Posters

Insight into pathogenesis and treatment of syndrome of ectopia lentis, spontaneous filtering blebs and craniofacial dysmorphism

Poster Details

First Author: B.Abiad LEBANON

Co Author(s):    A. Mansour   A. Rafic   A. Maamoun   Y. Mohamad     

Abstract Details

Purpose:

Report the UBM and surgical findings in a patient with the syndrome of ectopia lentis, spontaneous filtering blebs, and craniofacial dysmorphism previously described by Shawaf et al and Haddad et al.

Setting:

Referral tertiary university medical center

Methods:

Case report using 40 MHz UBM wide-field anterior segment scan and anterior segment OCT

Results:

16-year-old girl presented with visual loss to 6/60 (20/200) bilaterally. She had a central corneal opacification, retrocorneal thickening, flat chamber, poorly dilating pupils, central lens bilaterally with spontaneous blebs, and normal intraocular pressure. Apposition of the iris to the cornea with angle closure, distortion of the iris and angle structure, delineation of the bleb tract and rarefaction of the zonules were imaged by UBM. Under general anesthesia, deepening the anterior chamber with sodium hyaluronate 3% led to spontaneous dislocation of the lens into the anterior chamber facilitating its aspiration. Deepening and normalisation of the angle was found after lens removal.

Conclusions:

Early surgical removal of the lens is necessary in this syndrome to avoid corneal and trabecular meshwork damage in chronic apposition of the iris to the cornea. UBM can help in the delineation of the bleb tract and reversal of angle closure after surgery. FINANCIAL DISCLOUSRE: NONE