Download MBBS Ophthalmology PPT 41 Paediatric Retinal Diseases Lecture Notes

Paediatric Retinal Diseases

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Symptoms of Retinal Diseases

? Diminution of vision without pain
? Night blindness
? Photopsia- sparks or lightening flashes

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? Metamorphopsia- distorted images
micropsia & macropsia
? Peripheral constriction of visual field
? Patient unaware of symptoms

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Symptoms of Retinal Diseases in Paediatric

cases

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? Diminution of vision without pain

? Night blindness

? Photopsia- sparks or lightening flashes

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? Metamorphopsia- distorted images

? micropsia & macropsia

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? Peripheral constriction of visual field

? Patient unaware of symptoms

? Jerky movements of eyes[Nystagmus]

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Normal Fundus
What do we examine in fundus?

? Fundal glow/ ocular media

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? Optic disc
? Retinal blood vessels
? General (Background) fundus
? Macula & foveal reflex
? Peripheral fundus

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Examination of Fundus

(Ophthalmocopy)

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? Distant direct ophthalmoscopy
? Direct ophthalmoscopy
? Indirect ophthalmoscopy
? Slit lamp assisted Fundus examination-
i] Hruby lens

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ii] +90 D / +78 d lens
iii] 3-mirror contact lens
Types of Retinal diseases in Paediatric age

group

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? Retinopathy of Prematurity
? Retinitis Pigmentosa
? Phakomatosis
? Coat's disease

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? Hereditary Dystrophies of central retina and

choroid

? Myelinated nerve fibres

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Retinopathy of Prematurity(ROP)

? B/L abnormality
? Retinal neovascularization

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? Premature infants
? <1500 gm
? <32 week gestational age
? Given high conc of Oxygen during first

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10 days of life


ROP -Signs

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Dilatation of retinal vessels

+

hazy white patches in peripheral retina (temporal)

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Fibrous tissue

Proliferation & rol ing over (Pseudo glioma)

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Retinal detachment (Lost vision)



ROP Staging

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? Stage 1= a faint demarcation line
? Stage 2= an elevated ridge
? Stage 3= extraretinal fibrovascular tissue
? Stage 4= sub-total retinal detachment

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? Stage 5= total retinal detachment


ROP

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ROP- Treatment

? 80% infants spontaneous regression
? Treatment required if disease progresses
i] Photocoagulation/ Cryotherapy of avascular,

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immature retina

ii] Scleral buckling for RD
? Prophylaxis is most important

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ROP Prophylaxis

? Monitor umblical arterial Pa Oxygen level
(50-100 is normal)
? Examine temporal periphery of retina before

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the newborn/infant is discharged from

hospital (look for threshold disease)

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? All premature infants <32 week or <1500 gm

should be screened

? If ROP develops, follow-up exam

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Retinitis Pigmentosa

? Hereditary disease

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? Inheritance-Autosomal recessive

? Consanguinity of parents

? Progressive night blindness

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? Constriction of visual field

? B/L

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? Symmetrical , diffuse pigmentary retinal

dystrophy which affects Rods

? Mostly symptoms appear in young age

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Retinitis Pigmentosa

Signs-
?Bony specule

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pigmentation

?Arteriolar

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attenuation

?Waxy pallor of disc

Retinitis Pigmentosa

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Investigations-
? Electro-retinography- amplitude
? Electro-oculography- absence of light

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peak

? Visual fields- ring scotoma

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Retinitis Pigmentosa-Variants

Atypical RP

i. Retinitis

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pigmentosa sine

pigmento

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i . Retinitis puntata

albescens

i i. Sector retinitis

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pigmentosa

RP associated with systemic diseases-

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I. Laurence-moon-biedl syndrome(Bardet Biedle)

I . Bassen-kornzweig syndrome (Abeta

lipoproteinemia)

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I I. Refsum's syndrome

IV. Usher's syndrome

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V. Cockayne's syndrome

VI. Kearns-Sayre syndrome

VI . Friedreich's ataxia

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VI I.NARP syndrome


Persistent Hyperplastic Primary Vitreous

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? Failure of structures within primary vitreous to

regress

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? Unilateral white pupilary reflex in full term

infant

? May be Anterior/ Posterior

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? May be associated with cataract, Glaucoma,

Micro ophthalmos, persistent hyaloid artery

? Bilateral cases may be associated with Patau's

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syndrome or Norries disease.

Coat's Disease

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? Chronic, progressive,

vascular abnormality

? Telangiectic retinal vessels

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leak fluid

? Exudative bullous RD
? Boys 18months -18 years

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? Unilateral
? White pupillary reflex
? Tt: early photocoagulation
/cryotherapy

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Stargardt disease

? Recessive, progresstive tapetoretinal

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dystrophy of central retina

? Age 8-14 years
? Beaten bronze atrophy of fovea
? Extensive chorioretinal atrophy

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? Poor vision

Hereditary Dystrophies of central retina and

choroid

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Stargardtdisease/ fundus

flavimaculatus

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? Most common macular

dystrophy

? Gradual impairment of

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central vision


Phakomatosis

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(Systemic associations present)
? Angiomatosis of retina with

cerebellar

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haemangioblastoma (Von

Hippel Lindau disease)

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? Tuberous sclerosis

(Bourneville disease)

? Neurofibromatosis (Von

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Recling hausen disease)

Phakomatosis

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? Sturge-weber's syndrome- port wine stain along

distribution of trigeminal nerve of affected side

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White pupillary reflex (Leukocoria)

? Retinoblastoma- dealt in separate lecture

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White pupillary reflex (Leukocoria)

i. Retinoblastoma

ii. ROP

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iii. Coats disease

iv. Congenital cataract

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v. Toxocara infestation

vi. Persistant hyperplastic vitreous

vii. Retrolental hyperplasia

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viii.Retrolental inflammatory membrane due to

Uvitis
MCQs

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1] While working in a neonatal ICU your team

delivers a premature infant at 27 weeks of

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gestation and weighing 1500 gm. How soon will

you request fundus examination by an

ophthalmologist?

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(a) Immediately
(b) Three to four weeks after delivery
(c) At 34 weeks' gestational age
(d) At 40 weeks' gestational age

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MCQs

? With which of the following is PHPV

associated?

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(a) Patau's Syndrome
(b)Down syndrome
(c) Tuberous sclerosis
(d) Sturge Weber syndrome

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MCQs

Amaurotic cat's eye reflex is seen in:

a.Papilloedema

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b. Retinoblastoma
c. Papillitis
d. Retinitis