Diseases of the Retina — MCQs

Diseases of the Retina Indian Medical PG Practice Questions and MCQs

Question 201: All of the following are true about angioid streaks except:

A. They can be mistaken for blood vessels
B. They are situated near the optic disc
C. They are situated at a superficial level compared to the retinal vessels (Correct Answer)
D. They result from defects in Bruch's membrane

Explanation: **Explanation:** Angioid streaks are jagged, radiating lines that represent **linear dehiscences (cracks) in a thickened, calcified Bruch’s membrane**. **Why Option C is the correct answer (The False Statement):** Angioid streaks are located deep in the retina. Specifically, they occur at the level of the **Bruch’s membrane**, which lies between the retinal pigment epithelium (RPE) and the choriocapillaris. Therefore, they are situated **deep** to the retinal blood vessels. On ophthalmoscopy, the retinal vessels can be seen crossing over these streaks, confirming their deeper anatomical position. **Analysis of Incorrect Options:** * **Option A:** They often resemble blood vessels due to their reddish-brown or gray color and radiating pattern, which can lead to diagnostic confusion for a beginner. * **Option B:** They typically radiate outward from the **peripapillary area** (around the optic disc) in a circumferential or linear fashion, mimicking the appearance of a cracked windshield. * **Option D:** The primary pathology is indeed a defect or crack in the Bruch’s membrane, often associated with systemic conditions that cause elastic tissue degeneration. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for Associations (PEPSI):** **P**seudoxanthoma elasticum (most common), **E**hlers-Danlos syndrome, **P**aget’s disease of bone, **S**ickle cell anemia (and other hemoglobinopathies), **I**diopathic. * **Complication:** The most vision-threatening complication is **Choroidal Neovascularization (CNV)**, which can lead to subretinal hemorrhage and scarring. * **Appearance:** They are often described as "crack-like" or "spider-web" lesions.

Question 202: All of the following statements regarding diabetic retinopathy are false, except:

A. Very severe non-proliferative diabetic retinopathy has a 45% chance of progression to high-risk proliferative diabetic retinopathy within 1 year. (Correct Answer)
B. Argon laser photocoagulation is contraindicated in cases of ischemic maculopathy if the foveal avascular zone is less than 1000 microns.
C. Panretinal photocoagulation for proliferative diabetic retinopathy should be done in a single sitting as it can lead to visual field defects.
D. Aspirin at a dose of 650mg/day slowed the progression of diabetic retinopathy.

Explanation: ### Explanation **1. Why Option A is Correct:** According to the **ETDRS (Early Treatment Diabetic Retinopathy Study)** classification, the risk of progression to Proliferative Diabetic Retinopathy (PDR) is determined by the "4-2-1 rule." **Very Severe NPDR** is defined when two or more criteria of the 4-2-1 rule are met. Such patients have a **45% to 50% risk** of progressing to high-risk PDR within one year, making close follow-up or early panretinal photocoagulation (PRP) crucial. **2. Why the Other Options are Incorrect:** * **Option B:** In **Ischemic Maculopathy**, laser photocoagulation is generally **ineffective or contraindicated** because the vision loss is due to capillary non-perfusion, not edema. However, the threshold for the Foveal Avascular Zone (FAZ) enlargement indicating ischemia is typically >500–600 microns, not 1000. More importantly, laser cannot restore blood flow to an ischemic zone. * **Option C:** PRP is typically performed in **multiple sittings** (usually 2–3). Doing it in a single sitting is avoided because it can cause sudden choroidal effusions, exudative retinal detachment, or acute angle-closure glaucoma. * **Option D:** The ETDRS study conclusively showed that **Aspirin (650mg/day) does not slow the progression** of retinopathy, nor does it increase the risk of retinal hemorrhage. It is neutral for retinopathy but beneficial for cardiovascular health. **High-Yield Clinical Pearls for NEET-PG:** * **4-2-1 Rule for Severe NPDR:** 4 quadrants of microaneurysms/hemorrhages, 2 quadrants of venous beading, or 1 quadrant of IRMA (Intraretinal Microvascular Abnormalities). * **First Clinical Sign:** Microaneurysms (in the inner nuclear layer). * **Earliest Pathological Change:** Loss of pericytes and basement membrane thickening. * **Treatment of Choice for CSME:** Intravitreal Anti-VEGF (e.g., Ranibizumab, Aflibercept) is now the first-line treatment, surpassing focal/grid laser.

Question 203: Which of the following conditions may masquerade as uveitis?

A. Choroidal detachment
B. Retinal detachment (Correct Answer)
C. Age-related macular degeneration
D. Central retinal artery occlusion

Explanation: **Explanation:** **Retinal Detachment (RD)**, specifically long-standing or chronic rhegmatogenous retinal detachment, is a well-known **masquerade syndrome**. In these cases, degenerating photoreceptor outer segments migrate into the vitreous and anterior chamber. These cells, along with associated pigment (Schwartz-Matsuo syndrome), can be mistaken for inflammatory "cells" and "flare," leading to a misdiagnosis of chronic uveitis. Furthermore, chronic RD can cause hypotony and mild ciliary congestion, further mimicking the clinical picture of iridocyclitis. **Analysis of Incorrect Options:** * **Choroidal detachment:** While it presents with low intraocular pressure (hypotony), it typically follows trauma or surgery and presents with characteristic "kissing choroidals" on imaging, rather than mimicking intraocular inflammation. * **Age-related macular degeneration (AMD):** This is a degenerative condition of the macula. While exudative AMD involves subretinal fluid/hemorrhage, it does not typically present with anterior or vitreous chamber cells that mimic uveitis. * **Central retinal artery occlusion (CRAO):** This is a vascular emergency presenting with sudden, painless loss of vision and a "cherry-red spot." It does not involve inflammatory-like cellular debris. **High-Yield Clinical Pearls for NEET-PG:** 1. **Schwartz-Matsuo Syndrome:** A specific masquerade where chronic RD leads to secondary glaucoma because photoreceptor outer segments block the trabecular meshwork. 2. **Other Masquerades:** Retinoblastoma (most common in children), Intraocular Lymphoma (most common in elderly), and Malignant Melanoma. 3. **Key Differentiator:** If a patient with suspected "uveitis" has low IOP (hypotony) and does not respond to steroids, always perform a dilated fundus exam to rule out Retinal Detachment.

Question 204: Angioid streaks are seen in which of the following conditions?

A. Tuberous sclerosis
B. Pseudoxanthoma elasticum (Correct Answer)
C. Sturge-Weber syndrome
D. Septo-optic dysplasia

Explanation: **Explanation:** **Angioid streaks** are irregular, jagged, radiating lines deep to the retina that resemble blood vessels. They represent **linear dehiscences (cracks) in a thickened, calcified, and brittle Bruch’s membrane**. 1. **Why Pseudoxanthoma Elasticum (PXE) is correct:** PXE (Grönblad-Strandberg syndrome) is the most common systemic association of angioid streaks, occurring in approximately 80% of patients. In this condition, progressive calcification of elastic fibers makes Bruch’s membrane fragile, leading to the characteristic cracks. 2. **Why the other options are incorrect:** * **Tuberous Sclerosis:** Characterized by **astrocytic hamartomas** (mulberry lesions) of the retina or optic nerve head, not Bruch’s membrane pathology. * **Sturge-Weber Syndrome:** Classically associated with **diffuse choroidal hemangiomas** ("tomato catsup" fundus) and glaucoma, but not angioid streaks. * **Septo-optic Dysplasia (de Morsier Syndrome):** A congenital anomaly involving **optic nerve hypoplasia**, midline brain defects, and pituitary deficiencies. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for Angioid Streaks (PEPSI):** * **P** – **P**seudoxanthoma elasticum (Most common) * **E** – **E**hlers-Danlos syndrome * **P** – **P**aget’s disease of bone * **S** – **S**ickle cell anemia (and other hemoglobinopathies) * **I** – **I**diopathic * **Clinical Significance:** The primary vision-threatening complication of angioid streaks is the development of **Choroidal Neovascularization (CNVM)**. * **PXE Skin Finding:** Look for "plucked chicken skin" appearance (yellowish papules on the neck/axilla).

Question 205: Amaurosis fugax is:

A. Transient ischemic attack of the retina (Correct Answer)
B. Demyelinating disease of the optic nerve
C. Retinal detachment in diabetic retinopathy
D. Coat's disease

Explanation: **Explanation:** **Amaurosis Fugax** (Latin for "fleeting darkness") refers to a sudden, painless, and temporary loss of vision in one eye. It is essentially a **Transient Ischemic Attack (TIA) of the retina**, typically caused by an embolus (most commonly Hollenhorst plaques from the carotid artery) that temporarily occludes the retinal circulation. * **Why Option A is correct:** The underlying mechanism is transient hypoperfusion. When an embolus lodges in the retinal artery, it causes ischemia; once the embolus dissolves or moves distally, perfusion is restored, and vision returns—usually within seconds to minutes. It is a critical warning sign of an impending stroke or Central Retinal Artery Occlusion (CRAO). **Why the other options are incorrect:** * **Option B:** Demyelinating disease (e.g., Multiple Sclerosis) causes **Optic Neuritis**, which presents with subacute, painful vision loss and a Marcus Gunn pupil, not transient episodes. * **Option C:** Retinal detachment in diabetes is usually **Tractional RD**, which causes progressive, permanent vision loss or "floaters," not transient episodes. * **Option D:** **Coat’s disease** is an idiopathic condition characterized by telangiectatic retinal vessels and exudation, typically seen in young males; it does not present as transient vision loss. **High-Yield Clinical Pearls for NEET-PG:** 1. **Classic Presentation:** Patients describe a "curtain coming down" over one eye that lifts after a few minutes. 2. **Most Common Source:** Carotid artery stenosis (Atherosclerosis). 3. **Hollenhorst Plaques:** These are bright, refractive cholesterol emboli seen at retinal vessel bifurcations during fundoscopy. 4. **Management:** Immediate carotid Doppler ultrasound and cardiovascular workup are mandatory to prevent a major stroke.

Question 206: Which of the following stages of Retinopathy of Prematurity (ROP) can be managed with observation alone?

A. Aggressive posterior ROP
B. Stage 4 ROP
C. Stage 1, Zone 3 ROP (Correct Answer)
D. Stage 2, Zone 1 with Plus disease

Explanation: ### Explanation **Retinopathy of Prematurity (ROP)** is a vasoproliferative disorder affecting preterm infants. Management is determined by the **International Classification of ROP (ICROP)**, which categorizes the disease based on Zone (location), Stage (severity), and the presence of "Plus disease" (shunting/venous dilation). **Why Option C is Correct:** **Stage 1, Zone 3 ROP** represents the mildest form of the disease in the most peripheral part of the retina. * **Zone 3** is the temporal crescent of the retina, furthest from the optic nerve. * **Stage 1** indicates a simple demarcation line between vascular and avascular retina. Disease in Zone 3 rarely progresses to vision-threatening stages and almost always undergoes spontaneous regression. Therefore, it requires **observation (serial monitoring)** rather than active intervention. **Why Other Options are Incorrect:** * **A. Aggressive posterior ROP (AP-ROP):** A rapidly progressing, severe form characterized by prominent plus disease in the posterior pole. It requires urgent treatment (Laser or Anti-VEGF) to prevent total retinal detachment. * **B. Stage 4 ROP:** This indicates **partial retinal detachment** (4a: extrafoveal; 4b: foveal). It requires surgical intervention (Scleral buckling or Vitrectomy). * **D. Stage 2, Zone 1 with Plus disease:** This meets the criteria for **Type 1 ROP** (High-risk ROP). According to the ETROP study, any stage in Zone 1 with Plus disease requires immediate treatment. **High-Yield Clinical Pearls for NEET-PG:** * **Screening Criteria (India):** Birth weight **<1750g** or Gestational Age **<34 weeks**. * **First Screening:** Should be done at **4 weeks** of birth or **31 weeks** of post-conceptional age (whichever is later). * **Plus Disease:** Defined by tortuosity of arterioles and dilation of venules in at least 2 quadrants of the posterior pole. * **Treatment of Choice:** Diode Laser Photocoagulation (standard) or Intravitreal Anti-VEGF (Ranibizumab/Bevacizumab).

Question 207: A cherry red spot is seen in which of the following conditions?

A. Niemann-Pick disease
B. Tay-Sachs disease
C. Central retinal artery occlusion
D. All of the above (Correct Answer)

Explanation: ### Explanation The "Cherry Red Spot" is a classic ophthalmoscopic finding characterized by a bright red circular area at the center of the macula, surrounded by a pale, opaque, or milky-white retina. **The Underlying Mechanism:** The foveola is the thinnest part of the retina, lacking the ganglion cell layer. In certain pathological states, the surrounding retina becomes opaque due to either **intracellular storage of lipids** (in metabolic disorders) or **axonal swelling and edema** (in vascular occlusion). Because the foveola remains thin and transparent, the underlying vascular choroid shines through, creating a stark red contrast against the pale background. **Analysis of Options:** * **Central Retinal Artery Occlusion (CRAO):** This is the most common vascular cause. Ischemia leads to cloudy swelling (edema) of the retinal nerve fiber and ganglion cell layers. The foveola, supplied by the choroid, remains red. * **Tay-Sachs Disease (GM2 Gangliosidosis):** A lysosomal storage disorder where gangliosides accumulate in the retinal ganglion cells, making the macula appear white, except for the foveola. * **Niemann-Pick Disease (Type A):** Sphingomyelin accumulates in the ganglion cells, resulting in the same clinical appearance. **Clinical Pearls for NEET-PG:** * **Mnemonic for Cherry Red Spot:** *"**S**ome **P**eople **C**an **M**ake **G**ood **T**asty **C**herry **H**alwa"* * **S**ialidosis * **P**aralytic Ileus (Farber’s disease) * **C**RAO (Most common vascular cause) * **M**etachromatic Leukodystrophy * **G**aucher’s Disease (Type 2) * **T**ay-Sachs Disease (Most common metabolic cause) * **C**herubism * **H**urler’s Syndrome * **Differential Diagnosis:** In **Berlin’s Edema (Commotio Retinae)**, a "pseudo-cherry red spot" may appear due to retinal concussive injury. * **Key Distinction:** In CRAO, the spot appears acutely; in storage diseases, it develops progressively.

Question 208: In Von Hippel-Lindau Syndrome, retinal vascular tumours are often associated with intracranial hemangioblastomas. Which one of the following regions is associated with such vascular abnormalities in this syndrome?

A. Optic radiation
B. Optic tract
C. Cerebellum (Correct Answer)
D. Pulvinar

Explanation: **Explanation:** **Von Hippel-Lindau (VHL) Syndrome** is an autosomal dominant multisystem disorder caused by a mutation in the VHL gene on **Chromosome 3p**. It is characterized by the development of numerous benign and malignant tumors, most notably **hemangioblastomas**. 1. **Why the Cerebellum is Correct:** The hallmark of VHL syndrome is the association of **Retinal Angiomatosis** (Von Hippel disease) with **Capillary Hemangioblastomas of the Central Nervous System**. The **cerebellum** is the most common site for these intracranial tumors (occurring in up to 60-70% of patients), followed by the spinal cord and brainstem. These are highly vascular, slow-growing tumors that often present with symptoms of increased intracranial pressure or cerebellar ataxia. 2. **Why Other Options are Incorrect:** * **Optic Radiation, Optic Tract, and Pulvinar:** While VHL can involve various parts of the CNS, hemangioblastomas do not typically involve the visual pathway structures (tracts or radiations) or the thalamic nuclei (pulvinar). These regions are more commonly associated with infarcts or specific neurodegenerative processes rather than the vascular hamartomas seen in VHL. **High-Yield Clinical Pearls for NEET-PG:** * **VHL Gene:** Located on **Chromosome 3p25**. * **Retinal Lesion:** Presents as a "sea-fan" appearance or a reddish globular tumor with a dilated, tortuous feeder artery and a draining vein. * **Systemic Associations:** * **Renal Cell Carcinoma (RCC):** Clear cell type (major cause of mortality). * **Pheochromocytoma:** Often bilateral. * **Pancreatic cysts** and Endolymphatic sac tumors. * **Screening:** Annual ophthalmoscopy and periodic MRI of the brain/abdomen are essential for early detection.

Question 209: What is the primary objective of the MARINA trial?

A. To evaluate the role of new antibiotics in treating fungal ulcers.
B. To assess the efficacy of Anti-VEGF agents in managing diabetic retinopathy.
C. To determine the role of Aflibercept in wet age-related macular degeneration (ARMD).
D. To investigate the role of ranibizumab in the treatment of age-related macular degeneration (ARMD). (Correct Answer)

Explanation: The **MARINA trial** (Minimally Classic/Occult Trial of the Anti-VEGF Antibody Ranibizumab in the Treatment of Neovascular Age-Related Macular Degeneration) is a landmark study in ophthalmology that established **Ranibizumab (Lucentis)** as a gold standard for treating "wet" ARMD. ### **Explanation of the Correct Option** * **Option D:** The trial evaluated the efficacy of monthly intravitreal injections of Ranibizumab in patients with **minimally classic or occult choroidal neovascularization (CNV)** due to ARMD. It was revolutionary because it showed, for the first time, that Anti-VEGF therapy could not only prevent vision loss but actually **improve mean visual acuity** in nearly 95% of patients, compared to the natural decline seen in the sham group. ### **Why Other Options are Incorrect** * **Option A:** Fungal ulcers are managed with antifungals (e.g., Natamycin); the MARINA trial is strictly related to retinal vascular disease. * **Option B:** While Anti-VEGFs are used in diabetic retinopathy, the **DRCR.net** protocols (e.g., Protocol T) are the primary trials for that condition. * **Option C:** Aflibercept was primarily evaluated in the **VIEW 1 and VIEW 2** trials, which compared its efficacy to Ranibizumab. ### **High-Yield Clinical Pearls for NEET-PG** * **MARINA vs. ANCHOR:** While MARINA focused on *minimally classic/occult* CNV, the **ANCHOR trial** compared Ranibizumab to Photodynamic Therapy (PDT) in *predominantly classic* CNV. * **CATT Trial:** This trial compared **Ranibizumab vs. Bevacizumab** (Avastin) and found them to be equally effective for wet ARMD. * **Mechanism:** Ranibizumab is a recombinant humanized monoclonal antibody fragment (Fab) that binds to all isoforms of **VEGF-A**. * **Key Finding:** MARINA proved that Anti-VEGF therapy is superior to sham/placebo in maintaining and improving vision.

Question 210: Retinal detachment is not seen in which of the following conditions?

A. High myopia
B. Aphakia
C. Choroiditis
D. Hypermetropia (Correct Answer)

Explanation: **Explanation:** The correct answer is **Hypermetropia**. Retinal detachment (RD) is primarily associated with conditions that lead to structural weakening of the retina or changes in the vitreous volume and axial length of the eye. **1. Why Hypermetropia is the correct answer:** In Hypermetropia (farsightedness), the eyeball is typically **shorter** than normal (short axial length). This results in a relatively "crowded" posterior segment where the retina is firmly supported. Unlike myopia, there is no stretching of the retinal layers or thinning of the peripheral retina, making the risk of spontaneous or rhegmatogenous retinal detachment extremely low. **2. Why the other options are incorrect:** * **High Myopia:** This is a major risk factor for RD. The eyeball is elongated (increased axial length), leading to thinning of the retina and peripheral degenerations (like **Lattice degeneration**). This predisposes the eye to retinal breaks and subsequent detachment. * **Aphakia:** The absence of a lens (usually post-cataract surgery) leads to increased mobility of the vitreous humor (**vitreous syneresis**). This mechanical instability can exert traction on the retina, significantly increasing the risk of RD. * **Choroiditis:** Inflammatory conditions of the choroid can lead to **Exudative Retinal Detachment**. Fluid leaks from the inflamed choroidal vessels and accumulates in the subretinal space, pushing the retina away from the pigment epithelium. **Clinical Pearls for NEET-PG:** * **Most common type of RD:** Rhegmatogenous (due to a retinal hole/tear). * **Most common precursor:** Lattice degeneration (seen in 8-10% of the general population but highly associated with myopia). * **Tobacco Dust (Shafer’s Sign):** Presence of pigment cells in the anterior vitreous; a pathognomonic sign of a retinal tear. * **Treatment of choice:** Scleral buckling or Pars Plana Vitrectomy (PPV). For exudative RD, the primary treatment is managing the underlying medical cause (e.g., steroids for choroiditis).

Practice by Chapter

Retinal Anatomy and Physiology

Practice Questions

Age-Related Macular Degeneration

Practice Questions

Diabetic Retinopathy

Practice Questions

Retinal Vascular Diseases

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Retinal Detachment

Practice Questions

Hereditary Retinal Dystrophies

Practice Questions

Inflammatory Retinal Diseases

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Retinal Tumors

Practice Questions

Retinopathy of Prematurity

Practice Questions

Retinal Imaging Techniques

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Intravitreal Pharmacotherapy

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Vitreoretinal Surgery

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