Moran CORE

Open source ophthalmology education for students, residents, fellows, healthcare workers, and clinicians. Produced by the Moran Eye Center in partnership with the Eccles Library

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Curiosity Killed the Cataracts: A Curious Case of Congenital Cataracts

Home / Pediatric Ophthalmology and Strabismus / Childhood Cataracts and Other Pediatric Lens Disorders

Title: Curiosity Killed the Cataracts: A Curious Case of Congenital Cataracts
Author: Celestine Gregerson, MSIV, University of Utah Health, School of Medicine, Salt Lake City, Utah
Date: 8/21/2019
Keywords/Main Subjects: Cataract, congenital
Diagnosis: Congenital cataract
Brief Description: Case of bilateral congenital cataract
Format: Video
Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_27128
Copyright statement: Copyright 2019. Please see terms of use page for more information.


Ectropion Uvea and Secondary Glaucoma

Home / Glaucoma / Childhood Glaucoma

Title: Ectropion Uvea and Secondary Glaucoma
Author: Samaeh Dadashazar, MSIV, Texas Tech University Health Sciences Center, School of Medicine, Lubbock, Texas
Date: 8/21/2019
Keywords/Main Subjects: Uvea, glaucoma, ectropion
Diagnosis: Ectropion uvea and secondary glaucoma

Description: Ectropion uvea is a condition where there is movement of the 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.1,2,3,4,5 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. Surgical intervention will ultimately be needed in every patient in order to control intraocular pressures.5 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 cycloabalation 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 and other complications 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.

References:

  1. 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
  2. Prenshaw, J. & Salim, S. (2013). Ectropion uveae and secondary glaucoma. Ophthalmic Pearls. Retrieved from https://www.aao.org/eyenet/article/ ectropion-uveae-secondary-glaucoma
  3. 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.
  4. 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
  5. 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

Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_27125
Copyright statement: Copyright 2019. Please see terms of use page for more information.


A Case of Axenfeld Anomaly

Home / Glaucoma / Clinical Evaluation

Title: A Case of Axenfeld Anomaly
Author: Brian Bird, MSIV, School of Medicine, University of Nevada, Reno, Nevada
Date: 8/21/2019
Keywords/Main Subjects:Glaucoma, Axenfeld, anomaly, anterior segment
Diagnosis: Axenfeld anomaly
Brief Description: Axenfeld anomaly case description
Format: Video
Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_27122
Copyright statement: Copyright 2019. Please see terms of use page for more information.


Clinical Advances in Fluorescence Lifetime Imaging Opthalmoscopy (FLIO)

Home / Retina and Vitreous / Diagnostic Approach to Retinal Disease

Title: Clinical Advances in Fluorescence Lifetime Imaging Opthalmoscopy (FLIO)
Author: Lydia Sauer, MD
Date: 10/9/2019
Keywords/Main Subjects: Fluorescence Lifetime Imaging Opthalmoscopy; FLIO
Brief Description: Clinical Advances in Fluorescence Lifetime Imaging Opthalmoscopy (FLIO)
Format: Video
Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_27119
Copyright statement: Copyright 2019. Please see terms of use page for more information.


Case of Orbital Apex Syndrome and Cavernous Sinus Syndrome Secondary to Mucormycosis Infection

Home / Neuro-Ophthalmology / Grand Rounds Presentations and Cases

Title: Case of Orbital Apex Syndrome and Cavernous Sinus Syndrome Secondary to Mucormycosis Infection
Author: Mike Murri, MD
Date: 10/9/2019
Brief Description: Unilateral blurry vision and headache; Mucormycosis
Format: Video
Series: Moran Eye Center Grand Rounds or Moran Eye Center Resident Lectures
Identifier: Moran_CORE_27116
Copyright statement: Copyright 2019. Please see terms of use page for more information.


Burden of Preoperative History/Physical On Veterans Undergoing Surgery

Home Lens and Cataract / Preparing for Cataract Surgery in Special Situations

Title: Burden of Preoperative History/Physical On Veterans Undergoing Surgery
Author: Ariana Levin, MD
Date: 10/9/2019
Brief Description: Rethinking Preoperative H&P Requirement Prior to Cataract Surgery
Format: Video
Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_27113
Copyright statement: Copyright 2019. Please see terms of use page for more information.


Reframing Expectations of Life Potential of Older Adults with Vision Impairment

Home / Ethics

Title: Reframing Expectations of Life Potential of Older Adults with Vision Impairment
Author: Corinna Trujillo Tanner, PhD, MSN and Bob Christiansen, MD, FACS
Date: 9/25/2019
Keywords/Main Subjects: Vision loss, vision impairment, rehabilitation, low vision
Diagnosis: Vision loss
Brief Description: Discussion of the impact of vision loss and strategies to help patients maximize quality of life
Format: Video
Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_######
Copyright statement: Copyright 2019. Please see terms of use page for more information.


Uveal and Capsular Biocompatibility of a New IOL

Home / Lens and Cataract / Surgery for Cataract

Title: Uveal and Capsular Biocompatibility of a New IOL
Author: Sean Kennedy, MD
Date: 9/04/2019
Keywords/Main Subjects: Intraocular lens, posterior capsule opacification, capsule, lens
Diagnosis: Cataract
Brief Description: Research fellow discussion on new advances in IOL technology
Format: Video
Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_27107
Copyright statement: Copyright 2019. Please see terms of use page for more information.


Posterior Capsule Opacification Prevention

Home / Lens and Cataract / Surgery for Cataract

Title: Posterior Capsule Opacification Prevention
Author: Sneha Bontu, MD
Date: 9/04/2019
Keywords/Main Subjects: Intraocular lens, posterior capsule opacification, capsule, lens
Diagnosis: Cataract
Brief Description: Research fellow discussion on new advances in IOL technology
Format: Video
Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_27104
Copyright statement: Copyright 2019. Please see terms of use page for more information.


Corneal Collagen Crosslinking

Home / External Disease and Cornea / Clinical Approach to Ocular Surface Disorders

Title: Corneal Collagen Crosslinking
Author: Mark Mifflin, MD
Date: 8/28/2019
Keywords/Main Subjects: Crosslinking, keratitis, keratoconus
Diagnosis: Keratitis, keratoconus
Brief Description: Cornea Grand Rounds
Format: Video
Series: Moran Eye Center Grand Rounds
Identifier: Moran_CORE_27100
Copyright statement: Copyright 2019. Please see terms of use page for more information.