Rare Association Of Bilateral Microcornea, Iridochorioretinal Coloboma, Lentis Ectopia, And Cataract In A Pediatric Case

To report a rare association of bilateral microcornea,iridochorioretinal coloboma and lentis ectopia complicated with cataract in a twelve-year-old boy managed with In-the-Bag intra ocular lens (IOL) implantation technique associated with inferior pupilloplasty.

Ophtalmology Department, Habib Thameur University Hospital

A case study of a twelve-year-old boy presenting with bilateral ectopia was conducted, detailing medical history, clinical examination and surgical management.

It is a case of a 12-year-old boy born of a consanguineous marriage. General and cardiovascular screening were normal. Best visual acuity was 5/50 on the right eye and 1/50 on the left eye. Slit-lamp examination showed a bilateral microcornea, (maximal corneal diameter : 8mm), iris coloboma, lentis ectopia and subcapsular cataract. Fundus examination showed bilateral chorioretinal coloboma in both eyes. The Surgery involved phacoaspiration, capsular bag fixation to sclera with capsular tension segment, capsular bag support with capsular tension ring and intraocular lens implantation, associated to pupilloplasty. Postoperative outcomes showed improved visual acuity to 20/100 on the right eye and to 5/50 on the left eye at one month follow-up

Ectopia lentis may be a feature of numerous systemic and ocular disorders. To the best of our knowledge, association of microcornea,iridochorioretinal coloboma,lentis ectopia and cataract has not been reported. Timely diagnosis and surgical management of this condition remains a real challenge preventing amblyopia and optimizing quality of life for children.