Retinopathy of Prematurity — MCQs
Optic disc changes of retinitis pigmentosa:
What does extraretinal fibrovascular proliferation at the ridge indicate?
A child presents with night blindness, delayed dark adaptation. Which investigation is to be done further to confirm the diagnosis?
SAFE strategy is for:
A diabetic patient presents to you with visual acuity of 6/9 in one eye. Further investigations revealed preretinal hemorrhages with neovascularization at the optic disc. What is the next step in management?
All of the following are true for retinopathy of prematurity except which of the following?
One year old male child with cat's reflex and raised IOP. What is the most likely diagnosis?
What is the gold standard method for visualizing the periphery of the retina?
Retinoblastoma is bilateral in what percentage of cases?
Which of the following is NOT true about retinoblastoma?
Retinopathy of Prematurity Indian Medical PG Practice Questions and MCQs
Question 1: Optic disc changes of retinitis pigmentosa:
- A. Hyperemia of disc
- B. Consecutive optic atrophy (Correct Answer)
- C. No significant change
- D. Blurring of disc margins
Explanation: **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.
Question 2: What does extraretinal fibrovascular proliferation at the ridge indicate?
- A. Normal retina
- B. Stage II Retinopathy of Prematurity
- C. Stage III Retinopathy of Prematurity (Correct Answer)
- D. Stage I Retinopathy of Prematurity
Explanation: ***Stage III Retinopathy of Prematurity*** - Extraretinal fibrovascular proliferation at the ridge is the defining characteristic of **Stage III Retinopathy of Prematurity (ROP)**. - This stage signifies significant **neovascularization** extending into the vitreous, increasing the risk of **retinal detachment**. *Normal retina* - A normal retina does not exhibit **fibrovascular proliferation** or a distinct ridge, as its vascularization is fully developed and confined to the retinal plane. - Absence of any abnormal vascular growth or demarcation line indicates a healthy, mature retinal structure. *Stage II Retinopathy of Prematurity* - Stage II ROP is characterized by a **ridge** that is elevated and appears three-dimensional, but it **lacks extraretinal fibrovascular proliferation**. - This stage represents progression from Stage I, where the demarcation line becomes a prominent ridge, but without new vessel formation outside the retina. *Stage I Retinopathy of Prematurity* - Stage I ROP is characterized by a thin, flat **demarcation line** distinguishing vascularized from avascular retina, without any significant elevation or fibrovascular proliferation. - This initial stage indicates an arrested phase of retinal vascular development, but without the more severe signs of neovascularization.
Question 3: A child presents with night blindness, delayed dark adaptation. Which investigation is to be done further to confirm the diagnosis?
- A. ERG (Correct Answer)
- B. Retinoscopy
- C. Dark adaptometry
- D. EOG
Explanation: ***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.
Question 4: SAFE strategy is for:
- A. Onchocerciasis
- B. Glaucoma
- C. Diabetic retinopathy
- D. Trachoma (Correct Answer)
Explanation: ***Trachoma*** - The **SAFE strategy** is a comprehensive public health approach designed to eliminate **trachoma**, a preventable cause of blindness. - SAFE stands for **Surgery** for trichiasis, **Antibiotics** to treat active infection, **Facial cleanliness** to reduce transmission, and **Environmental improvement** (especially access to water and sanitation) to prevent reinfection. *Onchocerciasia* - This condition, also known as **river blindness**, is primarily managed through mass drug administration of **ivermectin**. - While public health interventions are crucial for onchocerciasis, the specific SAFE acronym is not associated with its control program. *Glaucoma* - The management of glaucoma focuses on lowering **intraocular pressure** through medications, laser treatment, or surgery. - It is a chronic eye condition that does not involve infectious agents like trachoma, and the SAFE strategy is irrelevant. *Diabetic retinopathy* - This complication of diabetes is managed by controlling **blood sugar**, blood pressure, and lipids, along with specific ophthalmological treatments like laser photocoagulation or anti-VEGF injections. - It is a non-infectious, metabolic disease, making the SAFE strategy inapplicable.
Question 5: A diabetic patient presents to you with visual acuity of 6/9 in one eye. Further investigations revealed preretinal hemorrhages with neovascularization at the optic disc. What is the next step in management?
- A. Focal laser photocoagulation
- B. Pan-retinal photocoagulation (Correct Answer)
- C. Grid laser photocoagulation
- D. Scleral buckling
Explanation: ***Pan-retinal photocoagulation*** - The presence of **preretinal hemorrhages** and **neovascularization at the optic disc (NVD)** indicates **high-risk proliferative diabetic retinopathy (PDR)**. - **NVD is a high-risk characteristic** for severe vision loss and requires urgent treatment with **pan-retinal photocoagulation (PRP)**. - PRP aims to ablate ischemic peripheral retina, which reduces the production of **VEGF** and other angiogenic factors that stimulate neovascularization. *Focal laser photocoagulation* - This treatment targets discrete leaking microaneurysms in cases of **clinically significant macular edema (CSME)**, which is not the primary issue here. - It is used for **non-proliferative diabetic retinopathy** with macular involvement, not for neovascularization. *Grid laser photocoagulation* - Grid laser is a type of focal laser used for **diffuse macular edema** where specific leaking microaneurysms cannot be identified. - It is not indicated for **neovascularization** or **preretinal hemorrhages**, which are signs of PDR. *Scleral buckling* - **Scleral buckling** is a surgical procedure primarily used to treat **retinal detachment** by indenting the sclera to relieve vitreoretinal traction. - It is not the initial or primary treatment for **proliferative diabetic retinopathy** or **neovascularization**.
Question 6: All of the following are true for retinopathy of prematurity except which of the following?
- A. Due to hypoxia there occurs neovascularization followed by fibroproliferation
- B. Occurs in premature infants due to abnormal retinal blood vessel development.
- C. End result is bilateral blindness (Correct Answer)
- D. Blindness can be prevented by early diagnosis and ablation of avascular peripheral retina with cryotherapy or photocoagulation
Explanation: ***End result is bilateral blindness*** - While retinopathy of prematurity (ROP) can lead to severe vision loss or blindness, it is not always a bilateral end result, especially with early diagnosis and treatment. The severity can vary between eyes, and some cases resolve spontaneously. - Modern screening and intervention strategies, such as laser photocoagulation or anti-VEGF injections, are often successful in preventing complete blindness in one or both eyes. *Due to hypoxia there occurs neovascularization followed by fibroproliferation* - This statement accurately describes the pathogenesis of ROP. The initial phase involves delayed normal retinal vascularization, followed by a proliferative phase characterized by **neovascularization** in response to hypoxia in the avascular retina. - These new, abnormal vessels are fragile and prone to bleeding, and their associated **fibrovascular proliferation** can lead to retinal detachment. *Blindness can be prevented by early diagnosis and ablation of vascular premature retina with cryotherapy or photocoagulation* - This is a true statement. **Early diagnosis** through ophthalmologic screening of premature infants is crucial, and treatments like **laser photocoagulation** or **cryotherapy** are effective in ablating the avascular peripheral retina to halt the progression of abnormal vessel growth. - These interventions reduce the hypoxic drive that fuels neovascularization, thereby preventing severe retinal detachment and subsequent blindness. *Occurs in premature infants due to abnormal retinal blood vessel development.* - This statement is correct. ROP is a disease primarily affecting **premature infants** because their retinal blood vessels have not completed development by the time of birth. - Postnatal factors, including oxygen fluctuations and low birth weight, further disrupt this critical development, leading to **abnormal vascularization**.
Question 7: One year old male child with cat's reflex and raised IOP. What is the most likely diagnosis?
- A. Toxocara canis
- B. Retinopathy of prematurity
- C. Retinoblastoma (Correct Answer)
- D. Toxoplasma gondii infection
Explanation: ***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 8: What is the gold standard method for visualizing the periphery of the retina?
- A. Direct ophthalmoscopy
- B. Indirect ophthalmoscopy (Correct Answer)
- C. Retinoscopy
- D. USG
Explanation: ***Correct: Indirect ophthalmoscopy*** - This method uses a **condensing lens** and a bright light source to provide a **wide-field, stereoscopic view** of the retina, making it ideal for visualizing the periphery. - It allows for examination even through some media opacities and is particularly useful for detecting peripheral retinal tears or detachments. - Provides a **field of view of 25-40 degrees** compared to only 5-10 degrees with direct ophthalmoscopy. *Incorrect: Direct ophthalmoscopy* - Provides a **highly magnified but narrow field of view**, making it difficult to systematically scan and visualize the entire peripheral retina. - It offers an **upright, monocular image** with limited depth perception, which is not optimal for assessing the three-dimensional structures of the retinal periphery. *Incorrect: Retinoscopy* - This is an objective method used to **determine the refractive error** of an eye, not for direct visualization of the retinal structures. - It involves observing the reflection of light from the retina as the examiner moves a light source across the eye. *Incorrect: USG* - **Ultrasound (USG)** is primarily used to visualize ocular structures when direct visualization is obscured by dense media opacities (e.g., severe cataracts, vitreous hemorrhage). - It provides 2D images and is not the gold standard for **routine, high-resolution visualization** of the retinal periphery when a clear view is obtainable.
Question 9: Retinoblastoma is bilateral in what percentage of cases?
- A. 100% of cases
- B. 1% of cases
- C. 50% of cases
- D. 30% of cases (Correct Answer)
Explanation: **Explanation:** Retinoblastoma (RB) is the most common primary intraocular malignancy of childhood. Its occurrence is governed by **Knudson’s "Two-Hit" Hypothesis**, which explains the distribution of unilateral and bilateral cases. **Why 30% is correct:** Approximately **25–30% of cases** are bilateral. These cases are almost always **hereditary (germline mutations)**, meaning the first "hit" (mutation in the *RB1* gene on chromosome 13q14) is present in every cell of the body. A second somatic hit in the retinal cells leads to tumor formation, often affecting both eyes and presenting at an earlier age (average 12 months). **Analysis of Incorrect Options:** * **A (100%):** Incorrect. While the germline mutation predisposes both eyes, not all RB cases are hereditary. * **B (1%):** Incorrect. This is far too low; bilateral involvement is a hallmark of the genetic form of the disease. * **C (50%):** Incorrect. While some older texts might suggest higher ranges, the standard epidemiological consensus for bilateral cases remains 25–30%. The remaining 70–75% are unilateral (mostly sporadic). **High-Yield Clinical Pearls for NEET-PG:** * **Most common presentation:** Leukocoria (white pupillary reflex), followed by strabismus. * **Genetics:** *RB1* gene on **Chromosome 13q14**. * **Pathology:** Look for **Flexner-Wintersteiner rosettes** (highly specific) and Homer-Wright rosettes. * **Trilateral Retinoblastoma:** Bilateral RB associated with a pinealoblastoma (pineal gland tumor). * **Calcification:** RB is the most common cause of intraocular calcification in an infant (visible on CT/Ultrasound). * **Treatment:** Chemoreduction is now preferred over enucleation to save the eye and vision where possible.
Question 10: Which of the following is NOT true about retinoblastoma?
- A. Bilateral in 20-30% of cases
- B. Affects the age group of 1-5 years
- C. More common in males (Correct Answer)
- D. Leukocoria is the earliest symptom
Explanation: **Explanation:** Retinoblastoma is the most common primary intraocular malignancy of childhood. Understanding its epidemiological and clinical profile is crucial for NEET-PG. **Why Option C is the correct answer (The False Statement):** Retinoblastoma shows **no gender predilection**. It affects males and females equally. There is also no significant racial predilection. Therefore, stating it is more common in males is incorrect. **Analysis of other options:** * **Option A (Bilateral in 20-30%):** This is true. Approximately 25-30% of cases are bilateral (usually germinal mutations). Unilateral cases account for about 70-75%. * **Option B (Age group 1-5 years):** This is true. Most cases present before age 5. The average age of diagnosis is 18 months for bilateral cases and 24-30 months for unilateral cases. It is rarely seen after age 6. * **Option D (Leukocoria is the earliest symptom):** This is true. **Leukocoria** (white pupillary reflex or "cat’s eye reflex") is the most common presenting sign (60%), followed by **strabismus** (20%). **High-Yield Clinical Pearls for NEET-PG:** * **Genetics:** Associated with the **RB1 gene** on chromosome **13q14**. It follows Knudson’s "Two-hit hypothesis." * **Pathology:** Look for **Flexner-Wintersteiner rosettes** (highly specific) and Homer-Wright rosettes. * **Calcification:** Intraocular calcification in a child under 3 years is pathognomonic for retinoblastoma on CT/Ultrasound. * **Trilateral Retinoblastoma:** Bilateral retinoblastoma associated with a pinealoblastoma. * **Most common spread:** Direct spread via the optic nerve to the brain.
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