Diseases of the Retina Practice Questions

Q: Angioid streaks occur in which of the following conditions?

Sickle cell disease. **Explanation:** **Angioid streaks** are jagged, radiating cracks in a thickened, calcified, and brittle **Bruch’s membrane**. They typically emanate from the optic disc and resemble blood vessels (hence "angioid"), but are actually dehiscences in the elastic lamina of Bruch's membrane. **Why Sickle Cell Disease is Correct:** Sickle cell disease (and other hemoglobinopathies like Thalassemia) is a classic systemic association. The underlying mechanism involves chronic hemolysis leading to iron deposition (hemosiderosis) in Bruch’s membrane, which increases its brittleness and leads to the characteristic linear streaks. **Analysis of Incorrect Options:** * **Sturge-Weber Syndrome:** Characterized by "tomato ketchup" fundus due to diffuse choroidal hemangiomas, not Bruch’s membrane pathology. * **Pseudoexfoliation Syndrome:** A systemic condition involving the deposition of fibrillar material in the anterior segment (lens capsule, iris), primarily leading to secondary open-angle glaucoma. * **Septo-optic Dysplasia (De Morsier Syndrome):** A congenital malformation involving optic nerve hypoplasia and midline brain defects; it does not involve retinal streaks. **High-Yield Clinical Pearls (NEET-PG):** To remember the major causes of Angioid Streaks, use the mnemonic **PEPSI**: * **P:** **P**seudoxanthoma elasticum (Most common association; "plucked chicken" skin appearance). * **E:** **E**hlers-Danlos syndrome. * **P:** **P**aget’s disease of the bone. * **S:** **S**ickle cell disease / Thalassemia. * **I:** **I**diopathic. **Clinical Significance:** The most vision-threatening complication of angioid streaks is the development of **Choroidal Neovascularization (CNVM)**, which can lead to subretinal hemorrhage and permanent central vision loss.

Q: Which of the following conditions can premature babies develop?

Retinopathy of prematurity. **Explanation:** **Retinopathy of Prematurity (ROP)** is the correct answer because it is a vasoproliferative disorder specifically linked to premature birth and low birth weight. In a fetus, retinal vascularization begins at the optic disc at 16 weeks and reaches the nasal periphery by 36 weeks and the temporal periphery by 40 weeks. Premature birth interrupts this process, leaving the peripheral retina avascular. Subsequent exposure to high oxygen levels (hyperoxia) followed by relative hypoxia triggers the release of **VEGF (Vascular Endothelial Growth Factor)**, leading to abnormal neovascularization. **Analysis of Incorrect Options:** * **Buphthalmos (A):** This refers to the enlargement of the globe seen in **Congenital Glaucoma** due to high intraocular pressure. It is a developmental defect of the angle of the anterior chamber, not specifically associated with prematurity. * **Increased incidence of myopia (C):** While premature infants *do* have a higher risk of refractive errors (including myopia and astigmatism) later in life, ROP is the primary, acute, and most characteristic condition directly resulting from the pathophysiology of preterm birth. * **Persistent hyaloid artery (D):** This is a failure of the fetal hyaloid vascular system to regress during the third trimester. While it is a congenital anomaly, it is not a consequence of premature birth itself. **High-Yield Clinical Pearls for NEET-PG:** * **Screening Criteria:** All babies born **<32 weeks** gestation or with a birth weight **<1500g** must be screened for ROP. * **Zone I** (centered on the disc) is the most critical area; disease here is more severe. * **Plus Disease:** Characterized by dilatation and tortuosity of retinal vessels; it indicates active, severe ROP. * **Treatment:** Laser photocoagulation or Intravitreal Anti-VEGF (e.g., Ranibizumab) are the mainstays for Type 1 ROP.

Q: The "pizza pie" appearance of the retina is characteristic of which of the following conditions?

CMV retinitis. **Explanation:** **CMV Retinitis (Correct Answer):** Cytomegalovirus (CMV) retinitis is the most common opportunistic ocular infection in patients with AIDS (typically when CD4 counts fall below 50 cells/mm³). The characteristic **"Pizza Pie"** or **"Cottage cheese and ketchup"** appearance refers to the fundoscopic finding of dense, white perivascular retinal opacification (representing necrosis and edema) interspersed with prominent retinal hemorrhages. It typically spreads along the retinal vessels in a "brushfire" pattern. **Analysis of Incorrect Options:** * **Toxoplasmosis:** Characteristically presents as a **"Headlight in the fog"** appearance, which refers to an active yellowish-white fluffy lesion (retinitis) seen through a hazy vitreous caused by intense vitritis. * **Congenital Rubella:** Classically associated with a **"Salt and pepper" retinopathy**, characterized by diffuse, mottled pigmentary changes in the retinal pigment epithelium (RPE), usually without affecting vision significantly. * **Drug Toxicity:** Various drugs cause specific patterns (e.g., Chloroquine/Hydroxychloroquine causes **"Bull’s eye maculopathy"**; Thioridazine causes "nummular" pigmentary changes), but none present with the hemorrhagic necrotic pattern of CMV. **High-Yield Clinical Pearls for NEET-PG:** * **Treatment of Choice:** Intravenous **Ganciclovir** (or Valganciclovir). Foscarnet and Cidofovir are alternatives. * **Frosted Branch Angiitis:** Another clinical variant of CMV retinitis characterized by severe perivascular sheathing. * **Key differentiator:** Unlike Toxoplasmosis, CMV retinitis typically presents with **minimal vitritis** ("cold" endophthalmitis) because the patient is severely immunocompromised.

Q: Hard exudates are not seen in which of the following conditions?

Toxemia of pregnancy. **Explanation:** Hard exudates are extracellular deposits of lipids and serum proteins in the outer plexiform layer of the retina. They result from **chronic vascular leakage** due to a breakdown of the blood-retinal barrier. **Why Toxemia of Pregnancy is the Correct Answer:** In **Toxemia of Pregnancy (Preeclampsia/Eclampsia)**, the primary pathology is acute, intense arteriolar spasm. The retinal findings typically include generalized or focal arteriolar narrowing, retinal edema, and in severe cases, exudative retinal detachment. Because the condition is **acute**, there is usually insufficient time for the chronic process of lipid deposition required to form hard exudates. **Analysis of Incorrect Options:** * **Diabetes Mellitus:** This is the most common cause of hard exudates. Microaneurysms and damaged capillaries leak fluid and lipids chronically, often forming a "circinate" pattern around the site of leakage. * **Hypertension:** Chronic hypertensive retinopathy (Grade 3 and 4) involves significant vascular permeability changes. Hard exudates are a hallmark feature, often accumulating in the Henle’s layer of the macula to form a **"Macular Star."** **High-Yield Clinical Pearls for NEET-PG:** * **Hard Exudates:** Located in the **Outer Plexiform Layer**; composed of lipoproteins; indicate chronic leakage. * **Soft Exudates (Cotton Wool Spots):** Located in the **Nerve Fiber Layer**; represent micro-infarctions (ischemia); seen in both Hypertension and Diabetes. * **Macular Star:** Differential diagnosis includes Hypertensive retinopathy, Neuroretinitis (Cat-scratch disease), and Papilledema. * **Elschnig Spots:** Small black spots surrounded by yellow halos seen in hypertensive choroidopathy (advanced toxemia/hypertension).

Q: Rubeosis iridis is NOT COMMONLY seen in:

CRAO (Central Retinal Artery Occlusion). **Explanation:** The development of **Rubeosis Iridis** (neovascularization of the iris) is driven by chronic retinal ischemia, which triggers the release of **Vascular Endothelial Growth Factor (VEGF)**. This factor diffuses into the anterior segment, stimulating the growth of abnormal vessels on the iris. **Why CRAO is the correct answer:** In **Central Retinal Artery Occlusion (CRAO)**, the blood supply to the inner retina is completely cut off, leading to rapid cell death (infarction). Dead retinal tissue cannot produce VEGF. Therefore, while rubeosis can occur in rare cases (approx. 2-5%), it is **not commonly** seen compared to other ischemic conditions. **Analysis of other options:** * **CRVO (Central Retinal Vein Occlusion):** Specifically the **Ischemic type**, this is the most common cause of rubeosis iridis. It is famously associated with "100-day glaucoma" (Neovascular Glaucoma). * **Diabetic Retinopathy:** Proliferative Diabetic Retinopathy (PDR) is a leading cause of rubeosis due to widespread, chronic retinal capillary non-perfusion. * **Neovascularization:** This is a general term for the process itself. Rubeosis iridis *is* neovascularization of the iris; therefore, it is inherently associated with the process. **NEET-PG High-Yield Pearls:** 1. **Most common cause of Rubeosis Iridis:** Diabetic Retinopathy. 2. **Most common cause of Neovascular Glaucoma (NVG):** Ischemic CRVO. 3. **100-Day Glaucoma:** Refers to NVG occurring roughly 3 months after an ischemic CRVO event. 4. **CRAO Clinical Sign:** "Cherry red spot" at the macula due to the visible choroid through thin foveal tissue amidst surrounding retinal edema.

Q: Roth's spots are seen in which of the following conditions?

Bacterial Endocarditis. **Explanation:** **Roth’s spots** are classic retinal findings characterized by **hemorrhages with a pale, white, or fibrin center**. 1. **Why Bacterial Endocarditis is correct:** The underlying pathophysiology involves a **Type III hypersensitivity reaction** (immune-complex mediated vasculitis). In Subacute Bacterial Endocarditis (SBE), circulating immune complexes deposit in the retinal capillary walls, leading to rupture (hemorrhage) and subsequent focal ischemia or inflammatory infiltration at the center, which creates the characteristic "white spot." 2. **Why other options are incorrect:** * **Trauma:** While trauma can cause various retinal hemorrhages (e.g., Purtscher's retinopathy), it does not typically present with the specific white-centered Roth's spots unless associated with secondary complications. * **Coats Disease:** This is an idiopathic condition characterized by telangiectatic retinal vessels and massive **subretinal exudation** ("light bulb" appearance), not white-centered hemorrhages. * **Trachoma:** This is a chronic keratoconjunctivitis affecting the **external eye** (conjunctiva and cornea), leading to Arlt’s lines and Herbert’s pits; it does not involve the retina. **High-Yield Clinical Pearls for NEET-PG:** * **Differential Diagnosis of Roth’s Spots:** Beyond Endocarditis, remember the mnemonic **"LIME"**: **L**eukemia (most common cause), **I**llness (Anemia), **M**ellitus (Diabetes), and **E**ndocarditis. * **Composition:** The white center is typically composed of **fibrin-platelet aggregates**, though in leukemia, it may consist of malignant white blood cells. * **Other Retinal Spots:** Do not confuse Roth’s spots with **Elschnig spots** (hypertensive choroidopathy) or **Cotton wool spots** (nerve fiber layer infarcts).

Q: In which of the following diseases is the eye finding shown below seen?

GM1 gangliosidosis. ***GM1 gangliosidosis*** - The **cherry-red spot** seen in the macula is a pathognomonic finding in **GM1 gangliosidosis**, caused by **ganglioside accumulation** in retinal ganglion cells. - This lysosomal storage disorder results in **neurodegeneration** with the fovea appearing red against the surrounding pale retina due to **lipid deposits**. *Gaucher disease* - Typically presents with **pingueculae** (yellowish conjunctival deposits) rather than cherry-red spots. - Ocular manifestations are less common and do not involve the **macular region** with cherry-red appearance. *Von Gierke disease* - This **glycogen storage disease** primarily affects the liver and does not cause **cherry-red spots**. - Ocular findings are not a characteristic feature of this **glucose-6-phosphatase deficiency**. *Galactosemia* - The classic ocular finding is **bilateral cataracts**, particularly **oil drop cataracts** in infants. - Does not cause **cherry-red spots** but rather lens opacification due to **galactitol accumulation**.

Q: What is true about Birdshot Chorioretinopathy?

Bilateral presentation. **Explanation:** **Birdshot Chorioretinopathy (BSCR)** is a rare, chronic, and potentially blinding form of posterior uveitis. 1. **Why Option B is Correct:** Birdshot Chorioretinopathy is characteristically a **bilateral**, symmetrical disease. It is an autoimmune inflammatory condition where the body attacks the choroid and retina. The hallmark clinical finding is the presence of multiple, cream-colored, oval "birdshot" spots (hypopigmented lesions) scattered throughout the fundus, typically starting around the optic disc and radiating towards the periphery. 2. **Why Other Options are Incorrect:** * **Option A:** It is almost never unilateral; bilateral involvement is a diagnostic requirement. * **Option C:** The onset is typically **chronic and insidious**. Patients often present with gradual blurring of vision, floaters, and night blindness (nyctalopia) rather than an acute crisis. * **Option D:** There is a slight **female predilection**, and it most commonly affects Caucasian individuals in their 5th to 6th decades of life. **High-Yield Clinical Pearls for NEET-PG:** * **HLA Association:** This is the strongest known HLA-disease association in ophthalmology. Over 95% of patients are **HLA-A29 positive**. * **Fundus Fluorescein Angiography (FFA):** Often shows retinal vasculitis and cystoid macular edema (CME), which is the most common cause of vision loss. * **Indocyanine Green (ICG) Angiography:** Superior to FFA for visualizing the "birdshot" lesions, which appear as hypofluorescent spots. * **Treatment:** Requires long-term systemic immunosuppression (e.g., Corticosteroids, Cyclosporine) to prevent progressive retinal atrophy.

Q: Snow banking is typically seen in which of the following conditions?

Pars planitis. **Explanation:** **Pars planitis** is a specific subset of intermediate uveitis characterized by idiopathic inflammation of the *pars plana* (the posterior part of the ciliary body). The hallmark clinical sign of this condition is **"Snow banking,"** which refers to the accumulation of white, exudative inflammatory material (fibroglial masses) over the inferior pars plana and ora serrata. This is often accompanied by **"Snowballs,"** which are inflammatory cells clumped in the vitreous. **Analysis of Options:** * **Endophthalmitis:** This is a severe intraocular inflammation (usually bacterial or fungal) involving the vitreous and aqueous humors. It presents with hypopyon, severe pain, and vision loss, but not localized snow banking. * **Coat’s Disease:** An idiopathic condition characterized by telangiectatic retinal vessels and massive **subretinal exudation** (hard exudates), typically seen in young males. It does not involve pars plana exudates. * **Eale’s Disease:** An idiopathic peripheral inflammatory venulitis (perivasculitis) that leads to peripheral non-perfusion, neovascularization, and recurrent **vitreous hemorrhage**. It is not associated with snow banking. **High-Yield Clinical Pearls for NEET-PG:** * **Most common complication:** Cystoid Macular Edema (CME) is the leading cause of vision loss in Pars planitis. * **Demographics:** Typically affects children and young adults; often bilateral (80%). * **Associations:** While idiopathic by definition, intermediate uveitis can be associated with **Multiple Sclerosis** and **Sarcoidosis**. * **Treatment:** Steroids (Periocular/Systemic) are the first line; Cryotherapy or Laser photocoagulation is used for the snow bank area if complications arise.

Q: Retinitis pigmentosa is associated with which of the following conditions, except?

Hallervorden-Spatz disease. **Explanation:** Retinitis Pigmentosa (RP) is a genetically heterogeneous group of rod-cone dystrophies. While most cases are isolated, approximately 25% are associated with systemic syndromes. **Why Hallervorden-Spatz disease is the correct answer:** Hallervorden-Spatz disease (now known as **Pantothenate Kinase-Associated Neurodegeneration or PKAN**) is a neurodegenerative disorder characterized by iron accumulation in the basal ganglia. Its hallmark ocular finding is **"Bull’s eye maculopathy"** rather than the typical pigmentary retinopathy (bone spicules) seen in Retinitis Pigmentosa. **Analysis of Incorrect Options:** * **Refsum’s Disease:** A metabolic disorder of phytanic acid alpha-oxidation. It presents with the classic pentad: RP, peripheral neuropathy, cerebellar ataxia, nerve deafness, and ichthyosis. It is a high-yield "treatable" cause of RP via dietary restriction. * **NARP Syndrome:** Stands for **N**eurogenic muscle weakness, **A**taxia, and **R**etinitis **P**igmentosa. It is a mitochondrial DNA disorder. * **Abetalipoproteinemia (Bassen-Kornzweig Syndrome):** Characterized by fat malabsorption, acanthocytosis, and RP. It is another treatable cause of RP (high-dose Vitamin A and E supplementation). **High-Yield Clinical Pearls for NEET-PG:** 1. **Usher Syndrome:** The most common systemic association of RP (RP + Sensorineural hearing loss). 2. **Bardet-Biedl Syndrome:** RP + Polydactyly + Obesity + Hypogonadism + Renal anomalies. 3. **Kearns-Sayre Syndrome:** RP + Chronic Progressive External Ophthalmoplegia (CPEO) + Heart block. 4. **Fundus Findings in RP:** Arteriolar attenuation, Bone-spicule pigmentation, and Waxy pallor of the optic disc. 5. **Earliest Symptom:** Nyctalopia (Night blindness); **Earliest Sign:** Ring scotoma on perimetry.

Question 1

Angioid streaks occur in which of the following conditions?

Accepted Answer

Sickle cell disease

Answer

Sturge-Weber's syndrome

Answer

Pseudoexfoliation syndrome

Answer

Septo-optic dysplasia

Question 2

Which of the following conditions can premature babies develop?

Accepted Answer

Retinopathy of prematurity

Answer

Buphthalmos

Answer

Increased incidence of myopia

Answer

Persistent hyaloid artery

Question 3

The "pizza pie" appearance of the retina is characteristic of which of the following conditions?

Accepted Answer

CMV retinitis

Answer

Toxoplasmosis

Answer

Congenital rubella

Answer

Drug toxicity

Question 4

Hard exudates are not seen in which of the following conditions?

Accepted Answer

Toxemia of pregnancy

Answer

Hypertension

Answer

Diabetes Mellitus

Answer

None of the above

Question 5

Rubeosis iridis is NOT COMMONLY seen in:

Accepted Answer

CRAO (Central Retinal Artery Occlusion)

Answer

CRVO (Central Retinal Vein Occlusion)

Answer

Diabetic retinopathy

Answer

Neovascularization

Question 6

Roth's spots are seen in which of the following conditions?

Accepted Answer

Bacterial Endocarditis

Answer

Trauma

Answer

Coats disease

Answer

Trachoma

Question 7

In which of the following diseases is the eye finding shown below seen?

Accepted Answer

GM1 gangliosidosis

Answer

Gaucher disease

Answer

Von Gierke disease

Answer

Galactosemia

Question 8

What is true about Birdshot Chorioretinopathy?

Accepted Answer

Bilateral presentation

Answer

Unilateral presentation

Answer

Acute onset

Answer

Most commonly seen in males

Question 9

Snow banking is typically seen in which of the following conditions?

Accepted Answer

Pars planitis

Answer

Endophthalmitis

Answer

Coat's disease

Answer

Eale's disease

Question 10

Retinitis pigmentosa is associated with which of the following conditions, except?

Accepted Answer

Hallervorden-Spatz disease

Answer

Refsum's disease

Answer

NARP syndrome

Answer

Abetalipoproteinemia

Diseases of the Retina — MCQs

Diseases of the Retina — MCQs

On this page

Practice by Chapter

Want unlimited practice?