Retina ConferenceRetina Conference

Eddie Apenbrinck, M.D.Eddie Apenbrinck, M.D.

University of LouisvilleUniversity of Louisville

Department of Ophthalmology and Visual Department of Ophthalmology and Visual SciencesSciences

6/5/20146/5/2014

PresentationPresentation

CCCC: abnormal fundus exam in a : abnormal fundus exam in a formerly premature infantformerly premature infant

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

HistoryHistoryPast Ocular HxPast Ocular Hx: ROP (?): ROP screenings normal until : ROP (?): ROP screenings normal until

3/21/14 when right eye showed signs of ROP stage 3 3/21/14 when right eye showed signs of ROP stage 3 zone 2, left eye normalzone 2, left eye normal

Past Medical Hx:Past Medical Hx: prematurity, patent foramen ovale, prematurity, patent foramen ovale, gastroesophageal refluxgastroesophageal reflux

Birth HistoryBirth History: 30 week gestation preterm male. : 30 week gestation preterm male. Pregnancy complicated by pre-ecclampsia and Pregnancy complicated by pre-ecclampsia and placental abruption.placental abruption.

Birth weight: 1170 gramsBirth weight: 1170 grams

Meds:Meds: multivitamin, Prilosec (1.5 mg/kg/day)multivitamin, Prilosec (1.5 mg/kg/day)

ExamExam ODOD OSOS

Visual AcuityVisual Acuity: Withdraws from light : Withdraws from light OUOU

IOPIOP: : Soft OU Soft OU

EOM:EOM: Full OU Full OU

Anterior Segment Anterior Segment : Unremarkable OU: Unremarkable OU

Clinical CourseClinical Course

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

RetCam Fundus Photos RetCam Fundus Photos ODOD

Subretinal yellow discoloration along inferior arcade.

Avascular temporal periphery, temporal vascular telangiectasia, tortuosity, and dilation.

RetCam Fundus Photos RetCam Fundus Photos OSOS

Normal fundus photo

Fluorescein Angiography Fluorescein Angiography ODOD

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

Fluorescein Angiography Fluorescein Angiography ODOD

AV phase: saccular aneurysm and vascular abnormalitiesAV phase: saccular aneurysm and vascular abnormalities

Assessment and PlanAssessment and Plan 3 month old male referred for abnormal retina 3 month old male referred for abnormal retina

exam. Dilated exam OD showed an avascular exam. Dilated exam OD showed an avascular temporal periphery and vascular abnormalities temporal periphery and vascular abnormalities (dilation, tortuosity, and telangiectasia)(dilation, tortuosity, and telangiectasia)

DDxDDx: : Coats’ DiseaseCoats’ Disease ROPROP FEVRFEVR RetinoblastomaRetinoblastoma

Plan: Plan: Initiate treatment with panretinal Initiate treatment with panretinal

photocoagulation (PRP) and cryotherapyphotocoagulation (PRP) and cryotherapy

TreatmentTreatment

Laser photocoagulation and Laser photocoagulation and cryotherapy to the temporal periphery cryotherapy to the temporal periphery and vascular abnormalities.and vascular abnormalities.

Treatment #1: 665 laser burns, 4 cryotherapy Treatment #1: 665 laser burns, 4 cryotherapy freezesfreezes

Clinical CourseClinical Course

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

Coats’ DiseaseCoats’ Disease

First described by George Coats, a First described by George Coats, a

Scottish medical student in 1908.Scottish medical student in 1908.

Coats’ disease is an idiopathic condition Coats’ disease is an idiopathic condition characterized by retinal vascular changes characterized by retinal vascular changes and exudation caused by a defect in retinal and exudation caused by a defect in retinal vasculature developmentvasculature development

Increased permeability of abnormal retinal Increased permeability of abnormal retinal vessels causes leakage into the intraretinal vessels causes leakage into the intraretinal and subretinal spaces.and subretinal spaces.

Coats’ DiseaseCoats’ Disease

EpidemiologyEpidemiology

Men (85%) affected more often than Men (85%) affected more often than womenwomen

Usually unilateral (80%)Usually unilateral (80%)

Average age at diagnosis 6-8 years, but Average age at diagnosis 6-8 years, but can occur in infants and adults.can occur in infants and adults.

Coats’ DiseaseCoats’ Disease

DiagnosisDiagnosis

Typically diagnosed as a result of the Typically diagnosed as a result of the recognition of poor vision, strabismus, or recognition of poor vision, strabismus, or leukocorialeukocoria

Ancillary testing may be useful in ruling Ancillary testing may be useful in ruling out other potential causes of leukocoria out other potential causes of leukocoria in children.in children. FA, CT, MRI, U/SFA, CT, MRI, U/S

Coats’ DiseaseCoats’ Disease TreatmentTreatment

The major goal of treatment in Coats' disease is to The major goal of treatment in Coats' disease is to preserve or improve visual acuity or, when this is preserve or improve visual acuity or, when this is impossible, to preserve the anatomical integrity of the impossible, to preserve the anatomical integrity of the eye.eye.

Treatment generally consists of photocoagulation, Treatment generally consists of photocoagulation, cryotherapy, and in severe cases, retinal cryotherapy, and in severe cases, retinal reattachment surgeryreattachment surgery

Anti-VEGF injections have been successfully used as Anti-VEGF injections have been successfully used as part of combination therapy in case series but there is part of combination therapy in case series but there is a risk of vitreoretinal tractiona risk of vitreoretinal traction

Exudative Retinal Exudative Retinal DetachmentDetachment

Retrospective consecutive series of 150 Retrospective consecutive series of 150 patients with Coats’ Disease over 25 years.patients with Coats’ Disease over 25 years. Successful treatment in early stages can be best Successful treatment in early stages can be best

achieved with laser photocoagulation or achieved with laser photocoagulation or cryotherapy.cryotherapy.

More advanced cases may require surgical More advanced cases may require surgical techniques of retinal reattachment, combined with techniques of retinal reattachment, combined with photocoagulation or cryotherapy.photocoagulation or cryotherapy.

Carefully selected treatment can anatomically Carefully selected treatment can anatomically stabilize or improve the eye with Coats disease in stabilize or improve the eye with Coats disease in 76% of cases. 76% of cases.

Classification of Coats’ Classification of Coats’ DiseaseDisease

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

Literature ReviewLiterature Review Concluded that earlier diagnosis and prompt Concluded that earlier diagnosis and prompt

treatment will result in better visual outcome treatment will result in better visual outcome and fewer enucleations for Coats disease in the and fewer enucleations for Coats disease in the future.future.

Retrospective case series of 8 patients with Coats’ Retrospective case series of 8 patients with Coats’ disease manifesting as partial or complete retinal disease manifesting as partial or complete retinal detachment treated with cryotherapy (n=8) and/or detachment treated with cryotherapy (n=8) and/or PRP (n=4) plus additional intravitreal bevacizumab PRP (n=4) plus additional intravitreal bevacizumab (n=8)(n=8)

Mean follow-up of 8.5 monthsMean follow-up of 8.5 months Resolution of retinopathy (8 of 8)Resolution of retinopathy (8 of 8) Resolution of Coats-related subretinal fluid (8 of 8)Resolution of Coats-related subretinal fluid (8 of 8) Resolution of retinal exudates (6 of 8)Resolution of retinal exudates (6 of 8) Development of retinal fibrosis (4 of 8)Development of retinal fibrosis (4 of 8)

3 of 4 developed traction retinal detachment3 of 4 developed traction retinal detachment

British Journal of Ophthalmology June 2011

ReferencesReferences1. BCSC: Pediatric Ophthalmology and Strabismus. Coats

‘Disease. Pgs 287-2882. BCSC: Retina and Vitreous. Coats’ Disease Pgs 143-1453. Shields, JA et al. Classification and Management of Coats

Disease: the 2000 Proctor Lecture. Am J Ophthalmology. 2001;31(5):572-583. 

4. Kanski, Jack J. Clinical Ophthalmology: A Systematic Approach. 6th ed. Elsevier Ltd., 2007.

5. 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.

6. Ridley ME, Shields JA, Brown GC, Tasman W. Coats’ disease: evaluation of management. Ophthalmology. 1982;89:1381-1387.

7. Coats G. Forms of retinal disease with massive exudation. Royal London Ophthalmic Hospital Reports.1908;17(3):440-525.

8. Cakir M, Cekiç O, Yilmaz OF. Combined intravitreal bevacizumab and triamcinolone injection in a child with Coats disease. J AAPOS. 2008;12(3):309.