Congenital Ectropion Uveae
Title: Congenital Ectropion Uveae
Author (s): Samareh Dadashazar, MS IV, Texas Tech University Health Sciences Center School of Medicine
Photographer: James Gilman, CRA, FOPS
Keywords/Main Subjects: Congenital Ectropion Uveae; Secondary Glaucoma
Diagnosis: Congenital Ectropion Uveae
Author: Samareh Dadashazar, MS IV, Texas Tech University Health Sciences Center School of Medicine
Ectropion uveae is a condition where there is migration of the posterior iris pigmented epithelium from the pupillary ruff onto the anterior surface of the iris. This process can either be congenital or acquired. The congenital form is noted to be rare and non-progressive.1 An exact mechanism is currently unknown, but many theories have postulated that a late developmental arrest of posterior neural crest cells plays a role.1,3 Others suggest that there may be a type of primordial endothelium in the anterior chamber that does not fully regress and subsequently induces a hyperplasia of the pigmented epithelium leading to the anterior movement.2,4 In contrast, the acquired form occurs when a membrane develops in the anterior segment secondary to any inflammatory, neoplastic, or ischemic process.1 This membrane eventually contracts, pulling the posterior pigmented epithelium of the iris anteriorly, thus forming the ectropion uvea. As such, the acquired type is considered progressive in nature. The most common causes of acquired ectropion uvea are neovascular glaucoma and neovascularization of the iris.2,3,4
Congenital ectropion uvea is usually a unilateral finding, but bilateral cases have been reported in the literature.4 The ectropion uvea can be an isolated finding, seen alongside ptosis with good levator function, or as part of a systemic disorder such as neurofibromatosis type 1 (NF1), Rieger’s anomaly, and Prader-Willi syndrome.1–5 An extremely important association to be aware of is the one between congenital ectropion uvea and secondary glaucoma. The literature reports up to 80-90% of patients with congenital ectropion uvea will eventually develop glaucoma at some point in their life.2,5 There are approximately 50 established cases of glaucoma secondary to congenital ectropion uvea in the literature.5 Most patients are affected in childhood or early adolescence, but the reported ages have ranged from 7 months to 42 years.2,4,5 The variation in age of presentation likely correlates to the degree of neural crest cell arrest and trabecular meshwork malformation.2,4 While there are a couple of exceptions reported in the literature, the trend seems to be that patients with both congenital ectropion uvea and NF1 develop an angle-closure glaucoma while those without NF1 develop an open-angle glaucoma.1,3,5 In most cases of congenital ectropion uvea without NF1, patients have anterior insertion of the iris, typically at the level of the trabecular meshwork but sometimes as anterior as Schwalbe’s line.2,5 With such anterior insertion, it is clear to see how these open but dysplastic angles predispose these patient to develop glaucoma over time.
Currently, there is not a consensus on what the best treatment is for patients with glaucoma secondary to congenital ectropion uvea. In contrast to primary congenital glaucoma, many articles in the literature have reported that goniotomies and trabeculotomies are not effective for long-term pressure control and that patients who received these interventions subsequently needed a trabeculectomy or cycloablation in order to bring their pressures back down.2,5 The longest reported case of intraocular pressure control due to goniotomy alone is only 2.5 years.5 Glaucoma drainage devices also seem to fail at controlling pressures in these patients.5 Currently, filtering procedures with antifibrotics, such as a trabeculectomy with mitomycin c, seem to be the most successful surgical intervention for managing long-term intraocular pressures in patients with congenital ectropion uvea.5
In conclusion, congenital ectropion uvea is itself a benign condition, however, it’s strong association with the development of glaucoma is important for physicians to be aware of in order to prevent future vision loss in these patients. If a patient is diagnosed with congenital ectropion uvea, regular tonometry evaluations are needed for their entire lifetime in order to monitor intraocular pressures. More extensive long-term follow-up is needed to establish which surgical intervention is the most successful in controlling intraocular pressures in patients with congenital ectropion uvea.
- Harasymowycz, P. J., Papamatheakis, D. G., Eagle Jr, R. C. Wilson, R. P. (2006). Congenital ectropion uveae and glaucoma. Arch Ophthalmol, 124(2), 271-273. doi:10.1001/archopht.124.2.271
- Prenshaw, J. & Salim, S. (2013). Ectropion uveae and secondary glaucoma. Ophthalmic Pearls. Retrieved from https://www.aao.org/eyenet/article/ ectropion-uveae-secondary-glaucoma
- Seymenoglu, G. & Baser, E. (2011). Congenital iris ectropion associated with juvenile glaucoma. International Ophthalmology, 31(1), 33-38. doi: 10.1007/s10792-010-9388-6.
- Shifa, J. Z., Nkomazana, O., Bekele, N. A. , & Kassa, M. W. (2016). A young Botswana patient with congenital iris ectropion uvea. The Pan African Medical Journal, 25(42). doi: 10.11604/pamj.2016.25.42.10593
- Wang, G. M., Thuente, D., & Bohnsack, B. L. (2018). Angle closure glaucoma in congenital ectropion uvea. American Journal of Ophthalmology Case Reports, 10, 215-220. doi: 10.1016/j.ajoc.2018.03.009