This site uses cookies. By continuing to browse the site you are agreeing to our use of cookies.  Read our Cookies Policy.
Close
Eye News
  • Features
    • Close
    • Features
    • Allied Professions
    • Humanitarian
    • Interviews
    • AI & Oculomics
    • Ophthalmology
    • Optometry
    • Podcast videos
    • Supplements
  • Education
    • Close
    • Education
    • Learning Curve
    • Quiz
    • Top Tips
    • Trainees
    • Medico-Legal
    • The Truth Behind The Headlines
    • Case Reports
    • Pete's Bogus Journey
  • Reviews
    • Close
    • Reviews
    • Book Reviews
    • Journal Reviews
    • What's trending?
    • Tech Reviews
    • My Top Five
    • The Culture Section
  • Events
  • News
  • Product Guide
  • Industry News
  • Contact us
    • Close
    • Contact us
    • Write for Eye News
  • Home
  • Reviews
  • Journal Reviews
  • Ophthalmic presentations and outcomes in Erdheim-Chester disease

Ophthalmic presentations and outcomes in Erdheim-Chester disease
Reviewed by Hetvi Bhatt

4 December 2023 | Hetvi Bhatt | EYE - Oculoplastic, EYE - Orbit
Share This

This is a comprehensive article describing clinical manifestations and pathogenesis of Erdheim-Chester disease (ECD) followed by a literature review of the available treatments. The authors look to compare historical treatment (HT) with vemurafenib, a BRAF inhibitor. ECD is a non-Langerhans histiocytosis that can have diffuse lipogranulomatous infiltration of the orbit. The overall five-year survival is around 68%, but recent advances in targeted therapy are thought to have increased this. There is a slight male predominance, and the average reported age is 49.6 years. The authors found 57 eyes in the literature that had HT and 12 eyes that had vemurafenib treatment. Although the lower patient numbers are understandable given the rarity of the disease, it does make comparison difficult, especially given the range of treatments in the HT group which include steroids, radiotherapy, interferon-α, caldribine and cyclophosphamide. Their analysis shows that the vemurafenib group have a better visual outcome and reduced mortality rate (although not statistically significant). Further studies with larger cohorts would help to confirm this.

Erdheim-Chester disease and vemurafenib: a review of ophthalmic presentations and clinic outcomes.
Park JK, Huang LC, Kossler A.
ORBIT
2023;42(3):233-44.
Share This
CONTRIBUTOR
Hetvi Bhatt

Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK.

View Full Profile
Specialty
  • EYE - Cataract
  • EYE - Cornea
  • EYE - General
  • EYE - Glaucoma
  • EYE - Neuro-ophthalmology
  • EYE - Oculoplastic
  • EYE - Oncology
  • EYE - Orbit
  • EYE - Paediatrics
  • EYE - Pathology
  • EYE - Refractive
  • EYE - Strabismus
  • EYE - Vitreo-Retinal
Archive
  • 2025
  • 2024
  • 2023
  • 2022
  • 2021
  • 2020
  • 2019
  • 2018
  • 2017
  • 2016
  • 2015
  • 2014
  • 2013

Top Of Page

9 Gayfield Square, 
Edinburgh EH1 3NT, UK.

Call: +44 (0)131 557 4184
www.pinpoint-scotland.com

WEBSITE DETAILS
  • Cookie Policy
  • Data Protection Notice
  • Privacy Policy
  • Terms and Conditions
ABOUT US
  • Who we are
  • Register
  • Contact us
  • Contributors
  • Company Awards
DIGITAL ISSUES/GUIDELINES
  • Digital issues - Library
  • Supplements - Library
  • Guidelines
Accreditations
IPSO_FLAG_TEAL 2025.png cpdcertified.png

Pinpoint Scotland Ltd (Registered in Scotland No. SC068684) | © 2025 - Website by Gecko Agency