Download MBBS Ophthalmology PPT 41 Paediatric Retinal Diseases Lecture Notes

Download MBBS (Bachelor of Medicine, Bachelor of Surgery) Ophthalmology PPT 41 Paediatric Retinal Diseases Lecture Notes

Paediatric Retinal Diseases



Symptoms of Retinal Diseases

? Diminution of vision without pain
? Night blindness
? Photopsia- sparks or lightening flashes
? Metamorphopsia- distorted images
micropsia & macropsia
? Peripheral constriction of visual field
? Patient unaware of symptoms


Symptoms of Retinal Diseases in Paediatric

cases

? Diminution of vision without pain

? Night blindness

? Photopsia- sparks or lightening flashes

? Metamorphopsia- distorted images

? micropsia & macropsia

? Peripheral constriction of visual field

? Patient unaware of symptoms

? Jerky movements of eyes[Nystagmus]

Normal Fundus
What do we examine in fundus?

? Fundal glow/ ocular media
? Optic disc
? Retinal blood vessels
? General (Background) fundus
? Macula & foveal reflex
? Peripheral fundus

Examination of Fundus

(Ophthalmocopy)

? Distant direct ophthalmoscopy
? Direct ophthalmoscopy
? Indirect ophthalmoscopy
? Slit lamp assisted Fundus examination-
i] Hruby lens
ii] +90 D / +78 d lens
iii] 3-mirror contact lens
Types of Retinal diseases in Paediatric age

group

? Retinopathy of Prematurity
? Retinitis Pigmentosa
? Phakomatosis
? Coat's disease
? Hereditary Dystrophies of central retina and

choroid

? Myelinated nerve fibres

Retinopathy of Prematurity(ROP)

? B/L abnormality
? Retinal neovascularization
? Premature infants
? <1500 gm
? <32 week gestational age
? Given high conc of Oxygen during first

10 days of life


ROP -Signs

Dilatation of retinal vessels

+

hazy white patches in peripheral retina (temporal)

Fibrous tissue

Proliferation & rol ing over (Pseudo glioma)

Retinal detachment (Lost vision)



ROP Staging

? Stage 1= a faint demarcation line
? Stage 2= an elevated ridge
? Stage 3= extraretinal fibrovascular tissue
? Stage 4= sub-total retinal detachment
? Stage 5= total retinal detachment


ROP

ROP- Treatment

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

immature retina

ii] Scleral buckling for RD
? Prophylaxis is most important
ROP Prophylaxis

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

the newborn/infant is discharged from

hospital (look for threshold disease)

? All premature infants <32 week or <1500 gm

should be screened

? If ROP develops, follow-up exam

Retinitis Pigmentosa

? Hereditary disease

? Inheritance-Autosomal recessive

? Consanguinity of parents

? Progressive night blindness

? Constriction of visual field

? B/L

? Symmetrical , diffuse pigmentary retinal

dystrophy which affects Rods

? Mostly symptoms appear in young age






Retinitis Pigmentosa

Signs-
?Bony specule

pigmentation

?Arteriolar

attenuation

?Waxy pallor of disc

Retinitis Pigmentosa

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

peak

? Visual fields- ring scotoma


Retinitis Pigmentosa-Variants

Atypical RP

i. Retinitis

pigmentosa sine

pigmento

i . Retinitis puntata

albescens

i i. Sector retinitis

pigmentosa

RP associated with systemic diseases-

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

I . Bassen-kornzweig syndrome (Abeta

lipoproteinemia)

I I. Refsum's syndrome

IV. Usher's syndrome

V. Cockayne's syndrome

VI. Kearns-Sayre syndrome

VI . Friedreich's ataxia

VI I.NARP syndrome


Persistent Hyperplastic Primary Vitreous

? Failure of structures within primary vitreous to

regress

? Unilateral white pupilary reflex in full term

infant

? May be Anterior/ Posterior
? May be associated with cataract, Glaucoma,

Micro ophthalmos, persistent hyaloid artery

? Bilateral cases may be associated with Patau's

syndrome or Norries disease.

Coat's Disease

? Chronic, progressive,

vascular abnormality

? Telangiectic retinal vessels

leak fluid

? Exudative bullous RD
? Boys 18months -18 years
? Unilateral
? White pupillary reflex
? Tt: early photocoagulation
/cryotherapy


Stargardt disease

? Recessive, progresstive tapetoretinal

dystrophy of central retina

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

Hereditary Dystrophies of central retina and

choroid

Stargardtdisease/ fundus

flavimaculatus

? Most common macular

dystrophy

? Gradual impairment of

central vision


Phakomatosis

(Systemic associations present)
? Angiomatosis of retina with

cerebellar

haemangioblastoma (Von

Hippel Lindau disease)

? Tuberous sclerosis

(Bourneville disease)

? Neurofibromatosis (Von

Recling hausen disease)

Phakomatosis

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

distribution of trigeminal nerve of affected side


White pupillary reflex (Leukocoria)

? Retinoblastoma- dealt in separate lecture


White pupillary reflex (Leukocoria)

i. Retinoblastoma

ii. ROP

iii. Coats disease

iv. Congenital cataract

v. Toxocara infestation

vi. Persistant hyperplastic vitreous

vii. Retrolental hyperplasia

viii.Retrolental inflammatory membrane due to

Uvitis
MCQs

1] While working in a neonatal ICU your team

delivers a premature infant at 27 weeks of

gestation and weighing 1500 gm. How soon will

you request fundus examination by an

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

MCQs

? With which of the following is PHPV

associated?

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

Amaurotic cat's eye reflex is seen in:

a.Papilloedema
b. Retinoblastoma
c. Papillitis
d. Retinitis