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Anatomy of Retina

  1. Retina extends from optic disc (thickest ) to ora serrata (thinnest)
  2. Optic disc: 1.5 mm in diameter
  3. Macula:
    1. 5.5 mm at posterior pole
    2. Temporal to optic disc
    3. Photopic and color vision are primary functions
    4. Fovea – Centralis :
      1. Central depressed part of Macula
      2. 1.5 mm in diameter
    5. Foveola - * 0.35 mm
      1. 2 DD away from temporal margin of optic disc.
    6. FAZ- ( Foveal Avascular Zone)-0.85MM-DETERMINED ON FFA
      Inside the fovea but outside foveola.
Microscopic Structure of Retina: (Out to IN)
  1. RPE                                                         
  2. Rods and cones
  3. External limiting membrane                                     
  4. Outer nuclear layer
  5. Outer plexiform layer                                               
  6. Inner nuclear layer
  7. Inner plexiform layer                                               
  8. Ganglion cell layer
  9. Nerve fibre layer                                             
  10. Internal limiting membrane



Fluorescein Angiography: (FA)
FA is used in studying the normal physiology of the retinal and choroidal circulation and to detect any abnormalities.
5 ml of 10% Flourescein is injected in antecubital vein* and photograph taken at 1 sec. Interval between 5 and 25 sec.* after injection.
Excitation filter-490nm/blue, observation-530nm/green



Difference between Rods & Cones is all except: (AIIMS May 09)
A. Intensity                      
B.  Number
C. Transduction                
D.  Color


Ans. C.  Signal Transduction

  1. Diabetic Retinopathy: (DR)
    Prevalence of DR is higher in IDDM (> 5yrs) than in NIDDM (can present with DR at time of diagnosis)
  • Risk Factors For DR:
    Diabetic retinopathy (DR) Risk factor
    • Duration of diabetes Q - In patients diagnosed with DM before the age of 30 years, the incidence of DR after 10 years is 50% and after 30 years 90% [most important]
    • DR rarely develops within 5 years of the onset of DM or before puberty, but about 5% of type 2 DM have DR at presentation
    • Poor metabolic control Q
    • Pregnancy Q - is occasionally associated with rapid progression of DR, predicating factors include poor pre pregnancy control of diabetes, too rapid tightening of control during the early stages of pregnancy and the development of pre-eclampsia
    • Hypertension Q -
    • Nephropathy Q -
    • Others Q - - smoking, obesity and hyperlipidemia
  • DR is a microangiopathy affecting the retinal precapillary arterioles, capillaries and venules .
  • Earliest hishopathogical change : Thickening of capillary wall basement WITH LOSS OF PERICYTES
  • mantra hallmark of Dr. : Neovascularisation
  1. NPDR(Non Proliferative Diabetic Retinopathy)
    1. Mild
      1. Atleast one microaneurysm
    2. Moderate (Any of these should be definitely present to a mild degree
      1. Cotton woofrpoks                                                
      2. ​Venous Beading             
    3. Severe NPDR (Any 1 of below)(4-2-1 rule)
      1. HE/Microaneurysms in all 4 quadrants                            
      2. Venous Beading in 2 or more quadrant
      3. IRMA in at least 1 quadrant
    4. Very severe NPDR (Any 2 features)
      1. Any 2 or more of Sever NPDR
  2. PDR (Proliferative Diabetic Retinopathy)
    1. Early PDR
      i. Presence of new vessels
    2. High risk PDR
      1. NVD ≥ 1/3 – ½  disc area or
      2. NVD and Vitreous or Preretinal haemorrhage or
NVE ≥ ½ disc area and Preretinal or Vitreous haemorrhage
  1. It delays the onset of DR, although it does not prevent
  2. It slows the progression of back ground DR
  3. It decreases the rate of conversion of NPDR to PDR
  4. It decreases the incidence of macular edema
  5. It decreases the need for laser photo coagulation
Non- Proliferative Diabetic Retinopathy
Clinical features :
  1. Microaneurysm is the earliest clinical feature : appear as minute, red dots arranged like a cluster of grapes
  2. Hard exudates : contain lipid laden macrophages
  3. Cotton wool spots : due to axonal stasis- axonal edema ( more common with hypertensive retinopathy
  4. IRMA ( intra-retinal microvascular abnormalities) -  shunt vessels from arterioles to venules
  5. Dot- blot haemorrhages: occurs from capillaries located in outer plexiform and inner nuclear layer
  6. Flame shaped haemorrhages : occurs from capillaries located in nerve fiber layer
Management :
  • Metabolic control for mild and maderate NPDR cases
  • Pan-retinal photocoagulation done in severe and very severe NPDR ( laser used : Argon Green laser / double frequency Nd: YAG laser)
Diabetic Macular Edema ( m/c cause of visual loss in DR)
Clinically Significant Macular Edema
  • Retinal thickening within 500 µm of the macular center.
  • Hard exudates within 500 µm of the macular center with adjacent retinal thickening.
  • One or more disc diameters of retinal thickening, part of which is within one disc diameter of the macular center
Investigation : Fluorescien angiography
  1. Focal leakage : treated with focal photocoagulation
  2. diffuse leakage : grid photocoagulation
  3. ischaemic maculopathy: no laser
  • Note: laser done for extra foveal lesions
  • For sub-foveal lesions : intravitreal triamcinolone / anti- VEGFs are used
  • Surgery – vitrectomy to relieve taut posterior hyaloid

Intravitreal anti-VEGF agents
  • VEGF increases retinal vascular permeability, causes breakdown of the blood-retina barrier, and results in retina edema. VEGF is up-regulated in diabetic retinopathy.
  • The anti-VEGF agents are pegaptanib sodium, ranibizumab, and bevacizumab.
  • Pegaptanib sodium is a pegylated aptamer directed against the VEGF-A165 isoform. It was the first FDA approved ophthalmologic anti-VEGF agent for the treatment of choroidal neovascularization (CNV) from age-related macular degeneration (ARMD).
  • Bevacizumab is a humanized monoclonal antibody, and was the first commercially available angiogenesis inhibitor. It stops tumor growth by preventing the formation of new blood vessels by targeting and inhibiting the function of a natural protein called vascular endothelial growth factor (VEGE) that stimulates new blood vessel formation
Proliferative DR Q
  1. Neovascularization Q is the hall mark of PDR, arise from the optic nerve head, there is neovascularization of the disc (NVD) and along the large vessels, as well as neovascularization elsewhere (NVE)
  2. It appears in areas of capillary closure and the accompanying fibrous tissue varies in extent, such fibrovascular tissue may lie flat on the retina or attach it self to the posterior vitreous face leading later to vitreous traction, retinal separation and the tearing of blood vessels.
  3. This is the commonest cause of spontaneous vitreous haemorrhage in adult
  4. Vitreous detachment and vitreous haemorrhage Q
  • Complication of PDR:
  1. Persistent intra-gel vitreous hemorrhage
  2. Tractional RD
  3. Opaque membrane- on posterior surface of detached hyaloid
  4. Burnt out sate: Increase in fibrous component
  5. Rubeosis iridis: NVG
Management :
  • Pan retinal photocoagulation if media clear
  • Vitrectomy : indications:
  1. Non- resolving vitreous haemorrhage
  2. taut posterior hyaloid face
  3. combined rhegmomatous + tractional retinal detachment
  • Screening of diabetic retinopathy
  1.  T1D : 5 years after diasnoses
  2. T2D : immediately on diagnosis.
  1. Central Retinal vein occlusion
Common, next to DR
  1. Predisposing factors
    a. Increasing age – 6th - 7th decade
    b. Systemic hypertension
    c. Blood dyscrasias – hyperviscosity due to chronic leukemias and polycythemia
    d. Raised IOP (POAG)
    e. Periphlebitis – sarcoidosis, Beh|et’s disease
  2. Classification of CRVO:
    a. Non – Ischemic
    b. Ischemic
  3. C/F
    a. Tortuosity and dilation of retinal veins
    b. Flame –shaped hemorrhage
    c. Cotton – wool spots
    d. Optic disc edema and hyperaemia
    e. Macular edema; complication 100 day glaucoma/90 day glaucoma (neovasular) macular edena-grid laver after 3 moths
  4. Treatment
    a. FA if ischemic prompt PRP to prevent NVG.
  1. Retinal artery occlusion (CRAO)
    Due to embolism from heart or carotid artery, occlusion at level of lamina crobrosa
    1. C.F:
      1. Sudden, painless loss of vision
      2. Marcus – Gunn pupil*
      3. Milky White retina due to narrowed retinal arterioles: retinal  edema
      4. Cherry red spot*
      5. “cattle track” appearance
    2. Treatment: It is an ocular emergency
      1. Firm ocular massage → May dislodge embolus                    
      2. Intravenous acetazolamide – 500mg, mannitol     
      3. Inhalation of mixture of 5% Co2 and 95% of O2
      4. Anterior chamber paracentesis
    3. D/D Of Cherry – Red Spot*:
      1. CRAO
      2. Tay Sachs Disease
      3. Niemann – pick Disease
      4. Gangliosidosis –type I
        1. generalized
        2. type II standoff’s disease
      5. Sialidosis: (cherry –red spot myoclonus syndrome)
  2. Hypertensive Retinopathy
    Earliest sign-arteriolar spasm
    1. Fundus picture is characterized by
      a. Vasoconstriction                             
      b. Leakage                            
      c. Arteriosclerosis
    2. Grading*
    3. Grade-I : Mild generalized arteriolar attenuation.
    4. Grade – II:
      1. Severe generalized and focal arteriolar constriction.
      2. Salus sign*
    5. Grade – III
      1. Copper wiring* of arterioles                               
      2. Bonnet sign/ Gunn’s sign*
      3. Hemorrhages                                          
      4. Exudates
    6. Grade – IV
      1. Grade III changes +silver wiring* of arterioles + Disc-swelling of optic disc (papilledema*)
      2. Elschnig’s spots/ Seigrest streaks  –ischemic choroidal infarcts
  1. Retinitis Pigmentosa
  1. It's a photoreceptor dystrophy characterized by progressive loss of photoreceptor and RPE function.
  2. Damage to rod system is predominant.
  1. Inheritance:
    1. Sporadic*: Most common.
    2. AD*: Has best prognosis
    3. X- linked* recessive : Worst prognosis and least common
  1. C/F:
    1. Classical Triad*
      1. Arteriolar attenuation-earliest
      2. Retinal bone- spicule pigmentation
      3. Pale, waxy disc (consecutive OA)
    2. S/s
      1. Nyctalopia – due to defective dark adaptation-Earliest symptiom
    3. Other ocular features
      1. CME                          
      2. OAG                                  
      3. ​Post Seubcapsular cata
  2. INV:
    1. Subnormal amplitude of ERG mainly scotopic, flat or extinguished ERG
    2. EOG shows absence of light rise
  3. Systemic associateion*
    1. Bassen- Kornzweig syndrome
    2. Refsum’s syndrome
    3. Ushers syndrome- associated with deafness
    4. Cockayne syndrome
    5. Kearns   Sayre syndrome – ass with (ocular myopathy)
    6. Mucopolysaccharidosis
    7. Bardet – Biedl syndrome- mental retardation + polydactyly
    8. Laurence – Moon syndrome- features of Bardet- Biedl syndrome + spastic paraplegia
    9. Friedreich’s ataxia
  4. D/D of night blindness*
    1. RD                                                                                                      
    2. Xerophthalmia
    3. Congenial stationary night blindness                                           
    4. Oguches disease
    5. High myopia                     

Retinitis pigmentosa is NOT associated with? (AIIMS May 08)
A. Usher syndrome                                                             
B. Kornzewig syndrome                     
C. Kearns-Sayre syndrome                                                
D. Marfan syndrome


Ans. D. Marfan syndrome

  1. Vitreo retinal degenerations
    1. Lattice Degenerations:-MC Retinal diger
    2. common in high myopes may be associated with holes or retinal tears.
  2. RD ( Retinal Detachment )
    Separation of sensory retina from retinal pigment epithelium (RPE) by subretinal fluid.
    1. Types:
      1. MC : Rhegmatogenous                                
      2. PDR : Tractional                                              
      3. Exudative
    2. T/t : Aim is closure of break
      1. Drainage of SRF
      2. vitrectomy
      3. Sealing of break-cryo.laser
      4. Tamponade
  3. Retinoschisis
    Retinoschisis is abnormal splitting of the internal-Air expandable gas (8F6), PFCL Enternal-Bond/Buckle
  4. Bull’s eye maculopathy:
    Central foveolar hyperpigmentation surrounded by depigmented zone and encircled by a hyperpigmented ring. Cause: Chloroquine toxicity*
  5. CME:  (cystoid macular oedema)
    1. Accumulation of fluid in the outer plexiform (Henle’s), centered about on the foveola.
    2. Large cystic space lamellar hole (Decrease in VA)
    3. FA: Flower – petal pattern*
    4. Etiology: honey coml. Appearance on opthalmoscopy.
      a. with retinal vascular leakage: DR, BRVO, intermediate uveitis, aphakic or pseudophakic CME
      b. Without retinal vascular leakage: RP
  6. Central serous retinopathy
    Idiopathic self – limited disease of young or middle – aged adult males
    1. Pathogenesis:
                        Dysfunction of RPE
                        Accumulation of fluid in subretinal space
        Local detachment of sensory retina at macula
  1. C/F:
  1. Sudden onset of blurred vision in one eye
  2. With relative central scotoma
  3. Micropsia
  4. Metamorphopsia
  5. VA is 6/9 6/12 and correctable by addition of plus lens.
  1. On Examination :
    Elevation of sensory retina at posterior pole, borders of which are outlined by glistening reflex.-ring reflex
  1. Smoke – stack appearance* (Typical)
  2. Ink-blot appearance* (Most common)
  1. Treatment
  1. Spontaneous resolution is common (by 6 – 12 months)
  2. Laser photocoagulation for leaks away from fovea.
  3. Subthreshold Photodynamic therapy (PDT)
  1. Myopic fundus-Pathological Myopia
    Mainly affects young males*.
    1. Etiology
      1. Idiopathic
      2. Hypersensitivity to tubercular protein leads to peripheral retinal vasculitis. Presentation: “Recurrent vitreous hemorrhage in young male VH sudden, painless loss of vision
    2. On Examination
      1. Sheathing of small peripheral retinal veins
      2. Massive proliferative retinopathy extensive vitreous or retinal hemorrhage TRD
    3. Treatment
      a. Inflammatony stage : System steroid
      b. Isechemic Stage: PRP Neovascularisation stage : anti vegf intravitrealy
  2. ARMD (Age Related Macular Degeneration)
    Two main types of ARMD are:
    1. Non-Exudative / Dry
      Most common-90% of cases
      1. Clinical Features: Complications sta.
      2. On examinations:
        1. Drusen
        2. Sharply circumscribed circular areas of RPE atrophy associated with varying degrees of loss
        3. Geographic atrophy*.
  1. Pathology   :  Slowly progressive atrophy of RPE and photoreceptors.
  2. Treatment
    1. No effective treatment.     
  1. Exudativ /wet ARMD:
    Less common but effect on vision is more devastating.
    1. On examination:
      1. RPE detachment                
      2. ​Choroidal neovascularization.
    2. Choroidal Neovascularization
      Proliferation of fibrovascular tissue from choriocapillaris through defects in Bruch's membrane into sub-RPE space and later into the sub- retinal space.
      Types :
      1. Classical (seen n FA)
      2. Occult (seen only ICG)
    3. Causes of Neovascular Membrane*
      1. Wet ARMD.
      2. High myopia.
      3. POHS-Presumed ocular histoplasmosis syndrome.
      4. Angioid streaks.
      5. Choroidal rupture.
    4. Treatment of Wet ARMD
  2. Laser photocoagulation
    1. Membranes can be­
      Laser photocoagulation is done for mainly extrafoveal lesions, blue-green argon laser
    2. For sub foveal and extremely juxta-foveal lesions:
      1. PDT-Photodynamic therapy
      2. TTT - Transpupillary Thermo-therapy
      3. Intravitreal anti-VEGF Bevacizumab
  1. Retinopathy of Prematurity (ROP)
    It is a proliferative retinopathy which affects pre-term infants. Incompletely vascularized temporal retina is particularly susceptible to oxygen damage.
Demarcation line
  1. CU
  2. Rop
  3. Chronic RD
It is divided into two types:
  1. Active ROP
  2. Cicatricial ROP
  1. Active ROP: It is divided into four stages:
    1. Stage I: (Demarcation line) : It separates avascular immature peripheral retina from vascularized posterior retina.
    2. Stage II: (Ridge):
    3. Stage III:  (Ridge with extra-retinal fibrovascular proliferation) Generally this stage occurs
    4. Stage IV: (Sub-total Retinal Detachment) : Tractional RD
    5. Stage V: Total Retinal Detachment.
  2. Treatment of ROP:
    1. Ablation of avascular immature retina by laser photocoagulation.
    2. If retinal detachment is present Scleral Buckling.
  3. Screening of ROP: Eyes of all infants < 32 weeks or weighing less than 1500 g who have received supplemental oxygen should be screened for ROP.
    The most useful time is between 32 weeks and 36 weeks.
  1. Toxic maculopathy
    It can be caused by following drugs*
    1. Chloroquine
    2. Quinine
    3. Phenothiazine.
      1. Chlorpromazine.                  
      2. ​Thioridazine.
    4. Tamoxifen.
  2. Leber's Amaurosis
    Clinical Features
    1. Blindness at birth or within first few years of life.
    2. Pupillary light reactions are absent or severely diminished.
    3. A characteristic feature is oculo digital syndrome* eye poking child causes enophthalmos as a result of resorption of fat.
    4. ERG* not recordable
  3. Dystrophies of the retinal pigment epithelium
    1. Best's Vitelliform Macular Dystrophy
      1. Diagnosis
        Lipofusin (ageing pigment) accllmulation is RPE at macula Loss of central vision. 
        ERG Normal
        EOG alnormal
        1. "Egg-yolk lesion" or "Sunny side up*"
        2. Scranbled egg appearance
  1. Stargardt’s Macular Dystrophy-Fundus Flavimaculatus
    Retinal pigment dystrophies at the macula beaten –brongy appearance
  1. Choroidal dystrophies
  1. Choroideremia
  1. Affect only males with female carriers..
  2. Night blindness. *
  1. Gyrate Atrophy
  1. Due. to deficiency of mitochondrial matrix enzyme--Ornithine Aminotransferase.
  2. Night Blindness
  3. Development of axial myopia
  4. Treatment
  1. Pyridoxine (Vitamin B6) *.
  2. A diet low in proteins and arginine*.

ICG angiography is primarily indicated in (AIPG 09)
A. Minimal classical CNV                                    
B. Occult CNV
C. Angioid streaks with CNV                              
D. Polypoidal choroidal vasculopathy


Ans: B. Occult CNV

  1. Retinoblastoma:
    1. Most common primary intraocular malignancy in children
    2. Average age of diagnosis is 18 months
    3. Inheritance – AD, but only 6% cases are familial *
    4. Familial cases
      1. early onset,
      2. Bilateral involvement
      3. Predisposed to develop second non ocular malignancy including pinealoblastoma and osteogenic sarcoma
      4. B/L Retinoblastoma with pinealoblastoma is termed as trilateral retinoblastoma
      5. Rb gene : long arm of chromosome 13 (13Q)
      6. It arises form primitive multipotential neuroectodermal cells of retina
      7. 13q syndrome*- Retinoblastoma when associated with other dysmorphic features ( Microcephaly)  broad nasal bridge, hypertelorism) and mental handicap.*
  1. C/F.: Mode of presentations are:
    1. Leukocoria 60%-most common                                                                
    2. Strabismus 20%
  2. C/F:  I/O with scleral indentation should be performed in both eyes. Growth may be
    1. Exophytic   - Mimic coats disease
    2. Endophytic - Mimic endophthalmitis (most common)
      1. Mixed most common rite of metastases : CNS (Brain)
  • D/D: {D/D of Leukocoria}
  1. PHPV                                                                
  2. ROP                                                  
  3. Toxocariasis                     
  4. Coats Disease                                                  
  5. Central Coloboma                          
  6. Retinal astrocytomag.     
  7. Congenital cataract.                                        
  8. Fungal endophthalmitis                
  9. Cyclitic membrane           
  10. Retinal dysplasia.
The tumour is composed of small basophilic cells (retinoblasts) with large hyperchromatic nuclei and scanty cytoplasm. Many retinoblastomas are undifferentiated but varying degrees of differentiation are characterized by the formation of rosettes, of which there are three types:
  1. Flexner – Wintersteiner  Rosettes consist of a central lumen surrounded by tall columnar cells. The nuclei of these cells lie away from the lumen.
  2. Homer – Wright Rosettes have no lumen and the cells form around a tangled mass of eosinophilic process.
  3. Flurettes are foci of tumour cells, which exhibit photoreceptor differentiation. Clusters of cells with long cytoplasmic processes project through a fenestrated membrane and the appearance resembles a bouquet of flowers.
Indirect ophthalmoscopy with scleral indentation must be performed on both eyes after full mydriasis. This is because without indentation pre-equatorial tumors may be missed and one eye may harbour multiple tumours. The clinical signs depend on tumour size and growth pattern.
  1. An intraretinal tumour is a homogenous, dome-shaped white lesion which becomes irregular, often with white flecks of calcification.
  2. An endophytic tumour projects into the vitreous as a white mass that may seed in to the vitreous.
  3. An exophytic tumour forms subretinal, multilobulated white masses, with overlying retinal detachment.
  1. US is used mainly to assess tumour size. It also detects calcification within the tumour and is helpful in the diagnosis of simulating lesions such as Coats disease.
  2. CT also detects calcification but entails a significant dose of radiation and is performed only if US has not detected calcification.
  3. MR cannot detect calcification but it is superior to CT for optic nerve evaluation and detection of extraocular extension or a pinealoblastoma, especially with contrast and fat suppression.
  4. Systemic investigations include physical examination and MR scans of the orbit and skull, as a minimum in high-risk cases. If these indicate the presence of metastatic disease, then bone scans, bone marrow aspiration and lumbar puncture are also performed.
  5. Genetic studies require fresh tumor tissue from the enucleated eye and a blood sample for DNA analysis. 
Staging of Retinoblastoma for planning. treatment and prognostication
RPC-IRCH Classification of Retinoblastoma not used now (International classification of RB is used).
  1. Stage I
    1. A: One/more lesion up to 3DD, 3 mm ht
    2. B: With vitreous seeds.
  2. Stage II
    1. A: One/more lesion 4-6 DD, 4-6 mm ht
    2. B: With vitreous seeds.
  3. Stage III
    1. A: One /more lesion> 6DD, >6mm ht
    2. B: With vitreous seeds.
  4. Stage IV Extraocular spread confined to the orbit
  5. Stage V Distant metastasis.
Recent Advances:
Table. International Classification of Retinoblastoma
Group                                     Features
A                                           Small tumor: ≤3 mm
                                             Large tumor: >3 mm
B                                           Macular: ≤3 mm to foveola
                                             Juxtapapillary: ≤3 mm to disc
                                             Subretinal fluid: ≤3 mm from the margin
                                             Focal seeds
C                                           Subretinal seeds: ≤3 mm
                                             Vitreous seeds: ≤3 mm
                                             Both subretinal and vitreous seeds: ≤3 mm
                                             Diffused seeds
D                                           Subretinal seeds: >3 mm
                                             Vitreous seeds: >3 mm
                                             Both subretinal and vitreous seeds: >3 mm
E                                           Extensive retinoblastoma occupying more than 50% or                                                            neovascular glaucoma or opaque media from hemorrhage in                                                    anterior chamber, vitreous or subretinal space

Treatment of small tumours
Small tumours, no more than 3mm diameter and 2mm thickness, may be treated as follows:
  1. Photocoagulation using a low-energy 532nm argon or 810nm diode laser achieves focal consolidation after chemotherapy. At least three treatment sessions are needed but excessive laser energy can cause vitreous seeding.
  2. Cryotherapy using the triple freeze-thaw technique is useful for pre-equatorial tumours without either deep invasion or vitreous seeding, repeated treatment may be necessary. Complications include vitreous haemorrhage, retinal detachment and scleral thinning.
  3. Chemotherapy with other treatment can be attempted for a macular tumour, to conserve as much vision as possible, but there is an increased risk of tumour recurrence.
Treatment of medium-sized tumours
Medium sized tumours, up to 12mm diameter and 6 mm thickness, may be treated as follows:
  1. Brachytherapy using iodine-125 or ruthenium-106 is indicated for an anterior tumour if there is no vitreous seeding.
  2. Primary chemotherapy with intravenous carboplatin, etoposide and vincristine (CEV) is given in three to six cycles according to the grade of retinoblastoma.
  3. External beam radiotherapy is avoided, if possible in patients with heritable retinoblastoma because of the risk of inducing a second malignancy such as osteosarcoma.
Treatment of large tumours:
  1. Chemotherapy to shrink the tumour (chemo reduction) facilitating subsequent local treatment, thereby avoiding enucleating or external beam radiotherapy, Chemotherapy also will have a beneficial effect if a smaller tumour is present in the fellow eye or if there is a pinealoblastoma.
  2. Enucleation is indicated if there is rubeosis, vitreous haemorrhage or optic nerve invasion. It is also performed if chemo reduction fails or a normal fellow eye makes aggressive chemotherapy inappropriate. It is also useful for diffuse retinoblastoma because of poor visual prognosis and high risk of risk of recurrence with other therapeutic modalities.
Treatment of extraocular extension:
  1. Adjuvant chemotherapy consisting of a 6-month course of CEV may be given after enucleation if there is retro laminar or massive choroidal spread.
  2. External beam radiotherapy is indicated when there is tumour extension to the cut end of the optic nerve at enucleation, or extension through the sclera.
Treatment of metastatic disease:
Metastatic disease is treated according to its extent using various techniques, which include high dose chemotherapy, intrathecal chemotherapy, myeloablative therapy with bone marrow rescue, focal, cranio spinal or total body radiotherapy. Patients with malignant cells in the cerebrospinal fluid may require intrathecal methotrexate.

Differential diagnosis
  1. Coats disease is unilateral, more common in boys and tends to present later than retinoblastoma.
  1. Focal therapies
    • include laser photocoagulation, thermotherapy, cryotherapy, and plaque radiotherapy.
    • Most of these therapies are employed for small tumors, especially those that have been reduced by chemoreduction.
    • Commonly, focal therapies are applied to an eye while the child is receiving chemoreduction, and they are repeated to each tumor at each chemotherapy session.
    • Plaque radiotherapy is generally reserved for tumors that fail other focal therapies, even those that reach a moderate size, up to 8 or 10 mm in thickness.
    • The remainder of the focal therapies are reserved for small tumors, generally those under 3 mm in greatest dimension. Laser photocoagulation is usually employed for small retinoblastomas posterior to the equator of the eye.
  2. Radiotherapy
    1. ​​Brachytherapy*:
      1. The advantage is that the rest of the eye receives a lower dose of radiation and thus the complications are less.
      2. Effective for stage II and stage III.
      3. Dose is 20-40 Gy which may be lower if prior chemotherapy is given.
  1. EBRT -(linear accelerator)
    1. Dose required is 40-50 Gy
    2. Platinum based chemotherapy, 6 months prior to radiotherapy enhances the effect of radiation.
  2. Chemotherapy
    1. Primary neo-adjuvant chemotherapy
      1. It plays role of chemo-reduction, where the size of tumour is decreased making it amenable
      2. to direct local therapy.
      3. Drugs used are:
  • Etoposide       *   - Very good ocular   penetration'
  • Carboplatin    *   -  Very good ocular   penetration'
  • Vincristine*
  • Teniposide*
VEC regiment
  1. Vincristine
  2. Etoposide
  3. Carboplatin
  1. Adjuvant chemotherapy:
    It has a role in cases with extensive disease and in preventing metastasis (in cases with risk factors)
    1. Drugs used are:
      Etoposide, carboplatin, vincristine, cyclophosphamide, cyclosporin
  2. Local chemotherapy:
    Studies with subconjunctival carboplatin and intraocular chemotherapeutic agents are being   conducted and results awaited.
  1. Surgical
    1. Enucleation:
      Implants-Medpor, hydroxyapatite, PMMA
    2. Exenteration: In orbital retinoblastoma, exenteration is done followed by radiotherapy. Once
    3. tumour breaches the sclera and extends to the orbit, prognosis for life is very dismal in spite of   aggressive treatment.

Recent Advances
Fluorescein angiography is used primarily to study blood circulation in and just beneath the surface of the retina, while ICG angiography is better for photographing the deeper choroidal vessels.
Use of ICG : a. Age-related macular degeneration are caused by leakage from the deeper choroidal blood vessels esp occult CNVM

  1. Retinal Telangiectasias
    They are a group of rare, idiopathic, congenital retinal vascular anomalies affecting the retinal capillaries.
    The conditions are characterized by dilation and tortuosity of retinal vessels and formation of multiple aneurysms
    1. Idiopathic JXT                            
    2. Leber's miliary aneurysms                            
    3. Coats' disease
  1. Idiopathic juxtafoveolar retinal telangiectasia (JXT) is commonly characterized by slow vision loss beginning in adulthood.
  2. Leber's miliary aneurysms represent a more severe form of telangiectasia.
    1. Patients often present in middle-age with a drop in VA. Fundus (retinal) findings include lipid deposition at the macula and dilation of arterioles and venules.
    2. Cryotherapy or photocoagulation may be needed to destroy the area of vascular abnormality. 
  3. Coats' Disease is a very severe, unilateral form of retinal telangiectasia that is most often seen in boys before age 10.
    1. Patients often present with strabismus (an eye turn) or leukocoria (a white pupil).
    2. The differential diagnoses include retinoblastoma (a malignant tumor of the retina that causes a white pupil) and retinopathy of prematurity.
    3. The disease is characterized by large areas of dilated, tortuous retinal blood vessels which overlie intra- and sub-retinal yellow exudate at the posterior pole (the optic nerve and macular areas) and in the periphery.
    4. Retinal edema may also be persistent, causing decreased acuity when it reaches the macula.
    5. Ischemia or decreased blood flow may develop as well, with neovascularization and subsequent vitreal hemorrhage and retinal detachment.
    6. Spontaneous resolution is rare and the disease often progresses to massive sub-retinal exudation, exudative retinal detachment, rubeosis iridis, secondary cataract, and secondary glaucoma.
    7. Treatment includes cryotherapy and photocoagulation to destroy the abnormal vessels and to prevent retinal detachment. 
  1. Retinal Degenerative Diseases
Retinal Degenerative Diseases
Best Disease (Vitelliform Dystrophy) This disease is characterized by a lesion in the macula, which leads to impaired central vision in one or both eyes. It is an autosomal dominant disease.
Stargardt Disease/Fundus Flavimaculatus This form of macular degeneration usually appears before the age of 20. It is characterized by a reduction of central vision with a preservation of peripheral vision. In most affected families, Stargardt and fundus flavimaculatus are autosomal recessive diseases, although autosomal dominant families have been identified.
Macular Degeneration Macular degeneration is divided into two broad categories: early onset and age-related. Early onset forms are inherited macular degenerations that include Best disease, Stargardt disease, fundus flavimaculatus and other rare macular dystrophies like Sorsby's macular dystrophy and North Carolina macular dystrophy. Age-related macular degeneration is the leading cause of central vision loss in people over the age of 60. The first symptom is usually a blank spot in the center of the visual field or a distortion of normal central vision. Its inheritance pattern is usually unknown. It is sometimes seen as an autosomal dominant disease in some families.
Bardet-Biedl (Laurence-Moon) Syndrome The Features include RP, extra fingers and/or toes, obesity, mental retardation and kidney disease. Bardet-Biedi syndrome is an autosomal recessive disease.
Bassen-Kornzweig Syndrome (Abetalipoproteinemia) RP and progressive neurologic problems are symptoms of this disease. Patients also have oddly shaped red blood cells. Bassen-Kornzweig syndrome is an autosomal recessive disease.
Choroideremia Choroideremia has symptoms similar to RP, including night blindness followed by loss of peripheral vision. It is characterized by degeneration of the retina and of the choroid. It has an X-linked inheritance pattern and affects males. Female carriers may experience mild symptoms of the disease.
Gyrate Atrophy This retinal degenerative disease is associated with a deficiency in the enzyme ornithine aminotransferase. Myopia, night blindness, reduction in peripheral vision and cataracts are characteristic of this syndrome. Gyrate atrophy is an autosomal recessive disease.
Leber Congenital Amaurosis Leber congenital amaurosis is characterized by severe visual impairment from birth or very early childhood. It is an autosomal recessive disease.
Refsum Syndrome Refsum syndrome is due to the absence of phytanic acid hydroxylase,. In addition to having RP, patients may have hearing loss, thickened nerves, neurologic problems and dry and/or flaky skin. It is an autosomal recessive disease.
Retinoschisis (Juvenile) In this disease, the layers of the retina separate, and the macula may also be affected. Juvenile retinoschisis has X-linked inheritance pattern and affects males.
Usher Syndrome The combination of RP and congenital sensorineusal hearing loss.


A young man presents with decreased vision in left eye. After three month decrease vision in right eye. Fundus show parafoveal telangiectasia & disc hyperemia with reflex normal. Most probable diagnosis is. (AIIMS May 09)
A. AION                                                 
B. Leber hereditary      
C. Pappilodema                                   
D. Toxic neuropathy


Ans. B. Leber hereditary neuropathy


Raised LDH levels in Aqueous is seen in (AIPG 09)
A. Galactosemia                   
B. Glaucoma                         
C. Hemangioblastoma                         
D. Retinoblastoma


Ans: D. 

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