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This 7-year-old boy who was said to have decreased vision in both eyes developed severe pain in the left eye, prompting the family to bring him to an eye doctor. The family claims that the child is otherwise healthy. The visual acuity is 20/65 in the right eye and 20/300 in the left eye with very high myopic correction. Pictures of the anterior segment of the left eye are shown. The lens in the right eye is also observed to be subluxated infero-nasally.  This boy is slightly taller than average, has blond hair and is said to have a behavior problem in school.

1.	What is the most likely diagnosis:
	a. Marfan syndrome b. Weill-Marchesani syndrome c. homocystinuria d.  aniridia
2.	Which test would be most helpful in confirming the diagnosis:
	a. cardiac ultrasound b. urine test for sodium-nitroprusside c. roentgenography of the hands d.  fundus examination
3.	What is the next best step for this patient:
	a. observation b. peripheral laser iridotomy c. lensectomy d.  trabeculectomy

For answers to the above, click here on or after March 1, 2005.