Riboflavin Corneal Crosslinking for Brittle Cornea Syndrome and Ehlers-Danlos Syndrome Type VI

NCT01307527 | Unknown

• 1 other identifier: BTL-CXL-HMO-CTIL
• Study Type: interventional
• Target: 1
• Locations: 1 country
• Sites: 1

Brief Summary

Brittle Cornea Syndrome and Ehlers-Danlos Syndrome (EDS) type VI are rare collagen-connective tissue disorders that predispose affected individuals to the development of perforated corneas from the mildest of eye trauma or even spontaneously. Clinical studies evaluating riboflavin-corneal crosslinking have found that it dramatically increases corneal rigidity. Given the success and safety of riboflavin crosslinking, the investigators believe that it can increase the corneal stability in patients affected these disseases, preventing perforation. It is furthermore possible, that riboflavin crosslinking will allow corneal transplants to successfully be performed on blind eyes that have already perforated and opacified. The purpose of the study is to determine whether corneal crosslinking can be safely performed on individuals with Brittle Cornea Syndrome or Ehlers-Danlos Syndrome type VI.

Conditions

Brittle Cornea Syndrome; Ehlers-Danlos Syndrome Type 6

Outcome Measures

Primary Outcomes (1)

• Adverse Events from Cornea Riboflavin Crosslinking in Brittle Cornea Syndrome or Ehlers Danlos Type VI

  3 Months

Interventions

Riboflavin DRUG

0.1%, applied every 5 minutes for 60 minutes

Kera-X DEVICE

3 mW/cm2, to the central 7.5 mm of the cornea, for 30 minutes

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Adult Patient with:
• Genetic diagnosis of either Brittle Cornea Syndrome or EDS-VI, and
• Either:
• Personal History of either spontaneous corneal perforation or corneal perforation due to minor ocular mechanical trauma or
• Immediate family member with history of either spontaneous corneal perforation or corneal perforation due to minor ocular mechanical trauma

You may not qualify if:

• Any patient that is deemed to be unable to fully cooperate during the crosslinking procedure

Sponsors & Collaborators

• Hadassah Medical Organization: lead

Study Sites (1)

Hadassah Medical Organization

Jerusalem, Israel

Location

MeSH Terms

Conditions

Brittle cornea syndrome 1; Ehlers-Danlos syndrome type 6

Interventions

Riboflavin

Intervention Hierarchy (Ancestors)

Flavins; Pteridines; Heterocyclic Compounds, 2-Ring; Heterocyclic Compounds, Fused-Ring; Heterocyclic Compounds; Heterocyclic Compounds, 3-Ring; Coenzymes; Enzymes and Coenzymes; Pigments, Biological; Biological Factors

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: PREVENTION
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER

Study Record Dates

• First Submitted: March 1, 2011
• First Posted: March 3, 2011
• Last Updated: March 3, 2011

Record last verified: 2011-02

Locations

IL (1)