Neonatal-Onset Congenital Ectropion Uveae: A Distinct Phenotype of Newborn Glaucoma

Characteristics and Outcomes of Glaucoma Associated With Congenital Ectropion Uvea

“…10 The fourth originated in India and compared neonatal onset-glaucoma associated with congenital ectropion uvea with primary congenital glaucoma. 13 The current study is the first in over 3 decades to report vision and glaucoma surgical outcomes in both bilateral and unilateral congenital ectropion uvea.…”

“…Although the majority of congenital ectropion uvea cases published in the literature are unilateral, bilateral presentations have been documented. 5 , 13 Kaushik and associates 13 reported 10 children with bilateral congenital ectropion uvea and neonatal onset glaucoma. These authors showed that babies with congenital ectropion uvea had worse corneal edema at presentation and a poorer response to angle surgery compared with infants with primary congenital glaucoma, consistent with our findings.…”

“…These authors showed that babies with congenital ectropion uvea had worse corneal edema at presentation and a poorer response to angle surgery compared with infants with primary congenital glaucoma, consistent with our findings. At the final follow-up, the majority of patients (70%) in Kaushik and associates 13 required at least 3 glaucoma medications or additional glaucoma surgery. However, that study did not describe visual outcomes, analyze survival curves for angle surgery, or discuss the use of nonangle surgeries.…”

“…10 , 12 Furthermore, although most cases are unilateral, bilateral congenital ectropion uvea has been reported and is associated with neonatal-onset glaucoma that has a worse prognosis than primary congenital glaucoma. 13 To the best of our knowledge, there are 4 previous case series (with more than 3 patients) in the English literature that describe a total of 32 patients with congenital ectropion uvea. 1 , 2 , 10 , 13 We add to the literature, as we present a series of 8 patients with congenital ectropion uvea including both unilateral and bilateral involvement.…”

Unilateral advanced glaucoma in isolated congenital ectropion uveae with ipsilateral ptosis: A pictorial description of five children

“…Children were characterized as suffering from CEU if they fulfilled the following criteria: presence of iris pigment epithelium on the anterior surface of iris with no other characteristic suggestive of any other anterior segment dysgenesis (ASD) syndrome such as Axenfeld Rieger’s anomaly, Peter’s anomaly, or partial aniridia. Neonatal-onset congenital ectropion uveae is a recently described phenotype of newborn glaucoma, which has a bilateral presentation with buphthalmos and cloudy cornea [ 8 ]. We did not include this entity in the present report.…”