Primary Objective: To determine the prevalence of endo- and extranasal deformities, problems with nasal functioning and satisfaction with nasal appearance in patients with TCS.Secondary Objective: To adjust and improve the treatment of patients with…
ID
Source
Brief title
Condition
- Musculoskeletal and connective tissue disorders congenital
- Congenital respiratory tract disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Study parameters/endpoints
Main study parameter/endpoint
Main study parameters/endpoints will be a detailed description of all endo- and
extranasal deformities (description of affected anatomical structures), nasal
functioning and satisfaction with nasal appearance (as addressed in NAFEQ
score).All are described in more detail in the -following- study procedures
Secondary outcome
Secondary endpoint is a change in the current treatment of TCS as will be
described in (as part of ) a treatment protocol.
Background summary
Treacher-Collins syndrome (TCS) is clinically associated with endonasal and
extranasal deformities and problems with nasal functioning. Patient reports
learn us that these functional problems of the nose are considered the most
significant impairment. However, the anatomical explanation for these findings
is lacking.
Study objective
Primary Objective: To determine the prevalence of endo- and extranasal
deformities, problems with nasal functioning and satisfaction with nasal
appearance in patients with TCS.
Secondary Objective: To adjust and improve the treatment of patients with TCS
according to our outcomes and summarize that together with the results of
MEC-2008-402 in a treatment protocol.
Study design
We will conduct a cross-sectional cohort study with a single cohort: a TCS
group. Patients in the TCS group are included if they are diagnosed with TCS
and have been treated at the multidisciplinary craniofacial team of the Erasmus
MC, University Medical Center since 1974 (i.e. a 37 year period). All adult
patients are eligible for inclusion.
Study burden and risks
History: A history anamnesis will be obtained concerning medical nasal issues
(five minutes).
Questionnaire: The NAFEQ score (Nasal Appearance and Function Evaluation
Questionnaire) will be filled out by the patients (five minutes).1
Physical examination: Anterior rhinoscopy and the Cottle test will be performed
(approximately five minutes).
Additional examination: Endonasal deformities will be determined with a
standardized flexible laryngoscope (approximately 5-10 minutes). No adverse
events are expected. Standardized digital photography will determine extranasal
deformities (10 minutes).
Outcomes of our (planned) study can determine the cause of reported endonasal
complaints and anatomic origin of extranasal deformities. Outcomes of this
study could lead to an additional treatment focus and thus to an improved
treatment of TCS based on a sufficient level of evidence. Multidisciplinary
treatment will be further optimised. This study will give some burden to the
patient with regard to the aspects that are not part of the standard clinical
practice. No part of this study is associated with (serious) adverse events and
the risks are negligible. Patients will receive a result of the endoscopy and
this will be saved in the medical chart and if necessary patients will be
treated.
dr. Molewaterplein 50
Rotterdam 3015 GE
NL
dr. Molewaterplein 50
Rotterdam 3015 GE
NL
Listed location countries
Age
Inclusion criteria
diagnosis Treacher Collins syndrome
informed consent
ability to speak Dutch
18 years or above
Exclusion criteria
no ability to speak Dutch
age < 18 years
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL39809.078.12 |