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section: "Cataracts", "News"


"cataract", "Congenital keratolenticular adhesion", "KLA", "PAX6", "Peters anomaly type 2"

Cataract Surgery in Children with Rare Congenital Disorder Improves Visual Acuity

Cataract Surgery in Children with Rare Congenital Disorder Improves Visual Acuity

shutterstock_85214245Researchers at the Pediatric Ophthalmology, Strabismus, and Adult Motility, Children’s Hospital of Pittsburgh of UPMC Eye Center in Pennsylvania, performed a review on medical reports of cataract surgery in children suffering from rare congenital keratolenticular adhesion (KLA). The study was published in the Journal of American Association for Pediatric Ophthalmology and Strabismus (JAAPOS) and is entitled “Cataract surgery in children with congenital keratolenticular adhesion (Peters anomaly type 2).

Congenital corneal opacity in children’s eyes is a rare event (around 3 in 100,000 newborns) that can lead to blindness. KLA also referred to as Peters anomaly type 2, is even rarer and corresponds to a congenital malformation of the anterior segment of the eye that results in corneal opacity with a posterior defect in Descemet’s membrane (membrane between the cornea and the endothelial layer) and the corneal endothelium. KLA is usually bilateral and exhibits incomplete separation of the cornea from the iris or the lens causing cornea opacity and blurred vision. KLA is often associated with cataracts (clouding of the eye lens). Mutation in the PAX6 gene, which is involved in the development of the anterior segment of the eye, can cause KLA.

The goal of this study was describe the visual and surgical results after cataract surgery in children with KLA. For this purpose, a retrospective review was conducted on clinical data from KLA patients with partial or complete aniridia (absence of the iris, the colored part of the eye) who underwent cataract surgery to remove the clouded lens. Postoperative complications, visual acuity and progression of corneal opacity were evaluated. A total of 4 eyes of 3 patients aged between 3 months to 7 years were analyzed.

Cataract surgery was performed at a mean age of 37 months with a mean follow-up period of 3.3 years. Researchers found that only one of the patients had a mutation in the PAX6 gene with complete aniridia, while the other two patients had partial aniridia. The visual acuity was improved in all four eyes analyzed and no corneal opacity progression was observed. There were also no postoperative complications.

The team concluded that children suffering with KLA, with partial or complete iris defects, and with localized corneal opacity and cataract can obtain better visual results by being submitted to cataract surgery using meticulous lensectomy (removal of the lens) with or without iridectomy (removal of part of the iris).

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