Transcript Coats` Disease - University of Louisville Ophthalmology

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
Visual Acuity:
OD
OS
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
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.
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
1. BCSC: Pediatric Ophthalmology and Strabismus. Coats ‘Disease. Pgs 287-288
2. BCSC: Retina and Vitreous. Coats’ Disease Pgs 143-145
3. 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.