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Coats' Disease

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Zachary Inman

on 22 September 2013

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Transcript of Coats' Disease

Pathophysiology
Etiology
& Epidemiology
Differential Diagnoses
Treatment
Coats' Disease
Table of Contents
Pathophysiology
Etiology
Signs & Symptoms
Disease Classification
Differential Diagnoses
Workup
Treatment & Management
Signs & Symptoms
Workup
Coats' Disease
Zachary Inman, OMS-IV
LECOM Bradenton

Disease Classification
The Shields (2001) published
a study of 150 Coats' patients.
"Yellow glow in
the family photo..."
Slowly progressive, unilateral loss of vision.
As disease worsens, may develop loss of fixation or even
painful glaucoma.
"Light bulb" telangiectasias may be seen in retinal periphery.
Yellowish exudates may be seen in SRF with predilection for posterior pole.
Advanced stages may
show exudative
retinal detachment.
Proposed a staging system for Coats' disease to estimate outcomes and guide therapy.
Stage 1:
Stage 2a:
"Telangiectasia
+ Extrafoveal Exudates"
Stage 4:
"Total RD + NVG"
Stage 3a:
"Exudative Subtotal RD"
"Telangectasias only"
0%...
Tx: Laser photocoagulation and/or frequent observation.
Stage 2b:
"Telangiectasia
+ Foveal Exudates"
Tx: Laser photocoagulation
and/or cryotherapy
53%...
Stage 3b:
"Exudative Total RD"
74%...
Tx: Cyrotherapy and/or surgical reattachment
Tx: ? enucleation for
severe pain.
Stage 5:
"Advanced End-Stage Disease"
Tx: None required.
100%...
Retinoblastoma
VHL
Familial Exudative Vitreoretinopathy (FEVR)
Persistant Fetal Vasculature (PFV)
Astrocytic
Hamartoma
Ocular
Toxocariasis
Processes that may present with leukocoria, SRF exudates, esp. in the pediatric population.
Sporatic or Heritable
(similar appearance)
Most common
pediatric malignancy
A.D.
Renal CCC, pheo,
pancreatic tumors
"Vessel leakage, exudate,
and RD."
ROP
Disorganized vessels.
May lead to scarring or RD.
Fibrous bands from optic disc to the retrolental region (failure of embryological vascular regression)
Usu. unilateral posterior pole granuloma,
may also present as a diffuse endophthalmitis.
Helmenth parasite
Toxocara canis (dogs)
Yellowish with glistening calcifications; doesn't produce exudates or RD
RD can be treated with
SRF drainage and scleral buckling.
Recurrances are common
(need to follow at 6-month intervals)
References:
1. Coats' disease. Del Longo, A. Orphanet Encyclopedia; Sept 2004.
2. Coats' disease. NIH Office of Rare Disease Research. rarediseases.info.nih.gov. Accessed 7.6.13
3. Classification and management of Coats' disease. Shields JA, Shields CL, Honavar SG. Am. J Ophthalmol; May 2001. 131(5):572-83.
4. Harley's Pediatric Ophthalmology: Fifth Edition. Nelson LB, Olitsky SE. LW&W, 2005
5. Opthalmology Secrets: Third Edition. Vander JF, Gault JA. Mosby Elsevier, 2007.
6. The Wills Eye Manual: Sixth Edition. Gerstenblith AT, Rabinowitz MP, et al. LW&W, 2012
7. BCSC, Section 12: Retina and Vitreous (2011-2012); American Academy of Ophthalmology. Coats' Disease, pp 170-171.
8. Morris B, Foot B. A Population Based Study of Coats' Disease In The United Kingdom. Eye (London). 2010 Dec; 24(12): 1797-801
9. Abramson DH, Frank DM. Presenting Signs of Retinoblastoma. J Ped 1998. (132); 505-8
10. Morteza, E. Resolution of Macular Edema in Coats' Disease with Intravitreal Bevacizumab. Indian J. Oph (2010), Jan-Feb 59(1) 80-2
11. The Wills Eye Manual: Fifth Edition. Ehlers, Justis and Shah, Chirag. Philadelphia, LW&W
12. Free Medical Textbook, at medpressfree.wordpress.com (sourced 8/14/2013), Chapter 120: "Coats' Disease and Retinal Telangiectasia"
Birth/Medical History:
May help you rule out both ROP and congenital cataract.
SLE:
Normal AS findings during early stages of Coats' (vs PHPV)
Indirect and FP/FA:
"Light bulb" telangectasias + yellow SRF
Lack of intraocular mass or calcification (vs Exophytic RB)
Ocular US (A- or B-scan):
CT scan:
Lack of intraocular calcifications (vs astrocytoma and 90% advanced RB cases)
MRI:
Presence of proteinaceous SRF in Coats' disease
#2:
Abnormal endothelium permits growth of telangiectasias and saccular aneurysms.
#1:
#3:
#4:
In Coats' disease, retinal capillary endothelial cells develop with abnormal fenestrations.
Vascular permeability allows massive lipid exudate into the intra- and subretinal space.
Subretinal deposits may become so massive as to cause partial or total retinal detachment.
#5:
Chronic exudative RD may progress to secondary involvement of the AS.
1
3
6
PE:
PE:
T2
A.D. and Bilateral...
Cryotherapy:
Equator to the ora serrata
Rationale: elimination of affected retinal blood vessels
? Osteosarcoma
(RB1 locus, Chro. 13)
Other...
For milder cases of lipid exudation:

DR
BRVO
Juxtafoveal Retinal Telangiectasia
Radiation Retinopathy
"Coats..."
This retinal condition is not hereditary and is not associated with any systemic vascular abnormalities.
Precise etiology is
yet undetermined.
?
Concomitant somatic mutations in the NDP gene (norrin).
2
Xp11.4
Most frequently males 8 to 10.
8-10% cases are in females.
90% cases are unilateral.
The severity and rate of progression appear greater in patients under the age of 4 years.
7
TH2 cells release which cytokine?
Adult Coats'...
Estimated population incidence of 0.09 per 100,000 of the population
8
80% dx before age three, extremely rare to diagnose after age 6.
9
Retinal angiomatosis + CNS
and visceral involvement.
7
Initial insult is failure of temporal vascularization.
7
Normal vessel development completes nasally at 36wg, temporally at 40wg.
7
90% unilateral
Indirect Laser:
Effective if mild/mod exudate;
"treatment of choice" in early stages of the disease.
Anti-VEGF medications may also
play a role in treatment...
A: pretreatment
B: 8 mos s/p Avas #3
C: 12 mos s/p Avas #3
"Resolution of macular edema in Coats' disease with intravitreal bevacizumab." Indian J. Oph, (2010)
10
Heal for 10-14 d.
Sore/Swollen
1
Severe exudation may necessitate a pars plana vitrectomy to drain the SRF before the pathologic vessels can be treated.
1
"...speaking of coats..."
"Failed his school
vision test..."
1
Intervention should
occur when:
1: Exudation is extensive or progressive
2: Exudates threaten central visual acuity
3: Significant peripheral RD is observed
Often ass'd w/
tuberous scler.,
rarely with
neurofibro.
Paracentesis of anterior chamber = eosinophils?
Usu. negative Fam Hx
Zones 1-3?
11
Lipid resolution may begin within six weeks of initial treatment if abnormal vessels can be eliminated.
If significant exudate is present, resolution may take over a year.
12
SRF Biopsy:
(Gold standard)
Used rarely, but confirmatory.
[+] cholesterol crystals, [-] tumor cells.
7
12
Norrie Disease = X-linked recessive

A brief video showing the treatment of extensive disease:
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Full transcript