Posterior chamber intraocular lens dislocation into the anterior chamber in a patient with Leber's congenital amaurosis due to repeated eye-poking

Purpose: To study and report a dislocated single piece posterior chamber (PC) intraocular lens (IOL) into the anterior chamber (AC) as a complication resulted from repeated eye poking in a patient with Leber’s congenital amurosis (LCA).

Methods: Clinical eye examination, genotyping, and surgical management was done for a 4 years old female with LCA.

Results: A 4-year-old full term female with a past ocular history of LCA presented to the emergency room with a dislocated single piece PC IOL into the AC in her left eye. There was no history of trauma or any other surgical interventions. Her visual acuity was light perception in different quadrants before and after the lens dislocation. She is status post lens aspiration and PC IOL implantation 2 years ago in another eye care facility. The patient was originally diagnosed clinically as a case of LCA and since then underwent genotyping that showed a homozygotic variant mutation of the gene GUCY2D which is inherited in an autosomal recessive manner. This mutation is the first gene described for LCA and constitute the most common variant of the disease accounting for more than 20% of the cases. A specific behavior known as Franceschetti oculo-digital sign is a characteristic feature of LCA; this sign consists of repeated pressing, poking and rubbing the eyes with knuckles and fingers to stimulate the photoreceptors. Researchers suspect that this behavior contributes to the deep-set eyes and keratoconus usually found in these cases. Removal of the dislocated IOL was performed and kept the patient with aphakic glasses.

Author(s): Faris Alarfaj                                                                                                                                                                

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