Diseases of the Retina Practice Questions

Q: A child presenting with a whitish pupillary reflex (leukocoria) was treated with enucleation. Histopathology of the specimen showed Flexner-Wintersteiner rosettes. What is the most likely diagnosis?

Retinoblastoma. ***Retinoblastoma*** - The presence of **leukocoria** (whitish pupillary reflex) in a child is the most common presenting sign of **retinoblastoma** [2]. - **Flexner-Wintersteiner rosettes** are characteristic histological features of retinoblastoma, confirming the diagnosis [1]. *Astrocytoma* - Astrocytomas are **brain tumors** that typically do not cause leukocoria or originate in the retina [3]. - Their histology involves glial cells and would not show Flexner-Wintersteiner rosettes [3]. *Medulloblastoma* - Medulloblastomas are **malignant brain tumors** of the cerebellum, presenting with symptoms like ataxia and hydrocephalus [4]. - They are not associated with leukocoria or retinal involvement. *Rhabdomyosarcoma* - Rhabdomyosarcoma is a **malignant tumor of skeletal muscle**, most commonly found in the head and neck, genitourinary tract, or extremities. - While it can occur in the orbit, its histology is distinct and involves rhabdomyoblasts, not rosettes, and it's less likely to present purely as leukocoria. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Eye, pp. 1341-1342. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 737-738. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 725-726. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1314-1315.

Q: What is the most common route of the spread of retinoblastoma?

Hematogenous. ***Correct: Hematogenous*** - **Hematogenous spread** is the most common route of metastatic dissemination in retinoblastoma, occurring when tumor cells invade choroidal blood vessels. - This leads to distant metastases in **bone marrow, bones, liver, and brain** through bloodstream circulation. - Studies of metastatic retinoblastoma show hematogenous spread accounts for the majority of extraocular dissemination patterns. *Incorrect: Optic nerve invasion* - While optic nerve invasion is the **most clinically significant** prognostic factor and provides direct CNS access, it is not the most common route statistically. - Occurs in approximately 20-30% of enucleated eyes and is a critical indicator for adjuvant therapy. - When present, it dramatically worsens prognosis due to leptomeningeal spread potential. *Incorrect: Lymphatic spread* - The globe lacks true lymphatic drainage, making lymphatic spread extremely rare. - Lymphatic involvement only occurs if tumor extends beyond the eye into orbital tissues with lymphatic channels. - Not a primary route of retinoblastoma dissemination. *Incorrect: Direct invasion* - Direct orbital invasion occurs with advanced intraocular tumors breaking through the sclera. - This is a local extension rather than a route of distant metastatic spread. - While serious, it represents local progression rather than the most common dissemination pathway.

Q: A 58-year-old woman is brought to the emergency room by her husband complaining, “I can’t see out of my right eye.” She was watching television last night when she covered her left eye due to an itch and discovered that she could not see. The patient denies any precipitating event, pain, swelling, flashes, floaters, or headaches. Her past medical history is significant for uncontrolled hypertension and angina. Her medications include hydrochlorothiazide, lisinopril, atorvastatin, and nitroglycerin as needed. Her physical examination is unremarkable. Fundus examination demonstrates generalized pallor and slight disc edema with no hemorrhages. What is the most likely explanation for this patient’s symptoms?

Occlusion of the ophthalmic artery by embolus. ***Occlusion of the ophthalmic artery by embolus*** - The sudden, painless monocular vision loss ("I can't see out of my right eye") in a patient with risk factors like **uncontrolled hypertension** and **angina** (suggesting atherosclerosis) is highly indicative of an **ophthalmic artery occlusion**, likely due to an **embolus**. - **Fundus examination** findings of **generalized pallor** and **slight disc edema with no hemorrhages** further support this, as it reflects ischemia without significant venous congestion. *Inflammation of the temporal artery* - This typically presents with **temporal headache**, **jaw claudication**, and elevated inflammatory markers like **ESR**, none of which are mentioned here. - While it can cause sudden vision loss (due to **anterior ischemic optic neuropathy**), the absence of other classic symptoms makes it less likely, and funduscopic findings often include a **pale, swollen optic disc**. *Optic neuritis* - Often causes **painful monocular vision loss** and is associated with **multiple sclerosis**. - Funduscopic examination may show a normal optic disc if retrobulbar, or **optic disc hyperemia** and swelling if papillitis, but not generalized retinal pallor. *Detachment of the retina* - Usually presents with symptoms like **flashes, floaters**, and a **"curtain-like" visual field defect**, which the patient specifically denies. - Fundus examination would reveal the detached retina, not generalized pallor of the fundus.

Q: A fundus examination shows 'sunset glow' appearance. Which fluorescein angiography finding would best support Vogt-Koyanagi-Harada disease?

Multiple pinpoint leaks. ***Multiple pinpoint leaks*** - **Multiple pinpoint leaks** on fluorescein angiography during the acute inflammatory phase of Vogt-Koyanagi-Harada (VKH) disease reflect areas of **choroidal inflammation** and **serous retinal detachment**. - This finding is highly characteristic of the disease's active stage, signifying widespread inflammation of the **choroidal vasculature**. *Cherry red spot* - A **cherry red spot** is a classic finding in **central retinal artery occlusion** or **lysosomal storage disorders**, not in VKH disease. - It indicates an opaque retina with a fovea that is relatively spared, allowing visualization of the underlying choroidal circulation. *Bull's eye maculopathy* - **Bull's eye maculopathy** is typically associated with drug toxicities, such as **chloroquine** or **hydroxychloroquine**, or certain **inherited retinal dystrophies**. - It describes a ring of atrophy and pigmentary changes around the fovea, which is distinct from the diffuse inflammatory changes of VKH. *Cigarette smoke appearance* - The **cigarette smoke** or **smoke stack** appearance on fluorescein angiography refers to hyperfluorescence with leakage from the **optic disc**, typically seen in conditions like **papillitis** or **optic disc vasculitis**. - While this is a recognized angiographic finding, it is not characteristic of VKH disease, which primarily shows **multiple pinpoint leaks** from choroidal inflammation rather than disc-related leakage.

Q: A child presents with night blindness, delayed dark adaptation. Which investigation is to be done further to confirm the diagnosis?

ERG. ***ERG*** - **Electroretinography (ERG)** measures the electrical responses of various retinal cells, including **rods** and **cones**, to light stimuli. - In conditions like **retinitis pigmentosa** which cause night blindness and delayed dark adaptation, ERG will show characteristic abnormal or extinguished responses, confirming retinal dysfunction. *Retinoscopy* - **Retinoscopy** is an objective method to assess the refractive error of the eye by observing the light reflex from the retina. - It does not directly evaluate the functional integrity of photoreceptors or diagnose conditions causing **night blindness**. *Dark adaptometry* - **Dark adaptometry** measures the time it takes for the eye to adapt to dim light after exposure to bright light, quantifying the function of **rod photoreceptors**. - While it can *detect* delayed dark adaptation, it is a functional test that assesses the symptom, not the underlying cause provided by ERG. *EOG* - **Electrooculography (EOG)** measures the potential difference between the cornea and the retina, primarily assessing the function of the **retinal pigment epithelium (RPE)**. - While useful for conditions like **Best's disease**, it is less direct for evaluating generalized rod dysfunction causing night blindness compared to ERG.

Q: Optic disc changes of retinitis pigmentosa:

Consecutive optic atrophy. **Consecutive optic atrophy** - In **retinitis pigmentosa**, the progressive degeneration of photoreceptors and retinal pigment epithelium leads to secondary or **consecutive optic atrophy**. - This atrophy is characterized by a **pale, waxy optic disc** due to loss of retinal ganglion cell axons and glia. *Hyperemia of disc* - **Hyperemia of the optic disc** indicates **inflammation** or **swelling** of the optic nerve head, such as in optic neuritis or papilledema. - This is not a typical feature of retinitis pigmentosa, which involves retinal degeneration, not acute inflammation of the optic nerve. *No significant change* - As **retinitis pigmentosa** progresses, significant changes occur in the retina and optic nerve, including **pigmentary deposits**, **vascular attenuation**, and **optic disc pallor**. - Therefore, stating no significant change would be incorrect as the disease significantly alters the fundus appearance. *Blurring of disc margins* - **Blurring of the optic disc margins** is a hallmark sign of **papilledema** (swelling due to increased intracranial pressure) or an acutely inflamed optic nerve head. - This is distinct from the **optic atrophy** seen in retinitis pigmentosa, which typically involves clear but pale disc margins.

Q: Cause of sudden loss of vision in a diabetic is due to:

Vitreous hemorrhage. ***Vitreous hemorrhage*** - **Vitreous hemorrhage** is the **most common cause** of sudden, painless vision loss in individuals with **proliferative diabetic retinopathy** - New, fragile blood vessels (neovascularization) on the retina in diabetes can rupture, leading to bleeding into the **vitreous gel** - Patients describe sudden onset of floaters, cobwebs, or a red haze obscuring vision *Central retinal vein occlusion* - **CRVO** causes sudden, painless vision loss with **retinal hemorrhages in all four quadrants** (blood and thunder appearance) - While diabetic patients are at increased risk, vision loss is typically less profound than vitreous hemorrhage - Fundoscopy shows widespread retinal hemorrhages, dilated tortuous veins, and cotton-wool spots *Neovascular glaucoma* - **Neovascular glaucoma** causes **painful** vision loss and elevated intraocular pressure due to new vessel growth on the iris and trabecular meshwork - While associated with diabetes, it usually presents with more **gradual onset** and pain, rather than sudden, painless vision loss - Characterized by rubeosis iridis and elevated IOP *Central retinal artery occlusion* - **CRAO** causes sudden, profound, painless monocular vision loss, often described as a "curtain coming down" - While diabetic patients are at higher risk for CRAO due to generalized atherosclerosis, it typically results in a **cherry-red spot** on the macula - This is usually embolic in nature and less specifically related to diabetic retinopathy itself

Q: Which of the following is seen in retinitis pigmentosa?

Arteriolar attenuation. ***Arteriolar attenuation*** - **Arteriolar attenuation** is a classic finding in retinitis pigmentosa, reflecting the progressive loss of retinal tissue and the associated reduction in metabolic demand, leading to narrowing of the retinal arterioles. - This sign indicates the ongoing degeneration of photoreceptors and the underlying retinal layers, which is characteristic of the disease. *Neovascularization* - **Neovascularization** (abnormal new blood vessel growth) is typically associated with conditions like proliferative diabetic retinopathy or age-related macular degeneration. - It is not a primary feature of retinitis pigmentosa, which is a degenerative disease rather than an ischemic or proliferative one. *Papilledema* - **Papilledema** is swelling of the optic disc due to increased intracranial pressure. - It is not a feature of retinitis pigmentosa; rather, the optic disc in retinitis pigmentosa often appears waxy pale due to optic atrophy. *Retinal artery thrombosis* - **Retinal artery thrombosis** involves the sudden blockage of a retinal artery, leading to acute vision loss and often presenting with a 'cherry-red spot' on the macula. - This is an acute vascular event and is not characteristic of the chronic, progressive degeneration seen in retinitis pigmentosa.

Q: Arden index is related to

EOG (Electrooculogram). ***EOG (Electrooculogram)*** - The **Arden index** is a measure derived from the **electrooculogram (EOG)**, which assesses the function of the **retinal pigment epithelium (RPE)**. - It represents the ratio of the maximum light peak to the minimum dark trough recorded during EOG, with a normal value typically above 1.8. *ERG (Electroretinogram)* - The **electroretinogram (ERG)** measures the **electrical responses of photoreceptors and other retinal cells** to light stimuli. - While it assesses retinal function, it does not directly yield the Arden index; that is specific to EOG readings. *VER (Visual Evoked response)* - **Visual evoked response (VER)** or **VEP (visual evoked potential)** measures the **electrical activity in the brain's visual cortex** in response to visual stimuli. - It assesses the integrity of the visual pathway from the optic nerve to the cortex, not directly the function of the RPE or the Arden index. *Perimetry* - **Perimetry**, or **visual field testing**, maps the sensitivity of the entire visual field, helping to detect deficits in peripheral or central vision. - It evaluates the functional extent of vision rather than the electrical activity of specific retinal layers or the RPE as assessed by the Arden index.

Q: One year old male child with cat's reflex and raised IOP. What is the most likely diagnosis?

Retinoblastoma. ***Retinoblastoma*** - A **cat's reflex (leukocoria)**, which is a white pupillary reflex, is the most common presenting sign of retinoblastoma in children. - **Raised intraocular pressure (IOP)** can occur in advanced retinoblastoma due to secondary glaucoma caused by tumor growth or neovascularization. *Toxocara canis* - Ocular **toxocariasis** can cause leukocoria and inflammation, but it's typically associated with **granuloma formation** and not usually primary elevated IOP. - This condition is caused by a **parasitic infection** from roundworms, often seen in children with exposure to contaminated soil or pets. *Retinopathy of prematurity* - Primarily affects **premature infants** exposed to high oxygen, leading to abnormal retinal vessel development. - While it can cause leukocoria in severe stages, it would be unusual for a **one-year-old** to present with this primary diagnosis especially with raised IOP. *Toxoplasma gondii infection* - Ocular **toxoplasmosis** typically presents with **chorioretinitis** and can cause inflammation, but **leukocoria** and **raised IOP** are not its primary or most characteristic features. - This is a parasitic infection, congenital or acquired, often presenting with **retinal scars**.

Question 1

A child presenting with a whitish pupillary reflex (leukocoria) was treated with enucleation. Histopathology of the specimen showed Flexner-Wintersteiner rosettes. What is the most likely diagnosis?

Accepted Answer

Retinoblastoma

Answer

Astrocytoma

Answer

Medulloblastoma

Answer

Rhabdomyosarcoma

Question 2

What is the most common route of the spread of retinoblastoma?

Accepted Answer

Hematogenous

Answer

Optic nerve invasion

Answer

Direct invasion

Answer

Lymphatic spread

Question 3

A 58-year-old woman is brought to the emergency room by her husband complaining, “I can’t see out of my right eye.” She was watching television last night when she covered her left eye due to an itch and discovered that she could not see. The patient denies any precipitating event, pain, swelling, flashes, floaters, or headaches. Her past medical history is significant for uncontrolled hypertension and angina. Her medications include hydrochlorothiazide, lisinopril, atorvastatin, and nitroglycerin as needed. Her physical examination is unremarkable. Fundus examination demonstrates generalized pallor and slight disc edema with no hemorrhages. What is the most likely explanation for this patient’s symptoms?

Accepted Answer

Occlusion of the ophthalmic artery by embolus

Answer

Inflammation of the temporal artery

Answer

Optic neuritis

Answer

Detachment of the retina

Question 4

A fundus examination shows 'sunset glow' appearance. Which fluorescein angiography finding would best support Vogt-Koyanagi-Harada disease?

Accepted Answer

Multiple pinpoint leaks

Answer

Cherry red spot

Answer

Bull's eye maculopathy

Answer

Cigarette smoke appearance

Question 5

A child presents with night blindness, delayed dark adaptation. Which investigation is to be done further to confirm the diagnosis?

Accepted Answer

ERG

Answer

Retinoscopy

Answer

Dark adaptometry

Answer

EOG

Question 6

Optic disc changes of retinitis pigmentosa:

Accepted Answer

Consecutive optic atrophy

Answer

Hyperemia of disc

Answer

No significant change

Answer

Blurring of disc margins

Question 7

Cause of sudden loss of vision in a diabetic is due to:

Accepted Answer

Vitreous hemorrhage

Answer

Central retinal vein occlusion

Answer

Neovascular glaucoma

Answer

Central retinal artery occlusion

Question 8

Which of the following is seen in retinitis pigmentosa?

Accepted Answer

Arteriolar attenuation

Answer

Neovascularization

Answer

Papilledema

Answer

Retinal artery thrombosis

Question 9

Arden index is related to

Accepted Answer

EOG (Electrooculogram)

Answer

ERG (Electroretinogram)

Answer

VER (Visual Evoked response)

Answer

Perimetry

Question 10

One year old male child with cat's reflex and raised IOP. What is the most likely diagnosis?

Accepted Answer

Retinoblastoma

Answer

Toxocara canis

Answer

Retinopathy of prematurity

Answer

Toxoplasma gondii infection

Diseases of the Retina — MCQs

Diseases of the Retina — MCQs

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