Coat’s-like exudation in rhodopsin retinitis pigmentosa: successful treatment with an intravitreal dexamethasone implant

Patil, Lalit; Lotery, Andrew

doi:10.1038/eye.2013.314

Cited by 17 publications

(10 citation statements)

References 8 publications

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“…Searching the literature for suggested management of patients having coat’s like disease and RP revealed multiple management plans including: vitrectomy and endolaser [ 9 ], cryotherapy, scleral buckling with subretinal fluid drainage and laser photocoagulation of the telangiectatic and neovascular lesions [ 10 ] and intravitreal dexamethasone implant [ 11 ].…”

Section: Discussionmentioning

confidence: 99%

Coat’s like vasculopathy in leber congenital amaurosis secondary to homozygous mutations in CRB1: a case report and discussion of the management options

et al. 2016

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BackgroundMutations in the CRB1 gene are associated with variable phenotypes of severe retinal dystrophies, and retinal dystrophies resulting from CRB1 mutations may be accompanied by specific fundus features such as coat’s like vasculopathy in retinitis pigmentosa patients. This is the first report of the occurrence of coat’s like vasculopathy in a patient diagnosed with Leber congenital amaurosis caused by a CRB1 mutation.Case presentationAn 18-year old Syrian female patient presented with bilateral gradual loss of vision since early childhood, with recent deterioration in her left eye. She appeared to have an asymmetric bilateral coat’s like vasculopathy which was more severe in the left eye. The diagnosis of Leber congenital amaurosis was suggested, and a genetic CRB1 sequencing for the patient and her two younger siblings, who also had severe vision loss, was done, upon which the diagnosis of Leber congenital amaurosis associated with exudative retinal detachment due to coat’s like vasculopathy was made. Treatment with panretinal photocoagulation was attempted in the worse left eye, but with no improvement. As the disease suddenly progressed in both eyes, pars plana vitrectomy with endolaser and silicone oil tamponade was performed in the better right eye which led to anatomical stabilization of the case without improvement in the visual acuity.ConclusionLeber congenital amaurosis is reported to be associated with multiple systemic and ocular findings, none of which is coat’s like vasculopathy. CRB1 gene mutations are associated with remarkable retinal findings in patients with retinitis pigmentosa and other fundus dystrophies. In this unique case we are reporting the incidence of coat’s like vasculopathy in a patient diagnosed with Leber congenital amaurosis caused by CRB1 gene mutation, and its management. CRB1 mutant patients should be followed up closely as sudden progression can have permanent poor outcomes and as early management is vital in such cases.

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Section: Discussionmentioning

confidence: 99%

Coat’s like vasculopathy in leber congenital amaurosis secondary to homozygous mutations in CRB1: a case report and discussion of the management options

et al. 2016

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“…The studies reporting on the use of DEX in macular edema related to retinitis pigmentosa consist of case report studies, which include only a few eyes ( Table 7 ). 77 – 80 Nevertheless, an anatomical and functional improvement has been shown, but a relapse of macular edema occurred before 6 months from the implant 77 and an additional DEX was required in some cases. 77 , 78 DEX implant proved to be safe with an IOP rise >21 mmHg recorded in only one eye.…”

Section: Retinitis Pigmentosamentioning

confidence: 99%

Widening use of dexamethasone implant for the treatment of macular edema

Bonfiglio¹,

Reibaldi²,

Fallico³

et al. 2017

DDDT

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Sustained-release intravitreal 0.7 mg dexamethasone (DEX) implant is approved in Europe for the treatment of macular edema related to diabetic retinopathy, branch retinal vein occlusion, central retinal vein occlusion, and non-infectious uveitis. The implant is formulated in a biodegradable copolymer to release the active ingredient within the vitreous chamber for up to 6 months after an intravitreal injection, allowing a prolonged interval of efficacy between injections with a good safety profile. Various other ocular pathologies with inflammatory etiopathogeneses associated with macular edema have been treated by DEX implant, including neovascular age-related macular degeneration, Irvine–Gass syndrome, vasoproliferative retinal tumors, retinal telangiectasia, Coats’ disease, radiation maculopathy, retinitis pigmentosa, and macular edema secondary to scleral buckling and pars plana vitrectomy. We undertook a review to provide a comprehensive collection of all of the diseases that benefit from the use of the sustained-release DEX implant, alone or in combination with concomitant therapies. A MEDLINE search revealed lack of randomized controlled trials related to these indications. Therefore we included and analyzed all available studies (retrospective and prospective, comparative and non-comparative, randomized and nonrandomized, single center and multicenter, and case report). There are reports in the literature of the use of DEX implant across a range of macular edema-related pathologies, with their clinical experience supporting the use of DEX implant on a case-by-case basis with the aim of improving patient outcomes in many macular pathologies. As many of the reported macular pathologies are difficult to treat, a new treatment option that has a beneficial influence on the clinical course of the disease may be useful in clinical practice.

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“… 33 Despite increasing availability of intravitreal agents, management continues to be an ongoing challenge with heterogeneous visual and anatomical outcomes. 34 In our report, disease control was achieved with laser photocoagulation in combination with intravitreal therapy. Interestingly, macular edema in our patient seemed to respond better when treated with triamcinolone than when bevacizumab was used.…”

Section: Discussionmentioning

confidence: 66%

Multimodal treatment of Coats-like exudative vitreoretinopathy in Goldmann-Favre syndrome

Nieves

Villegas

Patel

et al. 2022

American Journal of Ophthalmology Case Reports

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Coat’s-like exudation in rhodopsin retinitis pigmentosa: successful treatment with an intravitreal dexamethasone implant

Cited by 17 publications

References 8 publications

Coat’s like vasculopathy in leber congenital amaurosis secondary to homozygous mutations in CRB1: a case report and discussion of the management options

Coat’s like vasculopathy in leber congenital amaurosis secondary to homozygous mutations in CRB1: a case report and discussion of the management options

Widening use of dexamethasone implant for the treatment of macular edema

Multimodal treatment of Coats-like exudative vitreoretinopathy in Goldmann-Favre syndrome

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