Retina Conference Eddie Apenbrinck, M.D. University of Louisville Department of Ophthalmology and Visual Sciences 6/5/2014

Presentation CC: abnormal fundus exam in a formerly premature infant HPI: 3 month old white male referred by a community ophthalmologist to our Retina Clinic for abnormal dilated fundus exam of the right eye, initially thought to have retinopathy of prematurity (ROP).

History Past Ocular Hx: ROP (?): ROP screenings normal until 3/21/14 when right eye showed signs of ROP stage 3 zone 2, left eye normal Past Medical Hx: prematurity, patent foramen ovale, gastroesophageal reflux Birth History: 30 week gestation preterm male. Pregnancy complicated by pre-ecclampsia and placental abruption. Birth weight: 1170 grams Meds: multivitamin, Prilosec (1.5 mg/kg/day)

Exam OD OS Visual Acuity: Withdraws from light OU IOP: Soft OU EOM: Full OU Anterior Segment : Unremarkable OU

Clinical Course Pt was taken to Kosair Children’s Hospital for exam under anesthesia (EUA) and fluorescein angiography (FA)

RetCam Fundus Photos OD Subretinal yellow discoloration along inferior arcade. Avascular temporal periphery, temporal vascular telangiectasia, tortuosity, and dilation.

RetCam Fundus Photos OS Normal fundus photo

Fluorescein Angiography OD Arterial Phase: peripheral nonperfusion, vascular dilation, tortuosity vessel, and telangiectasia

Fluorescein Angiography OD AV phase: saccular aneurysm and vascular abnormalities

Assessment and Plan 3 month old male referred for abnormal retina exam. Dilated exam OD showed an avascular temporal periphery and vascular abnormalities (dilation, tortuosity, and telangiectasia) DDx: Coats’ Disease ROP FEVR Retinoblastoma Plan: Initiate treatment with panretinal photocoagulation (PRP) and cryotherapy

Treatment Laser photocoagulation and cryotherapy to the temporal periphery and vascular abnormalities. Treatment #1: 665 laser burns, 4 cryotherapy freezes

Clinical Course 2 weeks later, the patient developed an exudative retinal detachment OD Plan for follow up in 3 months

Coats’ Disease First described by George Coats, a Scottish medical student in 1908. Coats’ disease is an idiopathic condition characterized by retinal vascular changes and exudation caused by a defect in retinal vasculature development Increased permeability of abnormal retinal vessels causes leakage into the intraretinal and subretinal spaces.

Coats’ Disease Epidemiology Men (85%) affected more often than women Usually unilateral (80%) Average age at diagnosis 6-8 years, but can occur in infants and adults.

Coats’ Disease Diagnosis Typically diagnosed as a result of the recognition of poor vision, strabismus, or leukocoria Ancillary testing may be useful in ruling out other potential causes of leukocoria in children. FA, CT, MRI, U/S Leukocoria DDX Retinoblastoma (calcifications on imaging)  Retinal-choroidal coloboma  Retinopathy of prematurity  Toxocara infestation  Retinal detachment  Congenital cataract

Coats’ Disease Treatment The major goal of treatment in Coats' disease is to preserve or improve visual acuity or, when this is impossible, to preserve the anatomical integrity of the eye. Treatment generally consists of photocoagulation, cryotherapy, and in severe cases, retinal reattachment surgery Anti-VEGF injections have been successfully used as part of combination therapy in case series but there is a risk of vitreoretinal traction

Exudative Retinal Detachment

Retrospective consecutive series of 150 patients with Coats’ Disease over 25 years. Successful treatment in early stages can be best achieved with laser photocoagulation or cryotherapy. More advanced cases may require surgical techniques of retinal reattachment, combined with photocoagulation or cryotherapy. Carefully selected treatment can anatomically stabilize or improve the eye with Coats disease in 76% of cases.

Classification of Coats’ Disease Shields, JA et al. Classification and Management of Coats Disease: the 2000 Proctor Lecture. Am J Ophthalmology. 2001;31(5):572-583. 

Literature Review Concluded that earlier diagnosis and prompt treatment will result in better visual outcome and fewer enucleations for Coats disease in the future.

Mean follow-up of 8.5 months British Journal of Ophthalmology June 2011 Retrospective case series of 8 patients with Coats’ disease manifesting as partial or complete retinal detachment treated with cryotherapy (n=8) and/or PRP (n=4) plus additional intravitreal bevacizumab (n=8) Mean follow-up of 8.5 months Resolution of retinopathy (8 of 8) Resolution of Coats-related subretinal fluid (8 of 8) Resolution of retinal exudates (6 of 8) Development of retinal fibrosis (4 of 8) 3 of 4 developed traction retinal detachment

References BCSC: Pediatric Ophthalmology and Strabismus. Coats ‘Disease. Pgs 287-288 BCSC: Retina and Vitreous. Coats’ Disease Pgs 143-145 Shields, JA et al. Classification and Management of Coats Disease: the 2000 Proctor Lecture. Am J Ophthalmology. 2001;31(5):572-583.  Kanski, Jack J. Clinical Ophthalmology: A Systematic Approach. 6th ed. Elsevier Ltd., 2007. Ramasubramaniam A, Shields CL. Bevacizumab for Coats' disease with exudative retinal detachment and risk of vitreoretinal traction. Br J Ophthalmol. 2012 Mar;96(3):356-9. Ridley ME, Shields JA, Brown GC, Tasman W. Coats’ disease: evaluation of management. Ophthalmology. 1982;89:1381-1387. Coats G. Forms of retinal disease with massive exudation. Royal London Ophthalmic Hospital Reports.1908;17(3):440-525. Cakir M, Cekiç O, Yilmaz OF. Combined intravitreal bevacizumab and triamcinolone injection in a child with Coats disease. J AAPOS. 2008;12(3):309.