This document summarizes various retinal conditions including retinal detachment, retinoblastoma, and retinitis pigmentosa. It describes the classification, causes, clinical features, investigations, and treatments of these conditions. Retinal detachment can be rhegmatogenous, tractional, or exudative in nature. Retinoblastoma is a malignant tumor of childhood that may appear as a white pupil reflex and is typically treated with chemotherapy, radiation, or enucleation. Retinitis pigmentosa is a genetic retinal dystrophy characterized by night blindness and bony spicule pigmentation.

1. RETINA

2. RETINAL DETACHMENT
 Separation of Neuro sensory retina from pigmented epithelium
CLASSIFICATION
1. Primary – Rhegmatogenous
2. Secondary -
a) Tractional
b) Exudative

3. RHEGMATOGENOUS DETACHMENT
 Due to break in the retina in the form of hole or tear.
 Through this vitreous gains access to subretinal space.
CAUSES
1.Myopia
2. Aphakia
3.Trauma
4.Senile PVD
5. Cataract surgery

4. Tractional Detachment
 Due to contraction of fibrous tissue bands on surface of retina and in the
vitreous.
 CAUSES
1. Diabetic retinopathy
2. Retinal vein occlusion
3. Eales disease
4. Toxocariasis

5. Exudative detachment
 Accumulation of fluid (exudative) in the subretinal space and pushes it
forward
• SYSTEMIC DISEASE
• Malignant hypertension
• Toxemia’s of pregnancy
• INFLAMMATION
• Posterior scleritis
• Sympathetic
ophthalmitis
• VKH
VASCULAR
• CSR
• Coats disease
• Uveal effusion
syndrome
INFECTIONS
• Toxoplasmosis
• CMV retinitis
• TB
• Syphilis
NEOPLASMS
• Choroidal melanoma
• Retinoblastoma
HYPOTONY
• Trauma
• Ocular hypotony

6. CLINICAL FEATURES
 Transient flashes and floaters
 Sudden profound loss of vision
 Shadow in front of the eye

7. FUNDUS EXAMINATION
 Greyish white detached retina
 Oscillations of retinal folds with eye movements
 One or more retinal tears and holes in the periphery (supero temporal
region )
 Associated pigmentation, degenerations , hemorrhages in retina
 Advanced stage – Funnel shaped grey retina attached only at disc and ora
serrata

8.  Rhegmatogenous – Corrugated, undulated, moves with eye movement.
 Tractional – concave shape
 Exudative – convex shape, smooth shape, no folds, Shifting fluid sign

9. INVESTIGATIONS
 Visual field – scotoma corresponding to area of detachment
 ERG – subnormal/ absent
 Ultrasound – confirms diagnosis

10. TREATMENT
1. Sealing of retinal break
• Photocoagulation
• Diathermy
• Cryo-coagulation
2. SRF Drainage
Inserting a fine needle through the sclera and choroid in to subretinal space to
drain the fluid.
3. Maintain chorio-retinal apposition
• Pneumatic retinopexy
Sealing breaks with cryopexy followed by injection of expanding gas bubble in
the vitreous to remain in contact for 4-7 days.

11.  Scleral buckle / en-circlage
Principle – inward indentation of sclera by inserting explants.
1) Radial explant – isolated hole
2) Circumferential explants – breaks involving 3 or more quadrants
Pars plana vitrectomy
- 3 port pars plana vitrectomy
- Internal drainage of SRF
- Flattening of retina by injecting silliicon oil or gas bubble
- Creating chorio retinal adhesions by endo laser
- Maintaining retinal tamponade by
Sillicon oil – ( 3-6 months)
Gas bubble ( 1-2 months)
SF6 , C3F8

12. DD’S of amaurotic cat’s eye reflex
 Yellowish white reflex in the pupillary area of child’s eye
 Also called Leucocoria
Causes
1. Retinoblastoma ( Usually unilateral/ age by 3 yrs)
2. Congenital cataract ( unilateral/ bilateral)
3. ROP ( history of prematurity or low birth wt <34 wks / < 2000 g)
4. Coats disease ( always unilateral/ mainly in boys)
5. Persistant hyperplastic primary vitreous PHPV ( Developmental
disorder of vitreous with microphthalmos)
6. Toxocara endophthalmitis – usually 2-9 yrs age, contact with pet dog or
cat)
7. Choroidal coloboma

13. RETINOBLASTOMA
 Most common primary congenital malignant intra ocular tumor of
children.
 Originating from outer nuclear layer of retina.
 Malignant proliferation of retinal neuroglial cells called retinoblasts that
failed to evolve normally.
 ETIOLOGY
1. Autosomal dominant – 6%
2. Sporadic ( somatic mutation, no family history ) – 94%

14. PATHOGENESIS
 Kundson 2 hit hypothesis
All patients have one mutant allele of RPE 1 gene in their bodies (1hit)
Further mutagenic event(2nd hit) second allele is affected undergo malignant
transformation.
TYPES
1. Glioma exophytum
2. Glioma endophytum
3. Glioma Planum

15. HISTOLOGY

16. CLINICAL FEATURES
 Common in infants and young age 2-4 yrs
 Leucocoria (60%)
 Convergent squint (20%)
 Severe pain ( raised IOP)
 Orbital inflammation with proptosis

17. FINDINGS
 Unilateral dilated pupil
 Pseudo hypopyon – Masquerade syndrome due to unilateral iris invasion.
 Hetrochromia of iris
 Secondary glaucoma
 Tumor seeds in vitreous cavity
 FUNDUS – multiple polypoidal mass covered with hemorrhages
Exophytic – grows in subretinal space and give rise to exudative RD.
Endophytic – projects from retina as white or pearly pink colored mass
In to vitreous cavity with cottage cheese appearance due to calcium deposits.

20. INVESTIGATIONS
 X ray orbit – calcification in 75 % cases
 Biochemistry of aqous humor – LDH and phosphor glucose isomerase.
 Ultrasound – cauliflower like mass with numerous high internal echos and
orbital shadowing.
 CT scan – calcific densities, describe the extend of tumor in optic nerve,
orbit and CNS.
 MRI to detect extrocular extension

21. TREATMENT
 Photocoagulation – for small tumours <3mm in apical height and < 10 mm in
basal diameter.
 Cryotherapy – small peripheral tumor of size 3 * 10 mm located anteriorly
without vitreous or subretinal seeds.
 Genetic counselling – important in bilateral cases or if 2 or more cases of RB
family.
 Chemotherapy – primary treatment in bilateral cases, in presence of bilateral
metastasis.
 Radiotherapy – Recurrent or residual tumors , following enucleation in
advanced cases.
 Enucleation – Treatment of choice for unilateral advanced intraocular disease.
 Exenteration – Debulking procedure followed by chemotherapy and radiation

22. RETINITIS PIGMENTOSA
 It is a primary pigmentary retinal dystrophy affecting rods and cones.
 Inheritance
• Autosomal dominant – benign and symptomatic only in adult life
• Autsomal recessive – most common
• X linked recessive – least common, more severe

23. PATHOGENESIS
• Mutation in rhodopsin (RDS) gene.
• Degenerative changes in rods and cones starting in equatorial region ant
then spreading anteriorly and posteriorly.
• Proliferaion of pigment epithelium with migration of pigments to retina.
• Attenuation of blood vessels.
• Atrophy of optic disc

24. Clinical features
 Night blindness ( characteristic feature)
 Delayed dark adaptation
 Later stages Tubular vision
CLASSICAL DIAGNOSTIC TRIAD
1. Bony spicule pigmentation
2. Arteriolar attenuation
3. Waxy pallor of optic disc

25. • Perivascular jet black spots resembling bony corpuscles,
along the course of retinal veins, found initially in equatorial
region.
• Thread like narrowed retinal arteries and veins.
• Pale wax like yellowish optic disc.
• Cystoid maculopathy/ Cellophane maculopathy.

26. VISUAL FIELD CHANGES
• Annular ring shaped scotoma corresponding to degenerated
equatorial zone of retina.
• Scotoma progressing anteriorly and posteriorly resulting in tubular
vision.
ERG – subnormal or abolished wave form

27. ASSOCIATIONS OF RP
 Ocular – POAG, microphthalmos, posterior subcapsular cataract (PSC)
 Systemic
1. Usher’s syndrome
2. Kearns- Sayre syndrome
3. Laurence- Moon- Biedl syndrome
4. Cockayne’s syndrome
5. Refsum’s syndrome
6. Abeta- lipoproteinemia

28. ATYPICAL RP
 Retinitis pigmentosa sine pigmento
 Retinitis punctata albescens
 Central pigment degeneration
 Sectorial RP
 Pericentric/ Paravenous RP
 Unilateral RP

29. TREATMENT
 No specific treatment
 Treat complications like cystoid macular edma
 Low vision aid and night vision device
 Genetic counselling for consanguinous marriage