Diseases of the Retina Practice Questions

Q: The Reese-Ellsworth classification categorizes which of the following tumors with respect to numbers, size, and location?

Retinoblastoma. **Explanation:** The **Reese-Ellsworth (R-E) classification** was historically the primary system used to predict the prognosis for **eye salvage** (vision preservation) in patients with **Retinoblastoma** treated with external beam radiotherapy (EBRT). 1. **Why Retinoblastoma is correct:** The R-E classification divides Retinoblastoma into five groups (Group I to V) based on the **number** of tumors, their **size** (measured in disc diameters), and their **location** (equatorial vs. vitreous seeding). Group I (Very Favorable) typically involves small, solitary tumors, while Group V (Very Unfavorable) involves massive tumors involving over half the retina or vitreous seeding. * *Note:* In modern practice, the **International Classification of Retinoblastoma (ICRB)** has largely replaced R-E as it better predicts success with systemic chemotherapy. 2. **Why other options are incorrect:** * **Rhabdomyosarcoma:** This is the most common primary orbital malignancy in children. It is classified using the TNM system and clinical grouping (IRSG), not R-E. * **Optic Nerve Sheath Tumor/Meningioma:** These are orbital/neurological tumors. Meningiomas (e.g., optic nerve sheath meningioma) are staged based on imaging and extent of intracranial spread, often associated with Neurofibromatosis type 2. **High-Yield Clinical Pearls for NEET-PG:** * **Most common intraocular tumor of childhood:** Retinoblastoma. * **Most common presenting sign:** Leukocoria (white pupillary reflex), followed by strabismus. * **Genetics:** Mutation in the **RB1 gene** on chromosome **13q14**. * **Pathology:** Look for **Flexner-Wintersteiner rosettes** (highly specific) and Homer-Wright rosettes. * **Calcification:** Dystrophic calcification is a hallmark feature seen on B-scan USG or CT.

Q: Which antiviral drug is used for the treatment of cytomegalovirus retinitis?

Ganciclovir. **Explanation:** **1. Why Ganciclovir is the Correct Answer:** Cytomegalovirus (CMV) retinitis is the most common opportunistic ocular infection in patients with AIDS (typically when CD4 counts fall below 50 cells/µL). **Ganciclovir** is a nucleoside analogue that specifically inhibits CMV DNA polymerase. It is the gold standard treatment and can be administered intravenously, orally (Valganciclovir), or via intravitreal injection/implant. Other drugs used for CMV retinitis include **Foscarnet** and **Cidofovir**. **2. Why the Other Options are Incorrect:** * **Acyclovir (Option A):** While effective against Herpes Simplex Virus (HSV) and Varicella-Zoster Virus (VZV), Acyclovir has very poor activity against CMV because CMV lacks the viral thymidine kinase enzyme required to activate the drug. * **Trifluridine (Option C):** This is a topical antiviral used primarily for the treatment of **Herpetic Keratitis** (dendritic ulcers). It is too toxic for systemic use and does not reach therapeutic levels in the retina to treat CMV. **3. Clinical Pearls for NEET-PG:** * **Classic Appearance:** CMV retinitis presents as **"Pizza-pie retinopathy"** or "Cottage cheese with ketchup" appearance (areas of white retinal necrosis with associated hemorrhage). * **Side Effect:** The most significant dose-limiting side effect of systemic Ganciclovir is **bone marrow suppression** (neutropenia). * **Differential Diagnosis:** Do not confuse CMV retinitis with **ARN (Acute Retinal Necrosis)**, which is caused by HSV/VZV and *is* treated with Acyclovir. * **Drug of Choice:** Oral **Valganciclovir** is now preferred for induction and maintenance therapy due to its high bioavailability.

Q: What is the most common calcified intraocular malignancy seen in children?

Retinoblastoma. **Explanation:** **Retinoblastoma** is the most common primary intraocular malignancy of childhood. It originates from the neurosensory retina due to a mutation in the **RB1 gene** on chromosome 13q14. The hallmark feature of this tumor is **dystrophic calcification**, which occurs due to rapid tumor growth outstripping its blood supply, leading to necrosis and calcium deposition. On imaging (B-scan USG or CT), calcification is seen in over 90% of cases, making it a pathognomonic sign for diagnosis. **Analysis of Incorrect Options:** * **A. Rhabdomyosarcoma:** This is the most common primary **orbital** malignancy in children, but it is extraocular (not intraocular) and typically does not show intrinsic calcification. * **C. Metastasis:** While common in adults (usually from lung or breast), intraocular metastases are rare in children. When they occur, they are usually from neuroblastoma and rarely calcify. * **D. Leukaemia:** This is the most common systemic malignancy to involve the eye in children (often presenting as iris infiltration or hypopyon), but it is not a primary intraocular tumor and does not typically calcify. **High-Yield Clinical Pearls for NEET-PG:** * **Most common presentation:** Leukocoria (White pupillary reflex), followed by strabismus. * **Histopathology:** Look for **Flexner-Wintersteiner rosettes** (highly specific) and Homer-Wright rosettes. * **Imaging:** CT scan is the gold standard to detect calcification, though MRI is preferred to evaluate optic nerve involvement. * **Classification:** The International Classification for Intraocular Retinoblastoma (ICIR) is used for staging. * **Trilateral Retinoblastoma:** Bilateral retinoblastoma associated with a pineal gland tumor (Pinealoblastoma).

Q: Development of diabetic retinopathy depends on which of the following factors?

Any of the above. **Explanation:** The development and progression of Diabetic Retinopathy (DR) are multifactorial, influenced by the metabolic control, chronicity, and timing of the disease. 1. **Duration of the disease (Most Important Factor):** This is the single most significant predictor of DR. For Type 1 Diabetics, DR is rarely present at diagnosis but affects nearly 95% of patients after 20 years. In Type 2 Diabetics, about 60% show signs of DR after 20 years. 2. **Intensity of the disease (Metabolic Control):** Poor glycemic control (reflected by high HbA1c levels) accelerates microvascular damage. Chronic hyperglycemia leads to basement membrane thickening, pericyte loss, and microaneurysm formation. 3. **Age of onset:** While DR is rare before puberty, the age at which diabetes is diagnosed influences the cumulative lifetime risk. Patients diagnosed at a younger age have a longer duration of exposure to hyperglycemia, increasing the likelihood of developing proliferative changes. **Why "Any of the above" is correct:** All three factors—how long the patient has had the disease, how well it is controlled, and when it started—interact to determine the risk and severity of retinal damage. **High-Yield Clinical Pearls for NEET-PG:** * **First clinical sign:** Microaneurysms (located in the inner nuclear layer). * **First pathological sign:** Loss of pericytes and basement membrane thickening. * **Pregnancy:** Can lead to rapid progression of pre-existing DR (requires close monitoring). * **Hypertension & Nephropathy:** These are strong systemic co-factors that worsen the prognosis of DR. * **Classification:** Remember the ETDRS classification (NPDR vs. PDR) for management questions.

Q: Foster's fusch's spots are seen in which refractive error?

Myopia. **Explanation:** **Foster-Fuchs’ Spots** (or simply Fuchs’ spots) are a classic fundoscopic finding in **Pathological (High) Myopia**. **Why Myopia is correct:** In pathological myopia (typically > -6.00D or axial length >26.5 mm), the progressive elongation of the eyeball leads to mechanical stretching of the retina and choroid. This stress causes cracks in the Bruch’s membrane, known as **Lacquer cracks**. These cracks can lead to localized Subretinal Neovascularization (SRNV). When these fragile new vessels bleed and subsequently undergo organization and scarring, they leave behind a circular, pigmented (dark/blackish) lesion at the macula. This pigmented scar is the **Foster-Fuchs’ spot**, which results in a significant loss of central vision. **Why other options are incorrect:** * **Hypermetropia:** This involves a shorter axial length. While it is associated with conditions like "crowded discs" or predispositions to angle-closure glaucoma, it does not involve the degenerative stretching or Bruch’s membrane ruptures seen in myopia. * **Astigmatism:** This is a refractive error caused by an irregular curvature of the cornea or lens. It is an optical distortion and does not involve the structural/degenerative chorioretinal changes required to form Fuchs’ spots. **High-Yield Clinical Pearls for NEET-PG:** * **Pathognomonic triad of Pathological Myopia:** Posterior Staphyloma, Lacquer Cracks, and Foster-Fuchs’ spots. * **Lacquer Cracks:** These are the precursors to Fuchs’ spots and represent healed ruptures of the Bruch’s membrane. * **Other Myopic findings:** Myopic crescent (temporal), Weiss ring (due to PVD), and Lattice degeneration (predisposing to Retinal Detachment). * **Management:** If active CNVM is present, Anti-VEGF injections are the treatment of choice.

Q: Attenuated retinal vessels with bony corpuscular-like lesions are seen in which condition?

Retinitis pigmentosa. **Explanation:** The clinical triad of **attenuated retinal vessels**, **bony spicule-like pigmentation**, and **waxy pallor of the optic disc** is the hallmark of **Retinitis Pigmentosa (RP)**. **Why Retinitis Pigmentosa is correct:** RP is a hereditary progressive rod-cone dystrophy. The "bony corpuscular" lesions are caused by the migration of retinal pigment epithelium (RPE) cells into the sensory retina, typically accumulating around the retinal veins in a characteristic branching pattern. The arteriolar attenuation occurs as a secondary response to reduced retinal metabolic demand as photoreceptors degenerate. **Why the other options are incorrect:** * **Diabetes Mellitus:** Characterized by microaneurysms, dot-and-blot hemorrhages, hard exudates, and neovascularization (PDR), but not bony spicules. * **Hypertension:** Features generalized arteriolar narrowing, AV nipping, flame-shaped hemorrhages, and cotton wool spots (Grade III/IV), but lacks pigmentary migration. * **Eales Disease:** An idiopathic peripheral perivasculitis (primarily affecting young males) characterized by peripheral non-perfusion, "sea-fan" neovascularization, and recurrent vitreous hemorrhages. **High-Yield Clinical Pearls for NEET-PG:** * **Earliest Symptom:** Nyctalopia (Night blindness) due to early rod involvement. * **Visual Field:** Ring scotoma progressing to "Tubular vision." * **ERG Findings:** The Electroretinogram (ERG) is **subnormal or extinguished** (flat) even in early stages, making it the most sensitive diagnostic test. * **Associations:** Often associated with Usher syndrome (deafness), Posterior Subcapsular Cataract (PSC), and Cystoid Macular Edema (CME).

Question 1

Which of the following ocular conditions is characterized by calcification as a pathognomonic feature?

Accepted Answer

Retinoblastoma

Answer

Malignant melanoma of the choroid

Answer

Retinocytoma

Answer

Angiomatosis retinae

Question 2

The Reese-Ellsworth classification categorizes which of the following tumors with respect to numbers, size, and location?

Accepted Answer

Retinoblastoma

Answer

Rhabdomyosarcoma

Answer

Optic nerve sheath tumor

Answer

Meningioma

Question 3

Which of the following groups of drugs can cause cystoid macular edema?

Accepted Answer

Prostaglandin E2 analogues

Answer

Prostaglandin F2a analogue

Answer

Oral corticosteroids

Answer

Alpha blockers

Question 4

Which antiviral drug is used for the treatment of cytomegalovirus retinitis?

Accepted Answer

Ganciclovir

Answer

Acyclovir

Answer

Trifluridine

Answer

All of the above

Question 5

What is the most common calcified intraocular malignancy seen in children?

Accepted Answer

Retinoblastoma

Answer

Rhabdomyosarcoma

Answer

Metastasis

Answer

Leukaemia

Question 6

In a child with phthisical eye of unknown etiology, which of the following possibilities should be considered?

Accepted Answer

Retinoblastoma

Answer

Bilateral trauma

Answer

Still's disease

Answer

Untreated congenital glaucoma

Question 7

Development of diabetic retinopathy depends on which of the following factors?

Accepted Answer

Any of the above

Answer

Intensity of the disease

Answer

Age of onset

Answer

Duration of the disease

Question 8

Foster's fusch's spots are seen in which refractive error?

Accepted Answer

Myopia

Answer

Hypermetropia

Answer

Astigmatism

Answer

None of the above

Question 9

Attenuated retinal vessels with bony corpuscular-like lesions are seen in which condition?

Accepted Answer

Retinitis pigmentosa

Answer

Diabetes mellitus

Answer

Hypertension

Answer

Eales disease

Question 10

What is the treatment of choice for Eale's disease?

Accepted Answer

Corticosteroids

Answer

Antibiotics

Answer

Antihistaminics

Answer

Surgery

Diseases of the Retina — MCQs

Diseases of the Retina — MCQs

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