.Am J Ophthalmol. 2001; 131: 572-583Abstract Full Text Full Text PDF PubMed Scopus (267) Google Scholar The hallmark is retinal telangiectasias with aneurysmal dilatations. Intra- or sub-retinal exudation may be present but vitreoretinal traction is typically absent. Untreated disease usually progresses to massive subretinal exudation (MSRE) and total bullous exudative retinal detachment (TBERD) which can lead to visual loss and/or painful neovascular glaucoma (NVG) and potential enucleation of the affected eye.1Shields J.A. Shields C.L. Honavar S.G. Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture.Am J Ophthalmol. 2001; 131: 561-571Abstract Full Text Full Text PDF PubMed Scopus (244) Google Scholar, 2Shields J.A. Shields C.L. Honavar S.G. et al.Classification and management of Coats disease: the 2000 Proctor Lecture.Am J Ophthalmol. 2001; 131: 572-583Abstract Full Text Full Text PDF PubMed Scopus (267) Google Scholar, 3Silodor S.W. Augsburger J.J. Shields J.A. Tasman W. Natural history and management of advanced Coats' disease.Ophthalmic Surg. 1988; 19: 89-93PubMed Google Scholar Laser or cryotherapy (ablative therapy) of the retinal telangiectasias is the gold standard of treatment; however, MSRE may be prohibitive. Coats' disease is classified into 5 stages depending on the severity at presentation.2Shields J.A. Shields C.L. Honavar S.G. et al.Classification and management of Coats disease: the 2000 Proctor Lecture.Am J Ophthalmol. 2001; 131: 572-583Abstract Full Text Full Text PDF PubMed Scopus (267) Google Scholar Eyes with advanced disease, stages 3B (TBERD) and 4 (TBERD with NVG) are common, the most challenging to treat, and at highest risk for enucleation.2Shields J.A. Shields C.L. Honavar S.G. et al.Classification and management of Coats disease: the 2000 Proctor Lecture.Am J Ophthalmol. 2001; 131: 572-583Abstract Full Text Full Text PDF PubMed Scopus (267) Google Scholar Consequently, many physicians elect to observe such eyes as long as the eye is comfortable.1Shields J.A. Shields C.L. Honavar S.G. Demirci H. Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture.Am J Ophthalmol. 2001; 131: 561-571Abstract Full Text Full Text PDF PubMed Scopus (244) Google Scholar, 2Shields J.A. Shields C.L. Honavar S.G. et al.Classification and management of Coats disease: the 2000 Proctor Lecture.Am J Ophthalmol. 2001; 131: 572-583Abstract Full Text Full Text PDF PubMed Scopus (267) Google Scholar However, Sildor et al3Silodor S.W. Augsburger J.J. Shields J.A. Tasman W. Natural history and management of advanced Coats' disease.Ophthalmic Surg. 1988; 19: 89-93PubMed Google Scholar recommended treating such eyes to preserve the globe.3Silodor S.W. Augsburger J.J. Shields J.A. Tasman W. Natural history and management of advanced Coats' disease.Ophthalmic Surg. 1988; 19: 89-93PubMed Google Scholar But treatment is complex and hazardous in that aggressive external or internal subretinal fluid (SRF) drainage procedures are required to allow ablative therapy. We report 4 such eyes (Table 1, available at http://aaojournal.org) that consecutively presented within a 6-months period, and that were managed without surgical drainage. Almost total resolution of SRF was achieved within 4 weeks following a single intravitreal triamcinolone injection (IVTA), allowing for prompt application of ablative therapy using an indirect laser ophthalmoscope. In 2 eyes with a significantly limited space between the lens and the detached retina, the injection was performed under ultrasound guidance to avoid damage to the crystalline lens and retina. All eyes had their retina attached at last follow up (Figure 1, Figure 2, Figure 3, Figure 4, available at http://aaojournal.org), but none experienced a significant improvement in visual function, likely because of the TBERD and submacular lipid deposition associated with all eyes, as well as the NVG with one (case 1). No treatment-related short-term complications were noted. The response noted in these cases is probably a therapeutic effect of IVTA rather than a natural outcome of advanced Coats' disease, which typically progresses to NVG.2Shields J.A. Shields C.L. Honavar S.G. et al.Classification and management of Coats disease: the 2000 Proctor Lecture.Am J Ophthalmol. 2001; 131: 572-583Abstract Full Text Full Text PDF PubMed Scopus (267) Google Scholar, 3Silodor S.W. Augsburger J.J. Shields J.A. Tasman W. Natural history and management of advanced Coats' disease.Ophthalmic Surg. 1988; 19: 89-93PubMed Google Scholar IVTA was recently reported as an adjunctive therapy in Coats' disease; however, the results are conflicting. Bergstrom et al4Bergstrom C.S. Hubbard 3rd., G.B. Combination intravitreal triamcinolone injection and cryotherapy for exudative retinal detachments in severe Coats disease.Retina. 2008; 28: S33-S37Crossref PubMed Scopus (34) Google Scholar treated 5 men with TBERD using 4 mg IVTA followed by cryotherapy within 4 months. While an initial good response to IVTA was observed, a high rate of posttreatment complications was noted including inoperable rhegmatogenous retinal detachment (RRD) with severe proliferative vitreoretinopathy (PVR) in 3/5 eyes. Although the mechanism of RRD with PVR was unclear, cryotherapy was implicated and the authors recommend against combining IVTA with cryotherapy. We did not observe RRD following treatment possibly because cryotherapy was not used. Recently, Othman et al5Othman I.S. Moussa M. Bouhaimed M. Management of lipid exudates in Coats disease by adjuvant intravitreal triamcinolone: effects and complications.Br J Ophthalmol. 2010; 94: 606-610Crossref PubMed Scopus (35) Google Scholar reported 15 cases that responded well to 4 mg IVTA “adjuvant” therapy. However, only 8 cases had TBERD, and 6 of these underwent simultaneous surgical drainage, thus limiting the ability to assess the therapeutic effect attributable to IVTA alone. In contrast, we describe IVTA as a single alternative, rather than “adjuvant,” to surgical drainage. Besides reporting the use of 1 mg IVTA in advanced Coats' disease, this study has interesting implications. We observed a rapid response and possibly a narrow therapeutic window to IVTA. Three cases (cases 1, 2, and 3) had an increase in SRF by the time laser therapy was performed following IVTA. Therefore, we suggest close monitoring of SRF and prompt application of ablative therapy within 4 weeks following injection. Additionally, none of the 3 cases with diffuse telangiectasias developed ablatio fugax despite a single-session full laser treatment, suggesting that triamcinolone may be protective through its anti-inflammatory and anti-vascular permeability properties. This may impact the traditional approach of treating only 2 retinal quadrants at a time to avoid ablatio fugax.2Shields J.A. Shields C.L. Honavar S.G. et al.Classification and management of Coats disease: the 2000 Proctor Lecture.Am J Ophthalmol. 2001; 131: 572-583Abstract Full Text Full Text PDF PubMed Scopus (267) Google Scholar Lastly, the rapid SRF resolution noted allowing for prompt laser application without surgical drainage may lead to a paradigm shift in the management of advanced Coats' disease. If only a relatively simple initial intervention of IVTA injection proves to be a viable alternative to surgical drainage in larger studies, many physicians who traditionally observed such eyes may then elect to treat in an effort to salvage the globe.3Silodor S.W. Augsburger J.J. Shields J.A. Tasman W. Natural history and management of advanced Coats' disease.Ophthalmic Surg. 1988; 19: 89-93PubMed Google Scholar Table 1Patient CharacteristicsPatient Age(years)/genderDisease Stage/eyeInitial VFDisease Severity/Location Extent of RDIVTA DoseOutcome After IVTAAblative Therapy/Interval Post IVTAFinal OutcomeFollow UpCase 1.5/M4/OSNLPDiffuse/peripheral1 mgFlat retina in 2 weeksLIO/4 weeksFlat retina, Dense SRHE, NLP, IOP controlled4 monthsTotal bullous RDCase 2.2/F3B/OSNo Fix or FollowTemporal/peripheral1 mgSignificant resolution of SRF in 4 weeksLIO/6 weeksFlat retina, Temporal fold, Dense SRHE, Fix and follow4 monthsTotal bullous RDCase 3.3/M3B/OSUnable to assessDiffuse/peripheral4 mg, twiceFlat retina in 3 weeks, Mild SRF inferiorlyLIO/13 and 17 weeks, 10 months laterFlat retina except for shallow inferior RD, flat macula, possible LP18 monthsTotal bullous RDCase 4.3/M3B/OSNo Fix or followDiffuse/peripheral1 mgAlmost total resolution of SRF in 4 weeksLIO at same time of IVTASignificant resolution, NLP1 monthTotal RDF = female; IOP = intraocular pressure; IVTA = intravitreal triamcinolone; LIO = laser indirect ophthalmoscopy; M = male; NLP = no light perception; OD = right eye; OS = left eye; OU = each eye; RD = retinal detachment; SRF = subretinal fluid; SRHE = subretinal hard exudates; VF = visual function. Open table in a new tab Figure 2RetCam fundus photograph of the left eye of case 1 at final follow up, 4 months following 1 mg of intravitreal triamcinolone and subsequent ablative laser therapy. The retina is completely reattached with dense subretinal hard exudates involving the macula. Residual white triamcinolone crystals are still seen in the vitreous cavity.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3The left eye of case 2 at presentation (A-C) and 4 weeks after 1 mg of intravitreal triamcinolone (IVTA) (D). RetCam anterior segment (A) and fundus photographs (B) at presentation disclose total exudative retinal detachment almost touching the crystalline lens with diffuse, dense, yellowish, subretinal exudates involving the macular area. Note the extensive vascular telangiectasias with aneurysmal light bulb dilatations in the temporal retinal periphery where a thick retinal fold is also present. Ultrasonography at presentation (C) discloses a total, bullous, funnel-shaped retinal detachment with subretinal opacities and no calcification. Also note that the detached retina is thickened and is almost touching the crystalline lens. Ultrasonography 4 weeks after IVTA discloses significant resolution of the subretinal fluid with residual subretinal opacities (D).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 4RetCam fundus photograph of the left eye of case 2 at final follow up, 4 months following 1 mg of intravitreal triamcinolone and subsequent ablative laser therapy. The retina is completely reattached with dense subretinal hard exudates involving the macula. Also note the laser burns in the superotemporal periphery and also on the residual retinal fold in the inferotemporal periphery.View Large Image Figure ViewerDownload Hi-res image Download (PPT) F = female; IOP = intraocular pressure; IVTA = intravitreal triamcinolone; LIO = laser indirect ophthalmoscopy; M = male; NLP = no light perception; OD = right eye; OS = left eye; OU = each eye; RD = retinal detachment; SRF = subretinal fluid; SRHE = subretinal hard exudates; VF = visual function.