CASE REPORT Secondary Glaucoma Due to Congenital Ectropion Uveae Seher Koksaldi 1 , Gul Arikan 2 , Uzeyir Gunenc 3 1- Resident, Dokuz Eylul University Faculty of Medicine, Department of Ophthalmology, Izmir, Türkiye 2- Associate Professor, Dokuz Eylul University Faculty of Medicine, Department of Ophthalmology, Izmir, Türkiye 3- Professor, Dokuz Eylul University Faculty of Medicine, Department of Ophthalmology, Izmir, Türkiye Received: 28.02.2022 Accepted: 17.09.2022 J Glau-Cat 2022; 17: 200-203 DOİ: 10.37844/glau.cat.2022.17.33 Correspondence Address: Gül Arıkan Dokuz Eylul University Faculty of Medicine, Department of Ophthalmology, Izmir, Türkiye Phone: +90 531 791 9687 E-mail: gulozdenarikan@gmail.com ABSTRACT Here, we present a case with secondary glaucoma due to congenital ectropion uveae. A 6-year-old girl was referred to our clinic with the diagnosis of congenital ectropion uveae and secondary glaucoma in her right eye. In the ophthalmic examination, visual acuity as measured by Snellen charts was 0.4 (with correction of -2.50 D) in the right eye and 1.0 (uncorrected) in the left eye. In the slit-lamp examination, congenital ectropion uveae was present in the right eye and the left eye was normal. Intraocular pressure was 35 mmHg (with maximum medical treatment) in the right eye and 19 mmHg in the left eye. In addition, there was also mild ptosis in the right eye. There was no systemic abnormality in the patient. The patient was treated with Ahmed glaucoma valve implantation in the right eye. Glaucoma may develop due to angle dysgenesis in patients with congenital ectropion uveae. They should be examined in a periodical manner for the early detection of glaucoma. Key words: Ectropion uveae, Glaucoma, Ahmed glaucoma valve. 200 INTRODUCTION Ectropion uveae (EU) is a clinical presentation where iris pigment epithelium is seen on anterior surface of iris. There are two subtypes as congenital and acquired EU. The acquired form generally occurs as a result of membranous tractions secondary to conditions such as ischemia and inflammation. 1 Congenital ectropion uveae (CEU) is a neural crest-originated disorder which characterized by non-progressive proliferation of iris pigment epithelium on anterior surface of iris. It was first described by Wicherkiewicz in 1891 and by Spiro in 1896. 2, 3 Glaucoma development is associated to iridotrabecular dysgenesis. In patients with CEU, glaucoma can be present upon birth; however, it may also develop at infantile period or older ages. In cases with CEU, glaucoma is generally refractory, requiring aggressive treatment. 3 Here, we aimed to present a pediatric case with glaucoma secondary to CEU and treated with Ahmed glaucoma valve (AGV) implantation surgery. CASE REPORT A 6-year-old girl was referred to our clinic as CEU and glaucoma in the right eye from another facility where she presented with redness in her right eye. In initial ophthalmological examination in our clinic, visual acuity as measured by Snellen charts was recorded as 0.4 (with correction of -2.50 D) in the right eye and as 1.0 (uncorrected) in the left eye. In the slit-lamp examination, no pathological finding other than CEU was detected in the right eye (Figure 1) and the left eye was normal. Intraocular pressure (IOP) as measured by Goldmann applanation tonometry was 35 mmHg (under fixed combination of topical brinzolamide plus timolol with travoprost therapy) in the right eye and 19 mmHg in the left eye. In fundus examination, cup: disc ratio was 0.9 (Figure 2). On optical coherence tomography, there was thinning in peripapillary retinal nerve fiber layer in the right eye (Figure 3). In addition, there was congenital ptosis not interfering with visual axis in the right eye (Figure 4); however, levator palpebrae superioris function was normal. In her history, there was no systemic disease or medication. In systemic examination by pediatrics department, no abnormal finding was detected. The patient was diagnosed as secondary glaucoma due to CEU and AGV implantation was performed in the right eye (Figure 5). At postoperative period, antiglaucomatous agents were withdrawn and topical prednisolone acetate