q4 - usor

[ArchivalUser]

A 6-year-old boy is brought to clinic by his aunt at the recommendation of the school nurse, who suspects the child has some form of visual impairment. The child agrees that his vision has been blurry lately. He denies any eye pain or recent trauma. A focused ocular examination reveals that the boy™s previously normal visual acuity has declined by two and three lines in the right and left eyes, respectively. Conjunctivae, sclerae and corneas appear normal, but there is tremulousness of both irises. Under pharmacologic-induced cycloplegia there is tremulousness of both lenses and, under closer inspection, partial bilateral superior-temporal lens dislocation with apparent laxity of the zonular fibers. There is no sign of retinopathy or other ocular abnormality. Based on the ocular pathology described, what additional physical finding(s) would you expect to appear in this patient with a substantially higher probability compared to the general population?

Arachnodactyly
Facial cutaneous angiomata (port wine stain)
Joint hypermobility and skin hyperextensibility
Macroglossia and simian palmar creases
Short stature, neck webbing and widely spaced nipples

[ArchivalUser]

Arachnodactyly

[ArchivalUser]

arachnodactyly
marfan

[ArchivalUser]

Option A (Arachnodactyly) is correct. Marfan syndrome is the most common inherited condition associated with ectopia lentis. 50% to 75% of patients have lens dislocation. The dislocation is usually bilateral and superior-temporal