Adjunctive Intravitreal Triamcinolone Acetonide for Exudative Retinal Detachment in Coats Disease

Abstract

Purpose:

This work aims to assess the value of intravitreal triamcinolone acetonide (IVTA) as an adjunctive therapy in advanced Coats disease with exudative retinal detachment (ERD).

Methods:

A retrospective review was conducted of patients with Coats disease stage 3 or higher who received IVTA to decrease subretinal fluid (SRF), facilitate retinal ablative therapy, and avoid surgical drainage. Primary outcomes were SRF resolution and avoidance of surgical SRF drainage.

Results:

Seventeen eyes of 17 patients (mean, [SD] age, 3.9 [3.4] years) met the inclusion criteria. ERD configuration was bullous in 7 and shallow in 10 eyes. Following a single IVTA injection, ablative therapy was achieved after a mean (SD) of 2.1 (3.0) weeks. Complete SRF resolution was observed in 13 eyes (76.4%) after a mean of 1.3 IVTA injections and a mean of 2 (SD, 1.27) laser sessions, and none of these eyes required SRF drainage up to last follow-up (mean [SD], 50.5 [26.24] months). In 4 eyes with bullous ERD at presentation, SRF persisted (P = .015) despite additional measures including surgical drainage. Final visual acuity ranged from 20/100 to no light perception. Cataract developed in 12 of the 17 eyes (70.5%). None developed an increase in intraocular pressure at final follow-up.

Conclusions:

IVTA injection can be a helpful adjunctive modality to address SRF in advanced Coats disease. It may obviate the need to surgically drain SRF to effectively treat the condition, particularly when the ERD is not highly bullous.

Introduction

Coats disease is a progressive, nonfamilial, and nonhereditary developmental retinal vascular abnormality that typically presents unilaterally and predominantly in men. Most patients present during childhood with symptoms including decreased vision, strabismus, or leukocoria, ¹ but the condition may be encountered in older individuals. ^{1 -7} Retinal telangiectasia and aneurysmal dilations are the hallmark of the disease and lead to exudation and subsequent exudative retinal detachment (ERD). ERD represents stage 3 in the 5-stage classification of Coats disease proposed by Shields et al. ¹ When untreated, Coats disease may progress to neovascular glaucoma (NVG) and phthisis bulbi. ^{1 -4,6} Treatment of Coats disease consists primarily of ablating the retinal telangiectatic vessels with laser photocoagulation and/or cryotherapy to prevent and/or reduce fluid exudation and halt disease progression. ^1,4 However, when subretinal exudation is massive or if ERD is present, ablative therapy may become less effective. ^{5,8 -10} Therefore, surgical drainage of subretinal fluid (SRF) prior to definitive ablation has been reported as a treatment option in Coats disease. ^1,4,6

Another widely used agent in adult retinal vascular disease, such as diabetic retinopathy and retinal venous occlusions, is triamcinolone acetonide (TA), which decreases both vascular permeability and inflammation. However, its use in Coats disease has been rarely reported. ^11,12 In previous studies, the use of intravitreal TA (IVTA) in Coats disease showed significant decrease of SRF, suggesting IVTA as a reasonable treatment modality in this condition. ^13,14 Furthermore, 2 authors of the present work described the use of IVTA as an alternative to surgical SRF drainage in 4 eyes with stage 3B and showed successful anatomical outcome in all eyes. ⁴ Consequently, our group has adopted this approach before proceeding with incisional SRF drainage. The purpose of this study is to assess the efficacy and long-term outcomes of adjunctive IVTA therapy to retinal ablation in a larger cohort of patients with stage 3 and 4 Coats disease.

Methods

A retrospective review of medical records was performed for all cases of advanced Coats disease (Shields et al classification stage 3 or higher ¹ ) that received IVTA (Kenalog, Briston-Meyers Squibb) prior to definitive ablative therapy between January 2009 and January 2017. Cases were excluded if they had nonadvanced Coats disease, prior retinal ablative therapy or intravitreal injections, primary SRF surgical drainage, or less than 1 year of follow-up. IVTA was administered using a 27-gauge needle either directly or guided by ultrasonography, if needed, in cases of bullous ERD. The site of injection in relation to the limbus was performed according to age. ¹⁵

Collected data included: (1) patients’ age at presentation, sex, laterality of eye involvement and best corrected visual acuity (BCVA) when available; (2) bullous vs shallow ERD configuration at presentation, as labeled in the medical record by the treating physician and corroborated by a masked independent review of all fundus images by 2 authors (N.G.G. and S.R.N.), and verified further by the maximum SRF height in the supine position on B-scan ultrasonography, where 5 mm was the cutoff point; (3) number and dosage of IVTA injection(s), of which the dose varied from 1 to 4 mg according to the treating physicians’ decision; (4) degree of SRF resolution following the IVTA injections, assessed clinically under general anesthesia (GA) by the treating physician as total, partial or none; (5) type of ablative therapy and number of ablative treatment sessions; and (6) posttreatment follow-up information including BCVA, intraocular pressure (IOP), cataract development, any cause for poor visual outcome, and any surgical intervention.

Statistical Analysis

Data were analyzed using SPSS version 20.0 (IBM Inc). Descriptive analysis was primarily conducted. The χ² test was used to compare proportions between groups, and the Wilcoxon test was used to compare the IOP before and after intervention. Any output with a P value of .05 or less was interpreted as an indicator of statistical significance.

Results

Baseline Characteristics

A total of 32 patients (32 eyes) presented with Coats disease from January 2009 to January 2017. Fifteen eyes were excluded: 9 with nonadvanced disease (< stage 3) treated solely with retinal laser ablation, and 6 eyes that underwent primary SRF drainage. Seventeen treatment-naive eyes of 17 patients met the inclusion criteria for the study (Figure 1). Three cases have been previously reported ⁴ (cases 13, 14, and 17) (Table 1).

Figure 1.

Patient flowchart. Beva indicates, bevacizumab; Crx, cryotherapy; ERD, exudative retinal detachment; Endo-Lx, endolaser; Ext Drain, external drainage; incl., included; IVTA, intravitreal triamcinolone acetonide; Lx, laser; NVG, neovascular glaucoma; PPV, pars plana vitrectomy; SRF, subretinal fluid; TA, triamcinolone acetonide.

Table 1.

Patients’ Characteristics, Clinical Course, and Outcomes.

Case No. and ERD type	Sex, eye	Age at diagnosis	VA at diagnosis	Stage	Subfoveal nodule at presentation	Agents and No. of injections (dose in mg)	No. of ablation sessions	Time to full SRF resolution, wk	Length of F/U, mo	VA at last F/U	Structural outcome at last F/U	Complications
1-S	M OD	13 mo	F + F	3A1	No	IVTA (4)	Laser ×4	8	24	HM	Flat retina	None
2-S	M OS	20 mo	F + F	3B	Yes	IVTA (4)	Laser ×2	4	54	CF	Flat retina SF fibrotic nodulea	Cataract (operated)
3-S	M OS	34 mo	F + F	3A1	Yes	IVTA (4) IVTA (4) BEVA (1.25)	Laser ×3	3	45	20/300	Flat retina SF fibrotic nodulea	Cataract (operated)
4-S	M OS	8 y 9 mo	4/200	3A2	No	IVTA (2)	Laser ×1	8	16	20/300	Flat retina EF fibrotic nodule	None
5-S	M OS	20 mo	F + F	3A1	No	IVTA (2)	Laser ×3	7	22	F + F	Flat retina	None
6-S	F OD	28 mo	NA	3A1	No	IVTA (4) IVTA (2)	Laser ×4	12	42	20/100	Flat retina EF fibrotic nodule	Cataract
7-S	M OS	4 mo	NA	3A2	No	IVTA (4)	Laser ×1	4	84	LP	Flat retina Macular preretinal fibrosis	Cataract (operated)
8-S	M OS	5 y 10 mo	20/160	3A1	Yes	IVTA (2)	Laser ×1	4	36	20/300	Flat retina SF fibrotic nodulea	None
9-S	F OD	14 y 2 mo	CF	3B	No	IVTA (1)	Laser ×1	7	72	LP	Flat retina SF fibrotic nodule Extramacular  preretinal fibrosis	Cataract
10-S	F OD	18 mo	F + F	3A1	No	IVTA (1)	Laser ×2	4	48	20/400	Flat retina EF nodule	Cataract (operated)
11-Bb	F OS	7 y 3 mo	NLP	3B	No	IVTA (4)	Laser ×1 (Endolaser)	–	35	NLP	Persistent SRF	Cataract
12-B	M OD	16 mo	F + F	3B	No	IVTA (2) IVTA (2) IVTA (2) IVTA (1) BEVA (1.25) BEVA (1.25)	Laser ×4	12	33	F + F	Flat retina SF fibrotic nodule Extramacular  preretinal fibrosis and tractional fold	Cataract (operated)
13-Bc	M OS	3 y 4 mo	NA	3B	No	IVTA (4) IVTA (4)	Laser ×3	–	84	NLP	Persistent SRF	Cataract Corneal opacity
14-Bc	F OS	23 mo	NA	3B	No	IVTA (1)	Laser ×1	3	57	LP	Flat retina EF fibrotic nodule	None
15-Bb	M OS	28 mo	F + F	3B	No	IVTA (1)	Laser ×1	–	68	LP	Persistent SRF	Cataract
16-B	F OD	4 y 4 mo	NLP	3B	No	IVTA (2)	0	–	26	NLP	Persistent SRF	Cataract
17-Bc	M OS	5 y	NLP	4 (NVG)	No	IVTA (1) BEVA (1.25)	Laser ×2	2	112	NLP	Flat retina	Cataract Persistent glaucoma

Abbreviations: B, bullous; BEVA, bevacizumab; CF, counting fingers; EF, extrafoveal; ERD, exudative retinal detachment; F, female; F + F, fix and follow; F/U, follow-up; HM, hand motion; IVTA, intravitreal triamcinolone; LP, light perception; M, male; NA, not assessed; NLP, no light perception; NVG, neovascular glaucoma; OD, right eye; OS, left eye; S, shallow; SF, subfoveal; SRF, subretinal fluid; VA, visual acuity.

^a  Since presentation.

^b  Surgical drainage.

^c Previously reported.

Patients’ age ranged from 4 months to 14 years (mean, 3.9 years). Eleven patients were men (64.7%). Presenting symptoms included strabismus in 9 eyes (52.94%), leukocoria in 6 eyes (35.29%), or both in 2 eyes (11.76%). Snellen BCVA was measurable in 6 patients and ranged between 20/160 and no light perception (NLP). Seven patients could fixate and follow with the affected eye. In 4 children, vision assessment was not possible because of limited cooperation. IOP ranged from 11 to 24 mm Hg (mean, 16.5 mm Hg) with the exception of 1 case (case 17), who had NVG and an IOP of 42 mm Hg. ERD configuration was shallow in 10 eyes (58.8%) (Figure 2) and bullous in 7 eyes (41.2%) (Figure 3). Three eyes had a subfoveal gliotic nodule at presentation (cases 2, 3, and 8). IVTA injections were performed under GA in all cases. Ablative therapy was delivered at a mean of 2.1 weeks (SD, 3.0 weeks) after the primary IVTA injection. All patients were reevaluated under GA at 2 to 10 weeks (mean, 5.6 weeks) after the primary IVTA injection.

Figure 2.

Patient 2. (A) Fundus photograph of the left eye at presentation showing extensive lipid exudation involving the macula. Shallow exudative retinal detachment involving the inferior half of the retina was present (not shown). (B) Fundus photograph at 6 months after intravitreal triamcinolone injection and ablative therapy showing fewer exudates and a subfoveal nodule. (C) Ultra-widefield fundus photograph at last follow-up showing decreased exudation and attached retina with the subfoveal gliotic nodule.

Figure 3.

Patient 12. (A) Fundus photograph of the right eye at presentation showing bullous exudative retinal detachment. (B) Fundus photograph 6 months following treatment showing marked reduction in the amount of posterior pole exudates. (C) Fundus photograph at last follow-up depicting attached retina posterior to a circumferential tractional retinal fold in the temporal periphery.

Eyes With Shallow Exudative Retinal Detachment

Of the 10 eyes with shallow ERD, 2 received 1 mg, 3 received 2 mg, and 5 received 4 mg of IVTA as the initial dose. Eight eyes (80%) received concomitant retinal laser ablation. At the first follow-up evaluation (mean [SD], 5.3 [1.9] weeks), 9 eyes (90%) showed complete SRF resolution. Four of them required no additional laser ablation, whereas 5 required 1 to 3 additional laser sessions (a mean total of 2.2 sessions). In 1 case, although SRF had resorbed, a second injection of IVTA (4 mg) combined with bevacizumab was administered by the treating physician solely to minimize the residual subfoveal exudates. Only 1 eye in the group with shallow ERD (case 6, 10%) showed no response to the initial injection of 4 mg of IVTA. It responded, however, to a second IVTA injection of 2 mg and required a total of 4 laser ablation sessions. In all eyes, the retina remained attached until final follow-up (mean, 44.3 months).

Eyes With Bullous Exudative Retinal Detachment

Of the 7 eyes with bullous ERD, 3 received 1 mg, 2 received 2 mg, and 2 received 4 mg of IVTA as an initial dose. None of the eyes received concomitant laser ablation. At the first follow-up evaluation (mean [SD], 5.8 [3.25] weeks), 1 eye showed total and 3 eyes showed partial SRF resolution (4 of 7, 57%). In the case with total resolution (case 17, which had NVG), a concomitant intravitreal injection of 1.25 mg of bevacizumab had also been given to address iris neovascularization. Among the 3 eyes with partial SRF resolution, 1 eye (case 14) responded after laser ablation and the retina stayed attached up to the final follow-up; 1 eye (case 12) required 3 additional injections of 4 mg of IVTA and 2 bevacizumab injections to decrease subfoveal exudates, and it achieved retinal attachment at last follow-up; and 1 eye (case 11) underwent vitrectomy, endodrainage, and endolaser but the retina remained detached until the last follow-up. The mean number of total laser sessions in these 4 eyes was 1.7 (range, 1 to 4).

Three of the 7 eyes with bullous SRF showed no response to the first IVTA injection, and the retina remained detached up to last follow-up despite additional measures. These included a subsequent IVTA injection (4 mg) in addition to 3 laser sessions (case 13), and external SRF drainage combined with laser ablation (case 15). Further treatment was abandoned in case 16 because of lack of response, NLP vision, and poor posterior segment visibility owing to preexisting cataract and posterior synechia. Overall, 3 of 7 eyes (43%) with bullous ERD completely reattached within 2 to 16 weeks and remained attached until the final follow-up (mean, 59.3 months) (see Figure 1).

Anatomic and Visual Outcomes

All patients completed a minimum of 1-year follow-up with a mean duration of 50.5 months (SD, 26.24 months). Thirteen eyes (76.4%) had total retinal reattachment after a mean of 1.3 IVTA injections and a mean of 2 (SD, 1.27) laser sessions (range, 1-4). All laser applications were completed within the first 24 weeks from initiating treatment. Total retinal reattachment occurred between 2 and 24 weeks (mean [SD], 7.6 [5.9] weeks) after the primary IVTA injections, and none of the 13 eyes showed SRF recurrence up to the last follow-up. Persistent SRF at last follow-up was statistically significantly associated with a bullous ERD configuration at presentation (P = .015) while neither the disease stage (P = .058) nor the IVTA dosage (P = .564) were found to influence the final anatomical outcome.

At last follow-up, 9 eyes exhibited subretinal fibrotic nodules: 5 subfoveal (of which 3 existed at presentation) and 4 extrafoveal. Three eyes had preretinal fibrosis (see Table 1).

BCVA at last visit ranged from 20/100 to NLP (see Table 1). Three eyes had NLP vision including the eye with NVG at presentation. All 3 had bullous ERD at presentation. None of the eyes developed a blind, painful eye requiring enucleation.

Complications

Cataract developed in 12 eyes (70.5%) between 3 and 24 months after initiation of treatment. No statistical significance in the IVTA dose was found between eyes that developed cataract and those that did not (P = .162). Five eyes underwent lens aspiration with intraocular lens implantation. In 7 eyes, the cataract was not removed because of poor visual prognosis.

No increase in IOP was observed during follow-up examinations. At last follow-up, mean IOP was 17 mm Hg with no significant differences compared with mean pretreatment IOP (P = .953). Despite regression of iris neovascularization, the eye that presented with NVG continued to have high IOP until last follow-up.

Conclusions

To our knowledge, this was the largest series of eyes with advanced Coats disease, stage 3 and 4, that received IVTA to hasten SRF reabsorption and allow a more efficient ablative therapy. The main study findings included the following: (1) Thirteen of the 17 eyes were successfully managed with IVTA and ablative therapy without requiring surgical drainage; (2) effective retinal ablative therapy was achievable at a mean interval of 2.1 weeks (SD, 3.0 weeks) after the initial IVTA injection; (3) bullous configuration of ERD was associated with a higher likelihood of SRF persistence; (4) cataract developed in a significant proportion (70.5%) of eyes, but no case developed steroid-induced IOP elevation, rhegmatogenous retinal detachment, or proliferative vitreoretinopathy ¹⁶ after a mean follow-up of 50.5 months (SD, 26.24 months); and (5) no eye required enucleation.

Treatment of advanced Coats disease with ERD by laser photocoagulation is technically challenging. Approaches have included external drainage of SRF, with or without vitrectomy or scleral buckle, and the administration of intravitreal antivascular endothelial growth factor (VEGF) agents or TA, all with variable success. ^{10,13,14,16,17}

The use of IVTA has been of particular interest because of its anti-inflammatory effect through the inhibition of prostaglandin and leukotriene synthesis, its antipermeability effect, and its inhibition of multiple growth factors including VEGF. ¹⁸ IVTA in Coats disease has been shown to reduce macular edema, when injected alone or with anti-VEGF therapy. ^19,20 IVTA’s efficacy has also been reported in the management of ERD. ^4,13,14 Bergstrom and Hubbard ¹⁴ reported the combination of IVTA with cryotherapy and showed marked SRF resolution, which allowed cryotherapy in 4 of 5 eyes. Othman and associates, ¹³ in their series of 15 individuals, also showed excellent response in all cases when IVTA was combined with either cryotherapy or laser photocoagulation; eight patients with bullous ERD, however, underwent primary SRF drainage. ¹³

IVTA’s efficacy in Coats disease may be explained by that, at a histopathological level, mononuclear macrophages were found to be abundantly present in the subretinal space in enucleated eyes. ²¹ Another potential advantage of IVTA over anti-VEGF agents is the decreased likelihood of a crunch effect described with the latter. ²² Increased intraocular VEGF levels have been shown in Coats disease and correlated with the extent of retinal detachment. ²³ Several case series have explored the role of anti-VEGF in advanced Coats disease. Initial results were encouraging but concerns remain about the increased risk of fibrotic vitreoretinopathy. Gaillard et al reported 9 eyes with stages 3B and 4 that received ranibizumab in addition to ablative therapy. Although this treatment was successful in 5 eyes, it required an average of 9 ablation sessions, and 5 eyes developed fibrotic vitreoretinopathy. ²⁴ Ramasubramanian and Shields reported a 50% incidence of fibrotic vitreoretinopathy with the use of bevacizumab. ²² Ray et al compared the efficacy of bevacizumab plus ablation with conventional ablation alone and found no advantage in the number of sessions required or the time to full treatment. However, bevacizumab was effective in managing the more severe cases. ²⁵ It seems that in Coats disease, anti-VEGFs have a less dramatic effect than TA, often require multiple treatment sessions, and are associated with increased risk of fibrotic vitreoretinopathy.

Our present study showed that 76.4% of eyes with Coats disease stage 3 or higher that received primary IVTA without or with concomitant laser ablation had total SRF resolution within 2 to 24 weeks, with a mean of 2 (SD, 1.27) laser applications, all of which were completed within 6 months of treatment initiation. Such a relatively rapid resolution of SRF offers several potential advantages, the least of which is the ability to administer the needed laser ablation more rapidly with a minimum number of sessions, which in small children correlates with a lower risk of amblyopia and fewer sessions of GA. For comparison, in a study of 17 patients, of whom 14 had stage 3 Coats disease or higher, treated solely with repetitive infrared (810 nm) diode laser, a mean of 5 laser sessions was required. This reflected a higher need for GA and longer time for SRF resolution. ²⁶ Fourteen eyes in that study achieved complete resolution of exudation and SRF and relatively excellent visual outcomes. ²⁶ However, the median age at presentation was 9 years, which may suggest less severe disease and no amblyopia. ²⁷

In another study of 17 eyes with Coats disease, of which 8 had ERD treated solely with laser, a mean of 2.75 treatment sessions and an average of 12 months were required to achieve full treatment. One eye developed tractional retinal detachment and 12 eyes developed subretinal fibrosis. ²⁸ This may have been partially related to prolonged SRF presence. Although comparison between studies was not feasible, our series suggested a more rapid SRF resolution with TA use. It is noteworthy that Ghazi et al ⁴ suggested a possible narrow therapeutic window for ablative treatment following IVTA injection. They noted early reaccumulation of SRF at the time of ablation and recommended close monitoring and ablative therapy within 4 weeks. In our study, the mean interval between primary IVTA injection and ablative therapy was 2.1 weeks (SD, 3.0 weeks), which may be the optimal timing for this treatment strategy.

Although 8 eyes in our series received concomitant laser treatment with the primary IVTA injection, laser was attempted only in eyes with shallow ERD. Four of these eyes did not require any further ablative therapy, suggesting that laser treatment alone may have been sufficient to resolve the fluid. It is retrospectively difficult to determine the independent contribution of the IVTA and laser treatment; however, the speed at which SRF resolution occurred in those 4 eyes (5.75 [2.06] weeks) suggests that IVTA contributed to a faster recovery.

Repeated laser ablation sessions alone may suffice to effectively halt the exudation and allow resolution of SRF in some cases of advanced Coats disease. ^{20,28 -30} Yet, the adjunctive use of IVTA offers other advantages, some of which were supported by our findings: curtailing the ERD duration, which in turn decreases outer retinal ischemia and possibly the risk of NVG; potentially decreasing subretinal and preretinal fibrosis; ³¹ reducing the risk of laser-induced lens injury; ^28,29 and decreasing the aforementioned need for repeated GA in smaller children despite that many developed steroid-induced cataract that eventually required additional GA for surgery. Rapid SRF resolution also allows effective ablation of areas of retinal nonperfusion, which cannot be achieved on a detached retina. In addition, having steroids on board may reduce the risk of “ablatio fugax” that could result from extensive ablative therapy. ⁴

The natural history of Coats disease includes the development of preretinal and subretinal fibrosis. ^31,32 In one study, preretinal fibrosis was observed in 40% of eyes and was associated with the extent of retinal exudation and the presence and extent of ERD but not with the number of cryotherapy or laser sessions. Extramacular fibrosis had a worse prognosis with 39% and 7% of eyes developing tractional retinal and ciliary body detachments, respectively. ³¹ Rapid resolution of SRF may be desired to decrease the chance of fibrosis although this is not proven. ³¹ In our series preretinal fibrosis was encountered in 3 cases (18%), of which 2 were extramacular.

Subfoveal fibrotic nodule formation, a consequence of chronic lipid exudation and reported in 52.5% of eyes with Coats disease at stages 2B and 3A, ³¹ is another poor prognostic factor for visual recovery. Lipid deposition at the fovea may be temporarily exaggerated following laser ablation of peripheral exudative lesions, particularly when significant SRF is present. SRF resolution before ablation may, at least in theory, decrease this “macular dumping” phenomenon. Because IVTA decreases exudation and permeability, we believe it is ideally suited for this purpose. In our series, subfoveal nodules were observed at last follow-up in 5 eyes (29%), of which 3 were preexistent.

Our study demonstrated that IVTA could be helpful even in bullous ERD, although the success rate may be lower compared with cases with shallow ERD. For our 3 of 7 eyes that showed retinal reattachment, the interval for SRF resolution was within 2 to 16 weeks, shorter than in previous series. ^24,26,28,29 Considering the risks of lens injury if laser is administered when the retina is close to the lens, and the risks associated with surgical drainage of SRF, we would recommend considering IVTA before deciding on surgical drainage.

In our series cataract developed in 70.5% of eyes. Although intravitreal steroid increases the risk of cataract formation, advanced Coats disease alone can lead to cataract development in children. In one study, 28% of children with Coats disease developed cataract. ³³ ERD was an independent risk factor for cataract formation. ³³ In series treated with only laser ablation, cataract developed in 11% to 21% of eyes and occurred in the presence of highly elevated retinal detachment. The mechanism was thought to be related to laser energy’s effect on the lens. ^28,29 Although in our study IVTA reduced the ERD height, which should theoretically lead to protection of the lens from laser injury, the cataract rate was higher, most likely caused by the cataractogenic effect of intravitreal steroids. We found no correlation between doses of IVTA or number of IVTA injections and cataract formation, but the numbers were too small to definitively settle this question. Alternative steroid delivery routes that have a lower risk of cataract formation may have a potential role in advanced Coats disease. ³⁴

Limitations of our study include its retrospective nature, small sample size, and that there were multiple surgeons involved in treatment decisions. These limitations contributed to the lack of IVTA’s dosage uniformity as well as the lack of well-defined criteria for treatment with IVTA and for reinjection. In fact, 6 patients were excluded because of primary drainage, an approach that may have been selected because of unsafe space for needle entry or merely a surgeon’s preference. Furthermore, the concomitant laser therapy performed in some of our cases made it difficult to determine the independent contribution of the TA to the ultimate success of treatment, and the lack of a matched control group limited definitive conclusions as to what constitutes the best treatment strategy. Also, SRF resolution assessment in this study was based on clinical examination and not on precise optical coherence tomography and ultrasonography measurements. Lastly, because King Khaled Eye Specialist Hospital (KKESH) is a referral tertiary eye hospital, the sample may have been skewed to more severe cases. Prospective comparison between IVTA and other management modalities with long-term follow-up would be helpful in determining the best treatment strategy.