Published date: Jul 18 2019

Journal Title: Journal of Ophthalmic and Vision Research

Issue title: July–September 2019, Volume 14, Issue 3

Pages: 370 – 375

DOI: 10.18502/jovr.v14i3.4793

Mohammadmehdi Hatamidrmehdihatami@gmail.comOphthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Azadeh DoozandehOphthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Mohadeseh FeiziOphthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Purpose: To report a case of advanced childhood glaucoma secondary to congenital ectropion uveae (CEU).

Case Report: The patient was a seven-year-old boy with unilateral glaucoma secondary to CEU and facial asymmetry, mild unilateral ptosis, and proptosis in the left eye. The intraocular pressure (IOP) was 28 mmHg and cup-to-disc ratio was 0.8 in the left eye. After starting glaucoma medication, IOP decreased to 21 mmHg. In view of the uncontrolled IOP with medication and high cup-to-disc ratio and increased axial length of the left eye, mitomycin-C (MMC)-augmented trabeculectomy was planned. Despite sub-tenon MMC injection and bleb needling, the bleb failed after six months, and we had to perform a shunt procedure to control the IOP.

Conclusion: Although CEU is rare, ophthalmologists should be familiar with this syndrome because of the high frequency of glaucoma and its challenging management during childhood.

Keywords: Congenital Ectropion Uveae; Facial Hemihypertrophy; Intraocular Pressure; Mitomycin-C; Ptosis; Secondary Glaucoma

1. Ritch R, Forbes M, Hetherington J, Jr, Harrison R, Podos SM. Congenital ectropion uveae with glaucoma. Ophthalmology 1984;91:326–331.

2. Morales J, Chaudhry IA, Bosley TM. Glaucoma and globe enlargement associated with neurofibromatosis type 1. Ophthalmology 2009;116:1725–1730.

3. Edward DP, Morales J, Bouhenni RA, Patil J, Edward PR, Cummings TJ, et al. Congenital ectropion uvea and
mechanisms of glaucoma in neurofibromatosis type 1: new insights. Ophthalmology 2012;119:1485–1194.

4. Skuta GL, Cantor LB, Cioffi GA. Childhood glaucoma. In: Glaucoma 2013-2014. Section 10. San Francisco, CA:
American Academy of ophthalmology (AAO); 2004: 148.

5. Dowling JL, Jr, Albert DM, Nelson LB, Walton DS. Primary glaucoma associated with iridotrabecular dysgenesis and ectropion uveae. Ophthalmology 1985;92:912–921.

6. Wilson ME. Congenital iris ectropion and a new classification for anterior segment dysgenesis. J Pediatr Ophthalmol Strabismus 1990;27:48–55.

7. Bansal A, Luck J. Primary iris pigment epithelial hyperplasia and glaucoma. Brit J Ophthalmol 2002;86:352–353.

8. Beck AD. Diagnosis and management of pediatric glaucoma. Ophthalmol Clin North Am 2001;14:501–512.

9. Salim S, Shields MB. Iridocorneal Endothelial Syndrome and Glaucoma. EyeNet Magazine 2011;15:47–49.

10. Salim S, Walton DS. Goniotomy and trabeculotomy. In: Yanoff M, Duker JS. Ophthalmology. 3rd ed. New York:
Elsevier; 2008: 1241–1245.