Lens dislocation is a feature of which disorder?

Lens dislocation is a feature of which disorder?

• A. Homocystinuria
• B. Marfan syndrome
• C. Ehlers-Danlos syndrome
• D. Osteogenesis imperfecta

Answer: (B)

Lens dislocation, or ectopia lentis, is a classic ocular finding in Marfan syndrome. This occurs because a mutation in fibrillin-1 weakens the zonular fibers that hold the lens in place, allowing it to sublux or displace. In Marfan, the lens tends to shift upward and outward (superotemporal direction) and can become ectopic with time. This pattern helps distinguish it from lens dislocation seen in homocystinuria, where the subluxation is typically downward and inward and is accompanied by other systemic issues like thromboembolism and intellectual disability. Ehlers-Danlos syndrome and osteogenesis imperfecta are not defined by lens dislocation. So, the disorder most characteristically associated with lens dislocation is Marfan syndrome.

Lens dislocation, or ectopia lentis, is a classic ocular finding in Marfan syndrome. This occurs because a mutation in fibrillin-1 weakens the zonular fibers that hold the lens in place, allowing it to sublux or displace. In Marfan, the lens tends to shift upward and outward (superotemporal direction) and can become ectopic with time. This pattern helps distinguish it from lens dislocation seen in homocystinuria, where the subluxation is typically downward and inward and is accompanied by other systemic issues like thromboembolism and intellectual disability. Ehlers-Danlos syndrome and osteogenesis imperfecta are not defined by lens dislocation. So, the disorder most characteristically associated with lens dislocation is Marfan syndrome.