Diseases of the Retina Practice Questions

Q: Which of the following conditions may masquerade as uveitis?

Retinal detachment. **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.

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

Pseudoxanthoma elasticum. **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).

Q: Amaurosis fugax is:

Transient ischemic attack of the retina. **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.

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

All of the above. ### 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.

Q: 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?

Cerebellum. **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 1

All of the following are true about angioid streaks except:

Accepted Answer

They are situated at a superficial level compared to the retinal vessels

Answer

They can be mistaken for blood vessels

Answer

They are situated near the optic disc

Answer

They result from defects in Bruch's membrane

Question 2

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

Accepted Answer

Very severe non-proliferative diabetic retinopathy has a 45% chance of progression to high-risk proliferative diabetic retinopathy within 1 year.

Answer

Argon laser photocoagulation is contraindicated in cases of ischemic maculopathy if the foveal avascular zone is less than 1000 microns.

Answer

Panretinal photocoagulation for proliferative diabetic retinopathy should be done in a single sitting as it can lead to visual field defects.

Answer

Aspirin at a dose of 650mg/day slowed the progression of diabetic retinopathy.

Question 3

Which of the following conditions may masquerade as uveitis?

Accepted Answer

Retinal detachment

Answer

Choroidal detachment

Answer

Age-related macular degeneration

Answer

Central retinal artery occlusion

Question 4

Angioid streaks are seen in which of the following conditions?

Accepted Answer

Pseudoxanthoma elasticum

Answer

Tuberous sclerosis

Answer

Sturge-Weber syndrome

Answer

Septo-optic dysplasia

Question 5

Amaurosis fugax is:

Accepted Answer

Transient ischemic attack of the retina

Answer

Demyelinating disease of the optic nerve

Answer

Retinal detachment in diabetic retinopathy

Answer

Coat's disease

Question 6

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

Accepted Answer

Stage 1, Zone 3 ROP

Answer

Aggressive posterior ROP

Answer

Stage 4 ROP

Answer

Stage 2, Zone 1 with Plus disease

Question 7

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

Accepted Answer

All of the above

Answer

Niemann-Pick disease

Answer

Tay-Sachs disease

Answer

Central retinal artery occlusion

Question 8

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?

Accepted Answer

Cerebellum

Answer

Optic radiation

Answer

Optic tract

Answer

Pulvinar

Question 9

What is the primary objective of the MARINA trial?

Accepted Answer

To investigate the role of ranibizumab in the treatment of age-related macular degeneration (ARMD).

Answer

To evaluate the role of new antibiotics in treating fungal ulcers.

Answer

To assess the efficacy of Anti-VEGF agents in managing diabetic retinopathy.

Answer

To determine the role of Aflibercept in wet age-related macular degeneration (ARMD).

Question 10

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

Accepted Answer

Hypermetropia

Answer

High myopia

Answer

Aphakia

Answer

Choroiditis

Diseases of the Retina — MCQs

Diseases of the Retina — MCQs

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