Ocular Manifestations of Systemic Disorders Practice Questions

Q: Marfan's syndrome has which of the following prominent eye defects?

Ectopia lentis. **Explanation:** **Ectopia lentis** (displacement of the lens) is the most common and characteristic ocular manifestation of Marfan’s syndrome, occurring in approximately 50–80% of patients. The underlying pathology is a mutation in the **FBN1 gene**, which leads to a deficiency in **fibrillin-1**. Since the ciliary zonules (which hold the lens in place) are composed primarily of fibrillin, they become weak and stretched, leading to lens subluxation. In Marfan’s, the displacement is typically **superotemporal** (upward and outward), and the accommodation is usually preserved because the zonules remain intact though stretched. **Analysis of Incorrect Options:** * **Megalocornea (A):** While Marfan’s patients may have a slightly increased corneal diameter or a flat cornea (cornea plana), true megalocornea is more classically associated with X-linked Megalocornea or Congenital Glaucoma. * **Microcornea (B):** This is associated with conditions like Nanophthalmos or Microphthalmos, not Marfan’s. * **Microspherophakia (C):** This refers to a small, spherical lens. While it causes ectopia lentis, it is the hallmark of **Weill-Marchesani syndrome** (the "inverse" of Marfan’s), not Marfan’s syndrome itself. **High-Yield Clinical Pearls for NEET-PG:** * **Direction of Subluxation:** Marfan’s = **Upward** (Superior); Homocystinuria = **Downward** (Inferior). * **Zonular Integrity:** In Marfan’s, zonules are stretched/lax; in Homocystinuria, zonules are completely broken/absent. * **Other Ocular Features:** High myopia, increased axial length, and increased risk of Rhegmatogenous Retinal Detachment. * **Systemic Association:** Always screen for aortic root dilation/dissection and mitral valve prolapse (MVP).

Q: All are seen in acute iridocyclitis except:

Mucopurulent discharge. **Explanation:** Acute iridocyclitis (anterior uveitis) is characterized by sterile inflammation of the iris and ciliary body. The correct answer is **Mucopurulent discharge**, as this is a hallmark of **bacterial conjunctivitis**, not intraocular inflammation. **Why Mucopurulent Discharge is the Exception:** In iridocyclitis, the inflammation is internal. While there may be reflex lacrimation (watery discharge) due to pain, there is no bacterial infection of the conjunctival surface to produce pus or mucus. The presence of discharge should immediately point the clinician toward a diagnosis of conjunctivitis rather than uveitis. **Analysis of Other Options:** * **Pain:** This is a cardinal feature of acute iridocyclitis. It is typically a deep, throbbing ache caused by ciliary muscle spasm and irritation of trigeminal nerve endings. * **Ciliary Congestion:** Also known as circumcorneal flush, this is a dusky red injection most intense at the limbus. It occurs due to the engorgement of deep episcleral vessels that supply the iris and ciliary body. * **Small Pupil (Miosis):** The pupil is small and sluggishly reactive due to sphincter pupillae spasm and the presence of inflammatory exudates. This helps differentiate it from acute angle-closure glaucoma, where the pupil is mid-dilated and oval. **High-Yield Clinical Pearls for NEET-PG:** * **The "Triple Sign" of Iridocyclitis:** Ciliary congestion, miosis, and aqueous cells/flare (seen on slit-lamp). * **Keratic Precipitates (KPs):** Inflammatory cells on the corneal endothelium; "Mutton-fat" KPs suggest granulomatous uveitis (e.g., Sarcoidosis, TB). * **Festooned Pupil:** An irregular pupil shape caused by posterior synechiae (adhesions between the iris and lens). * **Treatment Gold Standard:** Topical steroids (to reduce inflammation) and Cycloplegics like Atropine (to relieve pain and prevent synechiae).

Q: Granulomatous uveitis is seen in which of the following conditions?

Vogt-Koyanagi-Harada disease. **Explanation:** Uveitis is clinically classified into **Granulomatous** and **Non-granulomatous** based on the type of inflammatory response and the appearance of Keratic Precipitates (KPs). **1. Why Vogt-Koyanagi-Harada (VKH) disease is correct:** VKH is a multisystem autoimmune disorder directed against melanocytes. It typically presents as a **bilateral chronic granulomatous panuveitis**. On examination, it is characterized by "mutton-fat" Keratic Precipitates (KPs), Busacca nodules on the iris, and Dalen-Fuchs nodules in the choroid. Systemic features include poliosis, vitiligo, alopecia, and meningeal signs. **2. Why the other options are incorrect:** * **Fuch’s Heterochromic Iridocyclitis (FHI):** This is a chronic **non-granulomatous** uveitis. It is characterized by fine, stellate KPs distributed over the entire corneal endothelium, iris heterochromia, and an absence of posterior synechiae. * **Behcet’s Syndrome:** This is a multisystem vasculitis that causes a classic **non-granulomatous** uveitis. It is famous for presenting with a "shifting hypopyon" (sterile pus in the anterior chamber) and occlusive retinal vasculitis. **NEET-PG High-Yield Pearls:** * **Granulomatous Uveitis Mnemonic (STLV):** **S**arcoidosis, **T**uberculosis, **L**eprosy, **V**KH (also includes Syphilis and Sympathetic Ophthalmitis). * **Mutton-fat KPs:** Large, greasy-looking clusters of epithelioid cells and macrophages, pathognomonic for granulomatous inflammation. * **HLA Association:** VKH is strongly associated with **HLA-DR4**. * **Behcet’s Association:** Associated with **HLA-B51**.

Q: All of the following are true for anaemic retinopathy except?

Veins are pale and narrow. **Explanation:** Anaemic retinopathy occurs due to retinal hypoxia and increased capillary permeability resulting from low oxygen-carrying capacity. **Why Option C is the correct answer (The False Statement):** In anaemic retinopathy, the retinal **veins are typically dilated and tortuous**, not narrow. This dilation is a compensatory mechanism to improve blood flow in response to systemic hypoxia. While the veins do appear **pale** (due to the reduction in red blood cell concentration), the "narrow" description makes this statement incorrect. **Analysis of Incorrect Options (True Statements):** * **Option A:** Retinopathy is rarely seen unless the anaemia is severe. It typically manifests when haemoglobin levels fall below **5 g/dL** or when the red cell count drops below 1.5 million/mm³. * **Option B:** Both arterioles and veins appear pale or "straw-colored" because the blood contains less oxyhaemoglobin, leading to a loss of the normal bright red color. * **Option C:** Common findings include flame-shaped (superficial) haemorrhages, Roth spots (haemorrhages with white centers), and occasionally subhyaloid haemorrhages. These occur due to endothelial damage from hypoxia. **High-Yield Clinical Pearls for NEET-PG:** * **Roth Spots:** Though classic for Subacute Bacterial Endocarditis (SBE), they are frequently seen in anaemic and leukaemic retinopathy. * **Combined Effect:** Retinopathy is significantly more severe if anaemia is associated with concomitant **thrombocytopenia**. * **Reversibility:** Anaemic retinopathy is generally reversible once the underlying systemic anaemia is corrected. * **Cotton Wool Spots:** These may also be present, representing micro-infarctions of the nerve fiber layer.

Q: What is the commonest cause of fungal uveitis?

Candida. **Explanation:** **Candida albicans** is the most common cause of fungal uveitis (specifically endogenous endophthalmitis). This occurs primarily via **hematogenous spread** from a distant site. The underlying medical concept involves the seeding of the highly vascular choroid during episodes of fungemia. It is most frequently seen in immunocompromised patients, intravenous drug users (IVDU), or those on long-term indwelling catheters or parenteral nutrition. **Analysis of Options:** * **Candida (Correct):** It typically presents as "fluffy white cotton-ball" colonies in the vitreous and multifocal chorioretinitis. It is the leading cause of endogenous fungal infections of the eye. * **Aspergillus:** While it is the second most common cause of endogenous fungal endophthalmitis, it is less frequent than Candida. It tends to be more aggressive, often causing rapid vision loss and vascular occlusion. * **Fusarium:** This is the most common cause of **fungal keratitis** (exogenous infection) worldwide, particularly following trauma with vegetative matter, but it is a rare cause of uveitis. * **Mucormycosis:** This typically causes **Rhino-oculo-cerebral mucormycosis**, characterized by orbital cellulitis and apex syndrome in diabetic ketoacidosis patients, rather than isolated uveitis. **High-Yield Clinical Pearls for NEET-PG:** * **Endogenous Fungal Endophthalmitis:** Most common organism is *Candida*. * **Exogenous Fungal Keratitis:** Most common organism is *Fusarium* (filamentous fungi). * **Classic Sign:** "String of pearls" or "Cotton ball" opacities in the vitreous are pathognomonic for Candidal endophthalmitis. * **Treatment:** Intravitreal Amphotericin B or Voriconazole are the drugs of choice.

Q: A 30-year-old male patient presented with anterior granulomatous uveitis, arthralgia, and respiratory difficulties. What is the probable diagnosis?

Ocular sarcoidosis. **Explanation:** The clinical triad of **granulomatous uveitis, arthralgia, and respiratory symptoms** in a young adult is a classic presentation of **Sarcoidosis**. 1. **Why Sarcoidosis is correct:** Sarcoidosis is a multisystem idiopathic disorder characterized by non-caseating granulomas. Ocular involvement occurs in about 25–50% of cases, most commonly presenting as **bilateral granulomatous anterior uveitis** (characterized by "mutton-fat" keratic precipitates and Iris nodules like Koeppe and Bussaca nodules). The systemic association with hilar lymphadenopathy or interstitial lung disease explains the respiratory difficulty, while joint involvement causes arthralgia. 2. **Why other options are incorrect:** * **Tuberculosis:** While it causes granulomatous uveitis and respiratory issues, it is usually associated with weight loss, night sweats, and fever. The specific combination with arthralgia (Lofgren’s syndrome) is more characteristic of Sarcoidosis. * **Behcet’s Syndrome:** This typically presents with **non-granulomatous** uveitis (often with a shifting hypopyon) and is characterized by the triad of oral ulcers, genital ulcers, and uveitis, rather than respiratory distress. * **HLA-B27 associated uveitis:** This is the most common cause of **acute non-granulomatous** anterior uveitis. While associated with Ankylosing Spondylitis (back pain), it does not typically present with granulomatous features or primary respiratory distress. **High-Yield Clinical Pearls for NEET-PG:** * **Lofgren’s Syndrome:** An acute form of sarcoidosis consisting of Erythema nodosum, bilateral hilar lymphadenopathy, and arthralgia. * **Heerfordt’s Syndrome (Uveoparotid fever):** Uveitis, Parotid swelling, and Facial nerve palsy. * **Investigation of Choice:** Chest X-ray (Bilateral hilar lymphadenopathy) and Serum ACE levels (elevated). * **Definitive Diagnosis:** Biopsy showing non-caseating granulomas.

Q: Which of the following is NOT a cause of Roth spots?

Hypersensitivity pneumonitis. **Explanation:** **Roth spots** are retinal hemorrhages characterized by a pale or white center. Pathophysiologically, they represent a **fibrin-platelet thrombus** at the site of a ruptured capillary wall. The white center is composed of fibrin, platelets, and focal ischemia, rather than inflammatory cells. **Why Hypersensitivity Pneumonitis is the correct answer:** Hypersensitivity pneumonitis is an immunologically mediated inflammation of the lung parenchyma (Type III and IV hypersensitivity). It does not typically cause the systemic hematological disturbances or microvascular damage required to produce retinal hemorrhages. Therefore, it is not associated with Roth spots. **Why the other options are incorrect:** * **Leukemia:** This is a classic cause. Hyperviscosity and abnormal white blood cell counts lead to capillary fragility and hemorrhage. * **Severe Anemia:** Low hemoglobin levels lead to retinal hypoxia and increased capillary permeability, resulting in flame-shaped hemorrhages with pale centers. * **Systemic Lupus Erythematosus (SLE):** SLE causes a systemic vasculitis. Immune complex deposition in the retinal vessels leads to micro-infarctions and hemorrhages. **Clinical Pearls for NEET-PG:** * **Mnemonic for Roth Spots:** "**L**ads **S**hould **B**e **D**oing **H**omework" (**L**eukemia, **S**ubacute Bacterial Endocarditis (SBE), **B**lastomycosis, **D**iabetes, **H**ypertension/HIV). * **Most Common Association:** While classically taught with **Subacute Bacterial Endocarditis (SBE)**, they are actually more frequently seen in **Leukemia** and **Diabetes Mellitus**. * **Differential Diagnosis:** Other causes include Carbon Monoxide poisoning, Preeclampsia, and Vitamin B12 deficiency.

Q: Nodules seen near the collarette are called?

Busacca nodules. **Explanation:** The question refers to the clinical features of **Granulomatous Uveitis**. In this condition, inflammatory cells (lymphocytes and epithelioid cells) aggregate on the iris surface to form nodules. 1. **Busacca Nodules (Correct Answer):** These are inflammatory nodules located on the **anterior surface of the iris stroma**, typically near the **collarette**. They are pathognomonic for granulomatous uveitis (e.g., Sarcoidosis, Tuberculosis). 2. **Koeppe’s Nodules:** These are also seen in granulomatous uveitis but are located specifically at the **pupillary margin**. They are smaller than Busacca nodules and can lead to the formation of posterior synechiae. 3. **Lisch Nodules:** These are melanocytic hamartomas of the iris. They appear as well-defined, dome-shaped, light brown elevations. They are a hallmark diagnostic feature of **Neurofibromatosis Type 1 (NF-1)** and are not inflammatory. 4. **Dalén-Fuchs Nodules:** These are small, yellowish-white spots seen in the **chorioretinal periphery**. They represent aggregates of epithelioid cells between the retinal pigment epithelium (RPE) and Bruch’s membrane, typically seen in **Vogt-Koyanagi-Harada (VKH) syndrome** and **Sympathetic Ophthalmitis**. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic:** **K**oeppe = **K**orner (Pupillary margin); **B**usacca = **B**ody (Iris stroma/collarette). * **Mutton-fat Keratic Precipitates (KPs):** Large, greasy inflammatory deposits on the corneal endothelium, also characteristic of granulomatous uveitis. * **Berlin’s Nodules:** Inflammatory nodules found in the angle of the anterior chamber (seen on gonioscopy).

Question 1

A 47-year-old woman develops accelerated hypertension (blood pressure 210/105 mm Hg) but no clinical symptoms except frequent headaches. Which of the following findings are most likely on examination of the fundii?

Accepted Answer

cotton wool spots

Answer

retinitis obliterans

Answer

retinal detachment

Answer

optic atrophy

Question 2

Marfan's syndrome has which of the following prominent eye defects?

Accepted Answer

Ectopia lentis

Answer

Megalocornea

Answer

Microcornea

Answer

Microspherophakia

Question 3

All are seen in acute iridocyclitis except:

Accepted Answer

Mucopurulent discharge

Answer

Pain

Answer

Ciliary congestion

Answer

Small pupil

Question 4

Granulomatous uveitis is seen in which of the following conditions?

Accepted Answer

Vogt-Koyanagi-Harada disease

Answer

Fuch's disease

Answer

Behcet's syndrome

Answer

All of the above

Question 5

All of the following are true for anaemic retinopathy except?

Accepted Answer

Veins are pale and narrow

Answer

Occurs when haemoglobin level falls below 5 gm%

Answer

Arterioles become pale

Answer

Superficial retinal and subhyaloid haemorrhage are seen

Question 6

What is the commonest cause of fungal uveitis?

Accepted Answer

Candida

Answer

Aspergillus

Answer

Fusarium

Answer

Mucor mycosis

Question 7

A 30-year-old male patient presented with anterior granulomatous uveitis, arthralgia, and respiratory difficulties. What is the probable diagnosis?

Accepted Answer

Ocular sarcoidosis

Answer

Tuberculosis

Answer

Behcet's syndrome

Answer

HLA B27 associated uveitis

Question 8

Which of the following is NOT a cause of Roth spots?

Accepted Answer

Hypersensitivity pneumonitis

Answer

Leukemia

Answer

Severe anemia

Answer

Systemic lupus erythematosus

Question 9

Nodules seen near the collarette are called?

Accepted Answer

Busacca nodules

Answer

Koeppe's nodules

Answer

Lisch nodules

Answer

Dalén-Fuchs nodules

Question 10

A 12-year-old girl presents with mood and emotional lability and a golden brown discoloration in Descemet's membrane. What is the most likely diagnosis?

Accepted Answer

Wilson's disease

Answer

Fabry's disease

Answer

Glycogen storage disease

Answer

Acute rheumatic fever

Ocular Manifestations of Systemic Disorders — MCQs

Ocular Manifestations of Systemic Disorders — MCQs

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