Ocular Manifestations of Systemic Disorders — MCQs

A 12-year-old girl complains of headaches and blurred vision. She has a history of high blood pressure but is not currently taking medication. Her blood pressure is 160/95 mm Hg and pulse is 95 per minute. Funduscopic examination reveals small retinal microaneurysms and cotton-like zones of retinal edema and necrosis. She is hospitalized for further evaluation. Renal arteriography shows segmental stenoses forming multiple ridges that project into the lumen. What is the most likely cause of secondary hypertension in this young patient?

Ocular Manifestations of Systemic Disorders Indian Medical PG Practice Questions and MCQs

Question 111: Vision loss in HIV is commonly due to infection with?

A. Herpes virus
B. Toxocara
C. Toxoplasma
D. Cytomegalovirus (Correct Answer)

Explanation: **Explanation:** **Cytomegalovirus (CMV)** is the most common cause of vision loss and the most frequent opportunistic ocular infection in patients with HIV/AIDS, typically occurring when the **CD4+ T-cell count falls below 50 cells/mm³**. CMV retinitis is characterized by a full-thickness retinal necrosis and vasculitis. Classically, it presents as the **"Pizza-pie" or "Cottage cheese and ketchup" appearance**, consisting of granular white areas of retinal necrosis associated with prominent intraretinal hemorrhages. **Analysis of Incorrect Options:** * **Herpes Virus:** While HSV and VZV can cause Acute Retinal Necrosis (ARN) or Progressive Outer Retinal Necrosis (PORN) in HIV patients, these are less common than CMV. PORN is specifically seen in severely immunocompromised patients but lacks the heavy inflammatory component of CMV. * **Toxocara:** This is a parasitic infection (larva migrans) usually seen in children. It typically presents as a posterior pole granuloma or endophthalmitis and is not specifically associated with HIV-related immunosuppression. * **Toxoplasma:** *Toxoplasma gondii* is a common cause of posterior uveitis, but in HIV patients, it usually presents as a reactivation of a previous infection. While it causes necrotizing retinochoroiditis, CMV remains statistically more prevalent as a cause of blindness in this population. **High-Yield Clinical Pearls for NEET-PG:** * **Most common ocular finding in HIV:** HIV Microangiopathy (Retinal Cotton Wool Spots). * **Most common cause of blindness in HIV:** CMV Retinitis. * **Drug of Choice for CMV Retinitis:** Intravenous Ganciclovir (or Valganciclovir). Foscarnet and Cidofovir are alternatives. * **Immune Recovery Uveitis (IRU):** An inflammatory response seen in CMV-infected eyes after starting HAART as the CD4 count rises.

Question 112: What is the best management for an immunocompromised patient with the following fundus findings?

A. Intravitreal steroids
B. Ganciclovir
C. Sulfadiazine plus pyrimethamine (Correct Answer)
D. Amphotericin-B

Explanation: ***Sulfadiazine plus pyrimethamine*** - The fundus findings show **toxoplasma retinochoroiditis** with characteristic **"headlight in fog"** appearance - focal necrotizing lesion with vitreous haze and pigmented scar - This combination provides **synergistic anti-toxoplasma activity** and is the gold standard treatment for ocular toxoplasmosis in immunocompromised patients *Intravitreal steroids* - **Contraindicated in active infectious retinochoroiditis** as steroids can worsen the infection and increase tissue destruction - May be used only **after initiating anti-toxoplasma therapy** to reduce inflammatory response, not as primary treatment *Ganciclovir* - Specific antiviral agent for **CMV retinitis**, which presents with **"cheese and ketchup"** or **"brush fire"** appearance - CMV retinitis shows **hemorrhagic retinal necrosis** without the characteristic vitreous haze seen in toxoplasmosis *Amphotericin-B* - Antifungal agent used for **fungal endophthalmitis** caused by organisms like **Candida** or **Aspergillus** - Fungal retinitis typically shows **white fluffy infiltrates** and **chorioretinal lesions** without the pathognomonic toxoplasma appearance

Question 113: Systemic corticosteroids in herpes zoster ophthalmicus are indicated when associated with which of the following conditions?

A. Facial nerve palsy
B. Optic neuritis (Correct Answer)
C. Post-herpetic neuralgia
D. All of the above

Explanation: **Explanation:** In **Herpes Zoster Ophthalmicus (HZO)**, the use of systemic corticosteroids is a nuanced clinical decision. While oral acyclovir is the mainstay of treatment, systemic steroids are reserved for specific inflammatory complications where the benefit of reducing immune-mediated damage outweighs the risk of viral reactivation. **Why Optic Neuritis is the Correct Answer:** Optic neuritis in HZO is an inflammatory, often occlusive, complication that can lead to permanent vision loss. Systemic corticosteroids (often starting with IV methylprednisolone) are indicated to reduce the inflammatory edema and prevent irreversible damage to the optic nerve fibers. This is a high-priority indication to salvage vision. **Analysis of Incorrect Options:** * **Facial Nerve Palsy (Option A):** While steroids are the standard of care for *Bell’s Palsy*, their role in HZO-associated cranial nerve palsies (like the 3rd, 4th, or 6th nerves) is controversial. Most cases are self-limiting and managed with antiviral therapy alone. * **Post-herpetic Neuralgia (PHN) (Option C):** Clinical trials have shown that while systemic steroids may reduce the *duration* of acute pain during the eruptive phase, they do **not** prevent the incidence or severity of chronic Post-herpetic Neuralgia. PHN is primarily managed with gabapentin, pregabalin, or tricyclic antidepressants. **High-Yield Clinical Pearls for NEET-PG:** * **Hutchinson’s Sign:** Vesicles on the tip of the nose indicate involvement of the nasociliary nerve, predicting a higher risk (76%) of ocular involvement. * **Topical Steroids:** Indicated for HZO-associated keratitis (nummular or disciform) and uveitis, but only under strict antiviral cover. * **Dose:** Systemic steroids are generally avoided in the presence of active viral replication unless a sight-threatening or life-threatening inflammatory complication (like orbital apex syndrome or encephalitis) is present.

Question 114: Red keratin precipitates are seen in which of the following conditions?

A. Granulomatous uveitis
B. Hemorrhagic uveitis (Correct Answer)
C. Old healed uveitis
D. Acute anterior uveitis

Explanation: **Explanation:** **Keratic Precipitates (KPs)** are inflammatory cell deposits on the corneal endothelium, typically seen in the lower triangular area (Arlt’s triangle) due to convection currents in the aqueous humor. **1. Why Hemorrhagic Uveitis is Correct:** In **hemorrhagic uveitis**, there is an accumulation of red blood cells (RBCs) in the anterior chamber. These RBCs deposit on the corneal endothelium, giving the precipitates a characteristic **red or "rusty" appearance**. This is distinct from standard inflammatory KPs, which are usually white or gray. **2. Analysis of Incorrect Options:** * **Granulomatous Uveitis:** Characterized by **"Mutton-fat" KPs**. These are large, greasy, yellowish-white clusters composed of epithelioid cells and macrophages. * **Old Healed Uveitis:** KPs in this stage appear **pigmented (crenated)** or "dusted" with melanin. They are usually shrunken and non-active. * **Acute Anterior Uveitis:** Typically presents with **small to medium-sized, discrete, white KPs** (lymphoid cells). They are non-greasy and indicate an active non-granulomatous process. **Clinical Pearls for NEET-PG:** * **Arlt’s Triangle:** The classic distribution of KPs on the inferior cornea. * **Krukenberg’s Spindle:** A vertical line of pigment on the endothelium (seen in Pigment Dispersion Syndrome), not to be confused with inflammatory KPs. * **Stellate KPs:** Fine, star-shaped precipitates distributed over the entire endothelium; characteristic of **Fuchs’ Heterochromic Iridocyclitis** and Viral Uveitis (CMV/Herpes). * **Plastic/Fibrinous Aqueous:** Seen in severe acute anterior uveitis (e.g., HLA-B27 associated).

Question 115: The use of highly active anti-retroviral therapy (HAART) is associated with the development of:

A. Keratitis
B. Anterior Uveitis
C. Posterior Uveitis (Correct Answer)
D. Optic neuritis

Explanation: **Explanation:** The correct answer is **Posterior Uveitis**, specifically in the context of **Immune Recovery Uveitis (IRU)**. **Why Posterior Uveitis is correct:** With the advent of Highly Active Anti-Retroviral Therapy (HAART), the CD4+ T-cell count in HIV-positive patients increases, and the systemic viral load decreases. This restoration of the immune system can lead to an exaggerated inflammatory response against residual CMV (Cytomegalovirus) antigens in the eye, a phenomenon known as **Immune Recovery Inflammatory Syndrome (IRIS)**. In the eye, this manifests most commonly as **Posterior Uveitis**, characterized by vitritis, cystoid macular edema (CME), and epiretinal membrane formation. It typically occurs when CD4+ counts rise above 100 cells/µL. **Why other options are incorrect:** * **Keratitis:** While HIV patients are prone to viral keratitis (HSV/HZV), it is a result of immunosuppression rather than a complication of HAART-induced immune recovery. * **Anterior Uveitis:** Although mild anterior chamber reaction can occur in IRU, the primary and diagnostic pathology is located in the posterior segment (vitritis/maculopathy). Note: Cidofovir (an anti-CMV drug) is a known cause of drug-induced anterior uveitis, but the question specifically asks about HAART. * **Optic Neuritis:** This is not a classic manifestation of HAART or IRIS. Optic nerve involvement in HIV is usually due to opportunistic infections (e.g., Syphilis, Cryptococcus) or CMV retinitis involving the disc. **High-Yield Clinical Pearls for NEET-PG:** * **Most common ocular infection in AIDS:** CMV Retinitis (occurs when CD4 < 50). * **Immune Recovery Uveitis (IRU):** The most common cause of new-onset vision loss in HIV patients *after* starting HAART. * **Classic IRU Triad:** Previous CMV retinitis + HAART-induced CD4 rise + Vitritis/CME. * **Drug-induced Uveitis:** Rifabutin (used for MAC prophylaxis in HIV) is another high-yield cause of uveitis in this patient population.

Question 116: Which one of the following is not an ocular emergency?

A. Angle closure glaucoma
B. Central retinal artery occlusion
C. Central serous retinopathy (Correct Answer)
D. Retinal detachment

Explanation: **Explanation:** In ophthalmology, an **ocular emergency** is defined as a condition that requires immediate intervention (within minutes to hours) to prevent permanent blindness or severe structural damage. **Why Central Serous Retinopathy (CSR) is the correct answer:** CSR is a **self-limiting** condition characterized by a localized serous detachment of the neurosensory retina at the macula, typically seen in young, stressed males (Type A personalities). Most cases resolve spontaneously within 3–4 months without active treatment. While it causes blurred vision and metamorphopsia, it is considered an **elective** or subacute condition, not an emergency. **Why the other options are emergencies:** * **Angle Closure Glaucoma:** A true emergency where a sudden rise in intraocular pressure (IOP) can cause permanent optic nerve damage and blindness within hours. It requires immediate pressure-lowering agents and peripheral iridotomy. * **Central Retinal Artery Occlusion (CRAO):** Often termed an "eye stroke," the retina can only survive total ischemia for about 90–100 minutes. Immediate measures (digital massage, paracentesis) are needed to dislodge the embolus. * **Retinal Detachment (RD):** Specifically "macula-on" RD is a surgical emergency. Prompt surgery is required to prevent the detachment from involving the fovea, which would lead to permanent loss of central vision. **High-Yield Clinical Pearls for NEET-PG:** * **CRAO:** Look for "Cherry Red Spot" and "Box-carring" of vessels. * **CSR:** Look for "Smoke-stack" or "Ink-blot" appearance on Fundus Fluorescein Angiography (FFA). * **Chemical Injuries:** The only ocular emergency where treatment (irrigation) starts *before* taking a visual acuity history. * **Endophthalmitis:** Another critical emergency characterized by severe pain and hypopyon following intraocular surgery.

Question 117: What is seen in the fundus of a patient with disseminated tuberculosis?

A. Choroid tubercles (Correct Answer)
B. Optic atrophy
C. Papilledema
D. Macular edema

Explanation: **Explanation:** **Choroid Tubercles (Correct Answer):** In disseminated or miliary tuberculosis, the bacilli reach the eye via the **hematogenous route**. The choroid is the most common site of involvement because it is the most vascularized tissue in the eye. Choroid tubercles appear as multiple, small (0.5 to 3.0 mm), yellowish-white, ill-defined nodules, typically located in the posterior pole. Their presence is a pathognomonic sign of miliary TB and often aids in the systemic diagnosis. **Why other options are incorrect:** * **Optic Atrophy:** This is a late-stage sequela of various conditions (like chronic papilledema or TB meningitis) but is not a primary or diagnostic fundus finding of disseminated TB itself. * **Papilledema:** While this can occur in TB patients, it is usually secondary to **Tuberculous Meningitis (TBM)** causing increased intracranial pressure, rather than the direct hematogenous spread of the bacilli to the globe. * **Macular Edema:** This is a non-specific finding seen in various inflammatory conditions (uveitis) or vascular disorders, but it is not the characteristic hallmark of disseminated TB. **High-Yield Clinical Pearls for NEET-PG:** * **Most common ocular lesion in TB:** Anterior uveitis (granulomatous). * **Pathognomonic sign of Miliary TB:** Choroid tubercles. * **Eales’ Disease:** An idiopathic peripheral perivasculitis (often linked to TB hypersensitivity) characterized by peripheral retinal neovascularization and vitreous hemorrhage. * **Treatment:** Standard Anti-Tubercular Therapy (ATT). Note that Ethambutol can cause **optic neuritis** (retrobulbar), a common side effect tested in exams.

Question 118: Which condition is characterized by the "Candle wax dripping sign"?

A. Sarcoidosis (Correct Answer)
B. Systemic Lupus Erythematosus (SLE)
C. Human Immunodeficiency Virus (HIV)
D. Rheumatoid arthritis

Explanation: **Explanation:** **Sarcoidosis (Correct Answer):** The "Candle wax dripping" sign (also known as *taches de bougie*) is a pathognomonic feature of **Ocular Sarcoidosis**. It refers to perivenous exudates caused by segmental retinal perivasculitis (vasculitis affecting the retinal veins). These yellowish-white, waxy exudates cluster along the retinal vessels, resembling melted wax dripping down a candle. Sarcoidosis typically presents as a granulomatous uveitis (mutton-fat keratic precipitates, Koeppe/Busacca nodules) and can affect any part of the eye. **Why the other options are incorrect:** * **SLE:** Characterized by retinal cotton wool spots and vasculopathy, but the classic sign is the presence of **cytoid bodies** in the nerve fiber layer, not candle wax exudates. * **HIV:** The most common ocular finding is **HIV Microangiopathy** (cotton wool spots). While CMV retinitis is a common opportunistic infection in HIV, it presents with a "Pizza-pie" or "Cottage cheese and ketchup" appearance. * **Rheumatoid Arthritis:** Primarily affects the outer coats of the eye, leading to **Keratoconjunctivitis sicca** (most common), episcleritis, or scleritis (including Scleromalacia perforans). It does not typically cause retinal perivasculitis. **High-Yield Clinical Pearls for NEET-PG:** * **Mutton-fat Keratic Precipitates:** Large, greasy inflammatory cells on the corneal endothelium (Granulomatous uveitis). * **String of Pearls:** Vitreous opacities characteristic of Sarcoidosis or Fungal endophthalmitis. * **Lofgren’s Syndrome:** Triad of Erythema nodosum, Bilateral hilar lymphadenopathy, and Arthritis (associated with Sarcoidosis). * **Heerfordt’s Syndrome (Uveoparotid fever):** Uveitis, Parotid enlargement, Facial nerve palsy, and Fever.

Question 119: All the following are causes of bluish sclerae in young children, EXCEPT:

A. Hallermann-Streiff syndrome
B. Ehlers-Danlos syndrome
C. Robe's syndrome
D. Sjögren-Larsson syndrome (Correct Answer)

Explanation: **Explanation:** The "blue sclera" appearance occurs due to **thinning of the scleral collagen** or increased transparency, which allows the underlying dark-pigmented uveal tissue (choroid) to shine through. **Why Sjögren-Larsson Syndrome is the correct answer:** Sjögren-Larsson syndrome is an autosomal recessive neurocutaneous disorder characterized by the triad of **ichthyosis, spastic diplegia/quadriplegia, and intellectual disability**. Its primary ocular manifestation is **glistening white dots in the retina** (crystalline maculopathy). It is not associated with scleral thinning or blue sclera. **Analysis of Incorrect Options:** * **Hallermann-Streiff Syndrome:** A rare oculodentodigital disorder characterized by "bird-like" facies, dental anomalies, and microphthalmos. Blue sclera is a documented feature along with congenital cataracts. * **Ehlers-Danlos Syndrome (Type VI):** This is a classic cause of blue sclera. Due to a defect in collagen synthesis (lysyl hydroxylase deficiency), the sclera is thin and prone to rupture (keratoglobus/fragile ocular coats). * **Robe's Syndrome:** Also known as **Osteogenesis Imperfecta (OI)**. This is the most common cause of blue sclera in clinical practice. It results from a mutation in Type 1 collagen. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for Blue Sclera:** "HE-MAN" (Hallermann-Streiff, Ehlers-Danlos, Marfan/Myopia, Alkaptonuria, Osteogenesis imperfecta). * **Differential Diagnosis:** Other causes include Buphthalmos (stretching of sclera in congenital glaucoma), Iron deficiency anemia, and Diamond-Blackfan anemia. * **Osteogenesis Imperfecta Type I** is the subtype most strongly associated with deep blue sclera. * **Sjögren-Larsson Syndrome** is specifically associated with **Fatty Alcohol Oxidoreductase (FAO)** deficiency.

Question 120: A 12-year-old girl complains of headaches and blurred vision. She has a history of high blood pressure but is not currently taking medication. Her blood pressure is 160/95 mm Hg and pulse is 95 per minute. Funduscopic examination reveals small retinal microaneurysms and cotton-like zones of retinal edema and necrosis. She is hospitalized for further evaluation. Renal arteriography shows segmental stenoses forming multiple ridges that project into the lumen. What is the most likely cause of secondary hypertension in this young patient?

A. Buerger disease
B. Fibromuscular dysplasia (Correct Answer)
C. Giant cell arteritis
D. Kawasaki disease

Explanation: **Explanation:** The patient presents with **secondary hypertension** and hypertensive retinopathy (cotton-wool spots and microaneurysms) at a young age. The definitive clue lies in the renal arteriography, which shows "segmental stenoses forming multiple ridges," classically described as the **"string of beads" appearance**. **1. Why Fibromuscular Dysplasia (FMD) is correct:** FMD is a non-inflammatory, non-atherosclerotic vascular disease that leads to stenosis of medium-sized arteries. It most commonly affects the **renal arteries** (causing renovascular hypertension via the Renin-Angiotensin-Aldosterone System) and the internal carotid arteries. It is most prevalent in **young females**. The "string of beads" occurs due to areas of medial fibroplasia alternating with aneurysmal dilation. **2. Why other options are incorrect:** * **Buerger Disease (Thromboangiitis obliterans):** An inflammatory vasculitis strongly associated with **heavy smoking**. It typically affects small-to-medium vessels of the extremities, leading to gangrene, not renal-induced hypertension. * **Giant Cell Arteritis:** A large-vessel vasculitis that occurs almost exclusively in patients **over age 50**. It presents with jaw claudication, scalp tenderness, and a high ESR. * **Kawasaki Disease:** An acute febrile vasculitis of childhood. While it affects medium-sized vessels, its hallmark is **coronary artery aneurysms**, not renal artery stenosis leading to chronic hypertension. **NEET-PG High-Yield Pearls:** * **FMD Pathophysiology:** Most common type is **Medial Fibroplasia** (85%). * **Ocular Link:** Severe hypertension from FMD can lead to **Grade IV Hypertensive Retinopathy** (Elschnig spots, Siegrist streaks, and macular stars). * **Diagnosis:** Digital Subtraction Angiography (DSA) is the gold standard, showing the "string of beads." * **Treatment:** Percutaneous transluminal angioplasty (PTA) is often the preferred intervention.

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Diabetes Mellitus

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Hypertension

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Autoimmune Disorders

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Thyroid Disease

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HIV and AIDS

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Hematological Disorders

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Neurological Disorders

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Dermatological Conditions

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Pregnancy-Related Eye Changes

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Metabolic Disorders

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Ocular Toxicity of Systemic Medications

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Infectious Systemic Diseases

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