Diseases of the Lens Practice Questions

Q: Which of the following conditions is associated with ectopia lentis?

Homocystinuria. ***Homocystinuria*** - **Ectopia lentis** (lens dislocation) is a common and characteristic ocular manifestation of homocystinuria. - The lens typically dislocates **downward and inward**, differentiating it from Marfan syndrome. *Alport syndrome* - Characterized by **glomerulonephritis**, **sensorineural hearing loss**, and ocular abnormalities. - Ocular manifestations include **anterior lenticonus** (which can be mistaken for ectopia lentis in some descriptions), posterior polymorphous corneal dystrophy, and retinal flecks, but not classic ectopia lentis. *Lowe syndrome* - Also known as oculocerebrorenal syndrome of Lowe, it primarily affects the **eyes, brain, and kidneys**. - Ocular features include **congenital cataracts** and glaucoma, but not ectopia lentis. *Sulphite oxidase deficiency* - This is a rare metabolic disorder affecting the metabolism of sulfur-containing amino acids, leading to severe neurological symptoms. - While it can manifest with **cataracts** and **lens subluxation** in some cases, ectopia lentis is more characteristically associated with homocystinuria, and the overall clinical picture of sulphite oxidase deficiency is dominated by severe neurological impairment.

Q: Polyopia/diplopia is seen in which type of cataract?

Cortical cataract. ***Cortical cataract*** - **Cortical cataracts** cause spokes or vacuoles that refract light in multiple directions, leading to **polyopia** or **diplopia**. - The irregular opacities disrupt the light path, causing the brain to perceive **multiple images**. *Nuclear cataract* - **Nuclear cataracts** primarily affect central vision and cause **myopic shift**, not typically polyopia or diplopia. - They cause **blurry vision** and difficulty seeing in dim light as the lens nucleus hardens and yellows. *Posterior subcapsular cataract* - **Posterior subcapsular cataracts** are characterized by glare and difficulty with reading, mainly affecting near vision. - They form at the back of the lens, close to the nodal point, causing earlier onset of **visual symptoms** with less impact on polyopia. *Anterior polar cataract* - **Anterior polar cataracts** are usually small, well-demarcated opacities at the front of the lens that typically do not cause significant visual impairment. - They rarely cause **polyopia** unless they are very large or dense, which is uncommon.

Q: Elschnig's pearls are a clinical sign of which condition?

Secondary cataract. ***Secondary cataract*** - **Elschnig's pearls** are bladder-like cells formed by **proliferation of residual equatorial lens epithelial cells** following extracapsular cataract extraction (ECCE) or phacoemulsification - They migrate posteriorly along the posterior capsule, contributing to **posterior capsular opacification (PCO)** - PCO is commonly called **"secondary cataract"** or **"after-cataract"** and is the most common complication of cataract surgery - Presents with **gradual visual decline** months to years after initially successful cataract surgery - Treated with **Nd:YAG laser capsulotomy** *Chronic uveitis* - While chronic uveitis causes various ocular complications (**synechiae**, **band keratopathy**, **cataract formation**, **macular edema**), **Elschnig's pearls are not a sign of uveitis** - They are specifically a **post-surgical complication** of cataract extraction, not an inflammatory finding - Uveitis patients may develop cataracts requiring surgery, which could subsequently lead to Elschnig's pearls, but this is an indirect relationship *Cystoid macular oedema* - **CME** involves fluid accumulation in the macula forming **cyst-like spaces**, causing decreased central vision - Can occur after cataract surgery (Irvine-Gass syndrome) or with uveitis - Has **completely different pathophysiology and clinical appearance** from Elschnig's pearls - CME affects the **retina**, while Elschnig's pearls involve the **lens capsule** *None of the options* - Incorrect because **secondary cataract (PCO)** is the established correct answer for Elschnig's pearls

Q: In the context of homocystinuria, which direction does the lens typically subluxate?

Inferonasal. ***Inferonasal*** - In **homocystinuria**, the **ectopia lentis** (lens subluxation) often occurs due to weakening of the **zonular fibers**. - The classic direction for lens subluxation in homocystinuria is **inferior and nasal**. *Inferotemporal* - While lens subluxation can occur in various directions, **inferotemporal** is not the classic or most common presentation in homocystinuria. - This direction is less specific and does not strongly point to homocystinuria as the underlying cause. *Superonasal* - **Superonasal** dislocation of the lens is more characteristic of **Marfan syndrome**, which is important to differentiate from homocystinuria. - This direction helps distinguish different causes of lens ectopia. *Superotemporal* - **Superotemporal** lens subluxation is the hallmark of **Marfan syndrome**, a genetic connective tissue disorder. - This specific finding is crucial for differential diagnosis in patients presenting with lens ectopia.

Q: What is the most common type of congenital cataract?

Blue dot. ***Blue dot*** - **Blue dot (punctate) cataracts** are the **most common type of congenital cataract overall**. - They appear as **multiple small, blue-white opacities** scattered throughout the lens cortex. - These cataracts are typically **bilateral, non-progressive, and asymptomatic**, rarely affecting visual acuity. - Often considered **physiological variants**, they usually do not require surgical intervention. *Zonular (Lamellar)* - **Zonular (lamellar) cataracts** are the **most common visually significant** congenital cataract. - They feature **opacities arranged in layers or lamellae** within the lens, classically with alternating clear and opaque zones. - Unlike blue dot cataracts, these **often require surgical intervention** due to visual impairment. *Capsular* - **Capsular cataracts** involve the anterior or posterior lens capsule and are relatively uncommon. - They appear as **small, well-circumscribed opacities** on the lens capsule. - Visual impact depends on size and location relative to the visual axis. *Coralliform* - **Coralliform cataracts** are a rare type characterized by **coral-like branching opacities**. - This distinctive morphology makes them one of the less common congenital cataract types.

Q: Which type of cataract is specifically associated with decreased reading ability?

Nuclear cataract. ***Nuclear cataract*** - **Nuclear cataracts** cause progressive hardening and yellowing of the lens nucleus with increased refractive index - This produces a **myopic shift** that initially causes **"second sight"** (temporary improvement in near vision) - However, as the cataract progresses, the increasing opacity leads to **overall visual decline affecting both distance and near vision**, including reading ability - Among the given options, nuclear cataract is the most common age-related cataract that significantly impairs vision including reading *Blue dot cataract* - **Blue dot cataracts** (cerulean cataracts) are small, bluish peripheral opacities, usually congenital and stationary - They rarely cause significant visual impairment and do not affect reading ability *Fusiform cataract* - **Fusiform cataracts** are congenital spindle-shaped opacities along the visual axis - While they can affect vision if dense, they are rare and not typically associated with progressive reading difficulty *Punctate cataract* - **Punctate cataracts** are small, scattered dot-like opacities in the lens - They are often congenital or age-related and cause minimal visual disturbance - Not specifically associated with decreased reading ability

Q: What is the first sign of iridocyclitis?

Aqueous flare. ***Aqueous flare*** - This is the earliest and most subtle sign, representing an increase in **protein content** in the aqueous humor due to breakdown of the **blood-aqueous barrier**. - It is detected by a **slit-lamp examination** as a hazy appearance in the anterior chamber, similar to a car headlight beam in fog. *KP* - **Keratic precipitates (KP)** are deposits of inflammatory cells on the corneal endothelium. - While characteristic of iridocyclitis, they typically appear **after** the initial breakdown of the blood-aqueous barrier that causes aqueous flare. *Congestion* - **Ciliary congestion** (perilimbal redness) is a common symptom of anterior uveitis, but it is a visible sign of inflammation. - The underlying inflammatory process causing the congestion first manifests as **subtle changes in the aqueous humor**, which is aqueous flare. *Trichiasis* - **Trichiasis** is the misdirection of eyelashes to rub against the cornea, causing irritation. - It is an **external ocular condition** and is not a sign of intraocular inflammation like iridocyclitis.

Q: Which type of cataract is associated with the phenomenon known as 'second sight'?

Nuclear cataract. ***Nuclear cataract*** - A nuclear cataract causes the lens to become more **myopic** or nearsighted due to an increase in its **refractive index**. - This temporary increase in nearsightedness can allow elderly individuals who previously needed reading glasses (due to presbyopia) to read without them, a phenomenon known as "**second sight**." *Cortical cataract* - Cortical cataracts primarily affect the **outer layer or cortex** of the lens, appearing as spokes or wedges radiating from the periphery. - They tend to cause **glare** and **reduced peripheral vision**, but generally do not improve near vision. *Zonular cataract* - A zonular cataract is a **congenital type of cataract**, characterized by opacity in specific layers (lamellae) of the lens, often in the fetal nucleus. - It does not typically lead to the "second sight" phenomenon, as it is present from birth and affects vision differently. *Punctate cataract* - Punctate cataracts are small, **dot-like opacities** scattered throughout the lens, often considered benign. - They are usually **incidental findings** and rarely cause significant visual disturbances or "second sight."

Q: Which type of congenital cataract is commonly associated with significant visual defects?

Nuclear cataract. ***Nuclear cataract*** - It involves the **nucleus** of the lens, which is the central and most visually critical part. - This type of cataract can cause **significant visual impairment** due to its central location and density, impacting early visual development. *Punctate Cataract* - These are **small, dot-like opacities** that are usually clinically insignificant and do not typically cause significant visual defects. - They are often **peripheral** or very fine, thus not obstructing the central visual axis. *Blue dot cataract* - Also known as **cerulean cataract**, this type consists of small, bluish opacities in the lens cortex. - While congenital, it often has **minimal impact on vision** and is frequently observed incidentally. *Fusiform cataract* - This cataract forms in the shape of a **spindle or needle**, usually along the visual axis. - While it can sometimes cause minor visual compromise, its impact is generally **less severe** compared to a dense nuclear cataract.

Q: The gene known to be associated with cataract is

CRYAA. ***CRYAA*** - The **CRYAA (crystallin alpha A)** gene is a significant contributor to both congenital and age-related cataracts. - Mutations in **crystallin genes** disrupt the normal structure and transparency of the lens proteins, leading to opacification. - CRYAA is one of the most commonly implicated crystallin genes in hereditary cataract formation. *PAX6* - **PAX6** is a master control gene for eye development and is primarily associated with **aniridia**, a condition characterized by a partial or complete absence of the iris. - While aniridia can be associated with secondary cataracts, PAX6 mutations are not a direct cause of primary cataract formation. *FKHL7* - This gene is more commonly referred to as **FOXC1** and is implicated in **ocular anterior segment dysgenesis**, such as **Axenfeld-Rieger syndrome**. - These conditions can lead to glaucoma and other ocular anomalies but are not directly linked to cataract as their primary presentation in the way crystallin genes are. *CYP1B1* - The **CYP1B1** gene is most notably associated with **primary congenital glaucoma**, particularly in individuals of Arab and Gypsy descent. - Mutations in this gene affect the development of the aqueous humor outflow pathways, leading to elevated intraocular pressure and optic nerve damage, not primarily cataract.

Question 1

Which of the following conditions is associated with ectopia lentis?

Accepted Answer

Homocystinuria

Answer

Alport syndrome

Answer

Lowe syndrome

Answer

Sulphite oxidase deficiency

Question 2

Polyopia/diplopia is seen in which type of cataract?

Accepted Answer

Cortical cataract

Answer

Nuclear cataract

Answer

Posterior subcapsular cataract

Answer

Anterior polar cataract

Question 3

Elschnig's pearls are a clinical sign of which condition?

Accepted Answer

Secondary cataract

Answer

Chronic uveitis

Answer

Cystoid macular oedema

Answer

None of the options

Question 4

In the context of homocystinuria, which direction does the lens typically subluxate?

Accepted Answer

Inferonasal

Answer

Inferotemporal

Answer

Superonasal

Answer

Superotemporal

Question 5

What is the most common type of congenital cataract?

Accepted Answer

Blue dot

Answer

Capsular

Answer

Coralliform

Answer

Zonular

Question 6

Which type of cataract is specifically associated with decreased reading ability?

Accepted Answer

Nuclear cataract

Answer

Blue dot cataract

Answer

Fusiform cataract

Answer

Punctate cataract

Question 7

What is the first sign of iridocyclitis?

Accepted Answer

Aqueous flare

Answer

KP

Answer

Congestion

Answer

Trichiasis

Question 8

Which type of cataract is associated with the phenomenon known as 'second sight'?

Accepted Answer

Nuclear cataract

Answer

Cortical cataract

Answer

Zonular cataract

Answer

Punctate cataract

Question 9

Which type of congenital cataract is commonly associated with significant visual defects?

Accepted Answer

Nuclear cataract

Answer

Punctate Cataract

Answer

Blue dot cataract

Answer

Fusiform cataract

Question 10

The gene known to be associated with cataract is

Accepted Answer

CRYAA

Answer

PAX6

Answer

FKHL7

Answer

CYP1B1

Diseases of the Lens — MCQs

Diseases of the Lens — MCQs

On this page

Practice by Chapter

Want unlimited practice?