This 56YO developmentally delayed male presented with seeing a “black spot” in his right eye. He was monocular with a phthisical left eye from unclear etiology. Vision in his right eye was decreased to 20/400 from his baseline of 20/70 at his prior visit.
He was largely nonverbal and difficult to examine due to extreme photosensitivity. Optos color RG imaging shows an inferiorly dislocated crystalline lens.
Urgent pars plana vitrectomy and lensectomy were performed. After discussion with the patient’s guardian, the decision was made to leave the patient aphakic rather than to place a secondary scleral-fixated or scleral-sutured lens due to a history of frequent eye rubbing. The patient was fitted with aphakic glasses, which restored his vision to baseline.
Learning Points:
Dislocation or subluxation of the crystalline lens can be caused by trauma, ocular conditions such as ectopia lentis et pupillae, pseudoexfoliation, aniridia, high myopia, or hereditary disorders such as Marfan syndrome, homocystinuria, Weill-Marchesani syndrome, Ehlers-Danlos syndrome, or sulfite oxidase deficiency. Our patient had a known cardiofaciocutaneous (CFC) syndrome secondary to BRAF mutation, but this has not been associated with ectopia lentis.
