Ocular Manifestations of Systemic Disorders Practice Questions

Q: Which of the following is NOT true in ocular ischemic syndrome?

Neovascularization of the iris (NVI) is never seen.. **Explanation:** **Ocular Ischemic Syndrome (OIS)** is a rare but vision-threatening condition caused by chronic ocular hypoperfusion, most commonly due to severe atherosclerotic stenosis of the internal or common carotid artery. **Why Option D is the Correct Answer:** Neovascularization of the iris (NVI), or rubeosis iridis, is actually a hallmark feature of OIS, occurring in approximately **60-90% of cases**. Chronic ischemia triggers the release of Vascular Endothelial Growth Factor (VEGF), leading to NVI. If left untreated, this often progresses to neovascular glaucoma (NVG). Therefore, stating that NVI is "never seen" is clinically incorrect. **Analysis of Other Options:** * **Option A:** OIS typically manifests when carotid artery stenosis reaches **90% or more**. At this level of obstruction, the perfusion pressure in the ophthalmic artery drops by approximately 50%. * **Option B:** Patients often complain of a **"vascular headache"** or dull periorbital ache (ocular angina). This pain is due to ischemia of the globe or increased intraocular pressure and is characteristically worse when the patient is upright/arising due to orthostatic changes in perfusion. * **Option C:** **Delayed recovery after light stress** (photostress test) is a classic symptom. The ischemic photoreceptors take significantly longer to regenerate visual pigments after being "bleached" by bright light. **NEET-PG High-Yield Pearls:** * **Fundus Findings:** Look for narrowed retinal arteries, dilated (but not tortuous) retinal veins, and mid-peripheral "dot-and-blot" hemorrhages. * **Differential Diagnosis:** Unlike Central Retinal Vein Occlusion (CRVO), OIS veins are dilated but **not** tortuous, and the hemorrhages are primarily in the mid-periphery. * **Systemic Importance:** OIS is a strong predictor of systemic vascular disease; the 5-year mortality rate is nearly 40%, primarily due to myocardial infarction.

Q: Which of the following are signs of uveitis?

Shallow anterior chamber. **Explanation:** In the context of this specific question, **Shallow anterior chamber** is the correct answer because it is a classic sign of **Acute Congestive Glaucoma**, which is the primary differential diagnosis for Acute Uveitis. In Uveitis, the anterior chamber depth is typically **normal or deep**. **Why the other options are characteristic of Uveitis:** * **Circumciliary Congestion (B):** This is a hallmark sign of acute anterior uveitis. It presents as a violaceous/purplish hue around the limbus due to the engorgement of anterior ciliary vessels. * **Cells and Flare (C):** These are the pathognomonic signs of active anterior uveitis. **Cells** (leukocytes) indicate active inflammation, while **Flare** (Tyndall effect) indicates protein leakage due to a breakdown of the blood-aqueous barrier. * **Generalized Conjunctival Congestion (A):** While more typical of conjunctivitis, some degree of diffuse redness can occur in uveitis, though it is less specific than circumciliary congestion. **Clinical Pearls for NEET-PG:** * **The "Big Three" Differentials:** Always differentiate Acute Uveitis from Acute Glaucoma and Acute Conjunctivitis. * **Pupil Size:** In Uveitis, the pupil is **small (miotic)** and sluggish due to sphincter spasm. In Glaucoma, it is **mid-dilated and vertically oval**. * **Intraocular Pressure (IOP):** IOP is usually **low or normal** in uveitis (due to ciliary body "stunning") but **severely elevated** in glaucoma. * **Keratic Precipitates (KPs):** These are inflammatory deposits on the corneal endothelium, a key diagnostic sign of uveitis. Large "mutton-fat" KPs suggest granulomatous uveitis (e.g., Sarcoidosis, TB).

Q: Recurrent bilateral hypopyon formation associated with thrombophlebitis is most consistent with which of the following?

Behcet's syndrome. **Explanation:** The correct answer is **Behcet’s syndrome**. This is a multisystemic, chronic relapsing inflammatory perivasculitis of unknown etiology, classically characterized by the triad of oral ulcers, genital ulcers, and uveitis. **Why Behcet’s Syndrome is correct:** The hallmark ocular finding in Behcet’s is **recurrent, bilateral non-granulomatous uveitis**. A unique feature is the **"shifting" or "transient" hypopyon**, which moves with head position due to its low fibrin content. Systemically, Behcet’s is a systemic vasculitis that frequently involves both arteries and veins; **thrombophlebitis** (superficial or deep vein thrombosis) is a major diagnostic criterion and a key differentiator in this question. **Why other options are incorrect:** * **HLA-B27 associated uveitis:** While it causes recurrent unilateral (alternating) acute anterior uveitis with heavy fibrin (fixed hypopyon), it is typically associated with sacroiliitis or ankylosing spondylitis, not thrombophlebitis. * **Syphilis:** Known as the "Great Mimicker," it usually presents with granulomatous uveitis or "salt and pepper" fundus. It does not typically present with recurrent shifting hypopyon and thrombophlebitis. * **Herpes Zoster:** Causes unilateral hypertensive uveitis (increased IOP) associated with sectorial iris atrophy and a vesicular rash in the trigeminal distribution. **High-Yield Clinical Pearls for NEET-PG:** * **Pathergy Test:** A skin hyper-reactivity test (formation of a sterile pustule 24-48 hours after a needle prick) is highly specific for Behcet’s. * **Hypopyon characteristics:** In Behcet’s, the hypopyon is sterile and shifts easily. In HLA-B27, it is "sticky" and fixed. * **Posterior segment:** Behcet’s can cause "occlusive vasculitis" (retinal peri-phlebitis), leading to a "stormy sunset" appearance of the fundus.

Q: All of the following are seen in albinism except:

Glaucoma. **Explanation:** Albinism is a group of inherited disorders characterized by a deficiency in melanin synthesis. The correct answer is **Glaucoma**, as it is not a characteristic feature or a direct consequence of albinism. **Why Glaucoma is the Correct Answer:** Glaucoma involves increased intraocular pressure and optic nerve damage. In albinism, the primary pathology is the lack of pigment in the uveal tract (iris and choroid) and the retinal pigment epithelium (RPE). While albinos may develop glaucoma coincidentally like the general population, it is not a manifestation of the disease itself. **Analysis of Other Options:** * **Photophobia:** Due to the lack of pigment in the iris (iris transillumination), excess light enters the eye, causing significant light sensitivity. * **Refractive Error:** High degrees of astigmatism and hyperopia/myopia are extremely common in albinism due to abnormal ocular development. * **Nystagmus:** This occurs secondary to **foveal hypoplasia** (underdevelopment of the macula). Because the retina cannot provide a sharp central image, the eyes develop a searching, rhythmic oscillation. **High-Yield Clinical Pearls for NEET-PG:** * **Foveal Hypoplasia:** The most important cause of poor visual acuity in albinos. * **Misrouting of Optic Fibers:** A classic finding where there is excessive decussation (crossing) of nerve fibers at the optic chiasm. * **Fundus Appearance:** The fundus appears bright orange-red, and choroidal vessels are easily visible due to the absence of RPE pigment. * **Systemic Associations:** Always rule out **Chediak-Higashi syndrome** (immunodeficiency) and **Hermansky-Pudlak syndrome** (bleeding diathesis) in patients with albinism.

Q: What is the most common cause of visual loss in patients with AIDS?

CMV retinitis. **Explanation:** **CMV Retinitis (Correct Answer):** Cytomegalovirus (CMV) retinitis is the most common opportunistic ocular infection and the leading cause of blindness in patients with AIDS, typically occurring when the **CD4+ T-cell count falls below 50 cells/µL**. It is characterized by a "pizza-pie" or "cheese and ketchup" appearance (confluent areas of yellow-white necrosis with associated retinal hemorrhage). Visual loss occurs due to macular involvement, optic nerve atrophy, or secondary rhegmatogenous retinal detachment. **Incorrect Options:** * **Toxoplasmosis:** While it is the most common cause of posterior uveitis in the general population, in AIDS patients, it is less common than CMV. It typically presents as a "headlight in the fog" appearance due to intense overlying vitritis. * **Herpetic Retinitis:** Includes Acute Retinal Necrosis (ARN) and Progressive Outer Retinal Necrosis (PORN). While highly destructive and rapidly progressive, these are statistically less frequent than CMV retinitis. * **HIV Retinitis:** This is the most common *finding* in the eyes of HIV patients (presenting as cotton wool spots), but it is usually asymptomatic and rarely causes significant visual loss. **High-Yield Clinical Pearls for NEET-PG:** * **Most common ocular finding in AIDS:** HIV Microangiopathy (Cotton wool spots). * **Most common ocular infection/cause of blindness in AIDS:** CMV Retinitis. * **Drug of choice for CMV Retinitis:** Induction with Ganciclovir (IV or intravitreal) or Valganciclovir (oral). * **Immune Recovery Uveitis (IRU):** An inflammatory reaction that occurs in AIDS patients with CMV retinitis after starting HAART, due to a rising CD4 count.

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).

Question 1

Which of the following is NOT true in ocular ischemic syndrome?

Accepted Answer

Neovascularization of the iris (NVI) is never seen.

Answer

It occurs when carotid artery obstruction reaches 90%.

Answer

Dull pain in the orbital region that worsens on arising.

Answer

Delayed recovery of vision following bright light exposure is seen.

Question 2

Which of the following are signs of uveitis?

Accepted Answer

Shallow anterior chamber

Answer

Generalized conjunctival congestion

Answer

Circumciliary congestion

Answer

Cells and flare in aqueous

Question 3

Recurrent bilateral hypopyon formation associated with thrombophlebitis is most consistent with which of the following?

Accepted Answer

Behcet's syndrome

Answer

HLA B27 associated uveitis

Answer

Syphilis

Answer

Herpes Zoster

Question 4

Which of the following investigations is NOT typically indicated for anterior uveitis in a 25-year-old male?

Accepted Answer

Ultrasound of the abdomen

Answer

HLA-B27

Answer

X-ray of the sacroiliac joint

Answer

TORCH agent testing

Question 5

All of the following are seen in albinism except:

Accepted Answer

Glaucoma

Answer

Photophobia

Answer

Refractive error

Answer

Nystagmus

Question 6

A patient presents with a history of dactylitis, anemia, and growth retardation. Funduscopic examination reveals sea fan neovascularization and salmon patches. What is the probable diagnosis?

Accepted Answer

Sickle cell anemia

Answer

Syphilis

Answer

Toxoplasmosis

Answer

Histoplasmosis

Question 7

What is the most common cause of visual loss in patients with AIDS?

Accepted Answer

CMV retinitis

Answer

Toxoplasmosis

Answer

Herpetic retinitis

Answer

HIV retinitis

Question 8

All of the following are true for Vogt-Koyanagi-Harada syndrome except:

Accepted Answer

More common in Japanese people, who are usually positive for HLA-DR4

Answer

Ocular features include chronic granulomatous anterior uveitis, posterior uveitis, and exudative retinal detachment

Answer

Cutaneous lesions include alopecia, poliosis, and vitiligo

Answer

Neurological lesions include meningism, encephalopathy, tinnitus, vertigo, and deafness

Question 9

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 10

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

Accepted Answer

Ectopia lentis

Answer

Megalocornea

Answer

Microcornea

Answer

Microspherophakia

Ocular Manifestations of Systemic Disorders — MCQs

Ocular Manifestations of Systemic Disorders — MCQs

On this page

Practice by Chapter

Want unlimited practice?