Neonatal-onset Congenital Ectropion Uveae: a distinct phenot
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Highlights
• Neonatal-onset Congenital Ectropion Uveae (CEU) is a distinct phenotype of newborn glaucoma.

• It has a characteristically bilateral presentation with CEU, varying degrees of iris hypoplasia and severe glaucoma

• The prognosis is less favorable than seen in neonatal onset Primary congenital glaucoma

• This entity appears to be phenotypically different from the unilateral CEU described in literature, usually reported in older children.

A study was conducted to describe “neonatal-onset congenital ectropion uveae (N-CEU)” as a distinct clinical entity of newborn glaucoma (NG), and study its significance towards the severity and outcome of NG.

Consecutive patients of NG who presented between 1st July 2016 to 30th September 2017, with a minimum post-operative follow up of 1 year. Babies with any ocular anomaly apart from CEU were excluded. N-CEU were compared with those with neonatal-onset primary congenital glaucoma(N-PCG). All babies underwent goniotomy or trabeculotomy with trabeculectomy depending upon corneal clarity.

Clinical features at presentation and outcome after one year of surgery (defined as good or satisfactory if intraocular pressure (IOP) was equal or less than 16.0 mm Hg under anesthesia without or with topical medications, respectively, and poor if the baby required additional surgery).

Twenty eyes of 10 patients with N-CEU were compared with 16 eyes of 9 patients with N-PCG. N-CEU babies had significantly worse corneal clarity, and poorer outcome compared to N-PCG. 7 of 16(43.7%) eyes with N-PCG had a cornea clear enough at presentation for a goniotomy, compared to only 2 of the 20(10%) eyes with N-CEU. 13 of 16(81.2%) N-PCG eyes had a good or satisfactory outcome, compared to 6 of 20 (30%) N-CEU eyes.

Neonatal-onset CEU appears to be distinct from the unilateral CEU in older patients described in literature, and may be considered a poorer prognosis phenotype of neonatal-onset glaucoma.

Source: https://www.sciencedirect.com/science/article/abs/pii/S0002939420305614?dgcid=rss_sd_all
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