Coats disease masquerading as acute posterior uveitis in a young adult

The Kaohsiung Journal of Medical SciencesVolume 38, Issue 2 p. 178-179 CORRESPONDENCEOpen Access Coats disease masquerading as acute posterior uveitis in a young adult Daniel Yu Lee, Daniel Yu Lee orcid.org/0000-0003-0449-6018 Department of Ophthalmology, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanSearch for more papers by this authorShih-Chou Chen, Shih-Chou Chen Department of Ophthalmology, Kaohsiung Veterans General Hospital, Kaohsiung, TaiwanSearch for more papers by this authorShwu-Jiuan Sheu, Corresponding Author Shwu-Jiuan Sheu sjiuansheu@gmail.com Department of Ophthalmology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Correspondence Shwu-Jiuan Sheu, School of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan. Email: sjiuansheu@gmail.comSearch for more papers by this author Daniel Yu Lee, Daniel Yu Lee orcid.org/0000-0003-0449-6018 Department of Ophthalmology, Kaohsiung Medical University Hospital, Kaohsiung, TaiwanSearch for more papers by this authorShih-Chou Chen, Shih-Chou Chen Department of Ophthalmology, Kaohsiung Veterans General Hospital, Kaohsiung, TaiwanSearch for more papers by this authorShwu-Jiuan Sheu, Corresponding Author Shwu-Jiuan Sheu sjiuansheu@gmail.com Department of Ophthalmology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Correspondence Shwu-Jiuan Sheu, School of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan. Email: sjiuansheu@gmail.comSearch for more papers by this author First published: 16 September 2021 https://doi.org/10.1002/kjm2.12451AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Dear Editor, Coats disease is a rare idiopathic telangiectatic neovascular disease of the retina that classically affects unilateral eye of young males. The definite diagnosis of Coats disease can be challenging especially in its advanced stage, as it could mimic other severe ophthalmic conditions, such as retinoblastoma.1 The natural history of Coats disease can progress to blindness and even bulbi atrophy if not treated properly.2 Thus, early diagnosis and appropriate treatment are important. Here, we report a case of Coats disease, who presented initially as acute posterior uveitis with vitreous hemorrhage. A 28-year-old Vietnamese man without any systemic disease was referred to our clinic for refractory posterior uveitis of his left eye for 6 months. Tracing back his history, he first visited other medical center for progressive blurred vision with photopsia and floater in his left eye. The initial vision at disease onset was "counting fingers at 20cm" in the left eye, and 20/20 in the right eye. The intraocular pressure was within normal limit in both eyes. Slit lamp examination revealed no specific findings in the anterior segment of both eyes. Right eye fundus was unremarkable. Indirect fundoscopy of the left eye revealed vitreous hemorrhage (Figure 1A), and suspected lipid exudates or retinitis patch along retinal vessels (Figure 1B). Under the impression of acute unilateral posterior uveitis with vitreous hemorrhage, routine surveillance of infectious and inflammatory uveitis was arranged. Laboratory studies reported normal except positive HSV-1 IgG and cytomegalovirus (CMV) IgG. On the other hand, follow-up fundus exam showed a suspected retinitis patch at temporal periphery. Under the impression of suspected acute retinal necrosis, oral valacyclovir was prescribed. However, retinitis persisted with newly developed exudative retinal detachment even after 3 months of antiviral treatment. Therefore, the treatment was switched to oral valganciclovir under the impression of suspected CMV infection, which turned out to be no help. Hence, the patient was referred to us. On the day of initial visit, the vision and ophthalmic examinations of right eye were normal. Left eye had vision of 6/10 with some residual vitreous opacity. Fundus exam revealed profuse lipid exudates along vessels with groups of telangiectatic vessels (Figure 1C). Moreover, fundus angiography showed typical light bulb dilations of the leaking telangiectatic vessels along with nonperfusion area at temporal and inferior periphery (Figure 1D). With the suspicion of Coats disease, the anti-viral agent was discontinued and three intravitreal injection of Ranibizumab followed by focal retinal laser photocoagulation around the telangiectatic vessels were done. Anterior retinal cryopexy with subtenon triamcinolone (40 mg) was supplemented for residual macular edema. Pars plana vitrectomy was also done to remove the subsequent epiretinal membrane. At the visit of 1-year follow up, the vision restored to 20/25 without macular edema. In this case, the initial vitreous opacity hinders the typical presentations of Coat's disease and masquerading as viral retinitis. Re-evaluation of the ocular features and further differential diagnosis is necessary in case of refractory uveitis. FIGURE 1Open in figure viewerPowerPoint Fundus image of the patient. (A) Initial fundus photography showing marked vitreous opacity and vitreous haze. (B) Initial fundus photography showing suspected exudative lipid deposition or retinitis along vessels. (C) Follow-up fundus photography showing profuse lipid exudates along vessels with groups of telangiectatic vessels. (D) Fluorescein angiography showing marked telangiectatic vessels, nonperfusion area (red arrowhead) and "light-bulb" aneurysms (blue arrowhead) REFERENCES 1Shields JA, Shields CL, Honavar SG, Demirci H. Clinical variations and complications of Coats disease in 150 cases: The 2000 Sanford Gifford memorial lecture. Am J Ophthalmol. 2001; 131(5): 561– 71. CrossrefCASPubMedWeb of Science®Google Scholar 2Daruich A, Matet A, Munier FL. Younger age at presentation in children with coats disease is associated with more advanced stage and worse visual prognosis: A retrospective study. Retina. 2018; 38(11): 2239– 46. CrossrefPubMedWeb of Science®Google Scholar Volume38, Issue2February 2022Pages 178-179 FiguresReferencesRelatedInformation