Typically bilateral inferior subluxations of the lens is seen in –
## **Core Concept**
The question tests knowledge on the ocular manifestations associated with systemic conditions, specifically focusing on the characteristic lens subluxation seen in certain disorders. Lens subluxation refers to the partial displacement of the lens from its normal position, often associated with conditions affecting the connective tissue.
## **Why the Correct Answer is Right**
The correct answer, **Marfan's syndrome**, is a genetic disorder that affects the body's connective tissue. A common ocular manifestation of Marfan's syndrome is the bilateral inferior or temporal subluxation of the lens, also known as ectopia lentis. This occurs due to the weakness of the zonular fibers (or suspensory ligaments) that hold the lens in place, which is a result of the defective fibrillin-1 protein produced in Marfan's syndrome.
## **Why Each Wrong Option is Incorrect**
- **Option A:** While **Ehlers-Danlos syndrome** also affects connective tissue and can have ocular manifestations, it is more commonly associated with ocular fragility and less specifically with bilateral inferior lens subluxation compared to Marfan's syndrome.
- **Option B:** **Homocystinuria** is a metabolic disorder that can indeed cause lens subluxation, typically downward and nasal, similar to Marfan's syndrome. However, it is not as commonly associated with bilateral inferior subluxations as Marfan's and has a distinct metabolic profile.
- **Option D:** **Weill-Marchesani syndrome** is characterized by short stature, brachydactyly, joint stiffness, and characteristic eye anomalies, including ectopia lentis. However, the lens subluxation in Weill-Marchesani syndrome is typically upward.
## **Clinical Pearl / High-Yield Fact**
A key clinical pearl is that Marfan's syndrome patients often have bilateral lens subluxation that is **inferonasal or temporal**, which can lead to significant visual disturbances. Recognizing this association is crucial for diagnosing Marfan's syndrome and managing its ocular complications.
## **Correct Answer:** . Marfan's syndrome