congenital facial deformity syndromic craniosynostosis
Conditions
Interventions
None listed
Sponsors
Mondziekten, Kaak- en Aangezichtschirurgie
Eligibility
Age
2 Years to 99 Years
Inclusion criteria
Inclusion criteria: Patients suffering from 1. syndromic craniosynostosis, 2. midline facial clefts, 3. oblique facial clefts (uni- or bilateral), and 4. frontofacial (meningo-) encephaloceles.
Exclusion criteria
Exclusion criteria: Patients are excluded if surgical correction was performed elsewhere or if data are incomplete, if patients in the Control group had peri-orbital surgical correction. Patients and parents do not speak the native Dutch language or are mentally retarded, will be excluded from the questionnaires.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Peri-orbital functions - Visual acuity: the visual acuity (logMAR-chart) and visual field is assessed; the performed tests are age-dependent. - Ocular motility: An orthoptist will assess movement with specific attention for A and V patterns in the nine positions of gaze in a standardized way. Causes of strabismus will be evaluated. - Closure and position of eyelids: the presence of lagophtalmus, exorbitism, enophtalmus, ectropion, entropion, downward slanting, or scleral show, dystopia of the medial and lateral canthi, and the presence of epicanthus will be assessed. - Functioning of the lacrimal system/adnexen. Peri-orbital esthetic appearance - Overall appearance: This will be assessed with the Versnel scoring list based on standardized photographs. - Hypertelorism: The interdacryon distance will be analyzed on CT or CBCT. In addition intra-operative measurements of the interdacryon distance in the patient will be performed before and after correction by the surgeon with a ruler. At the level of soft tissue, hypertelorism will be studied on the (3D-) photographs. Intercanthal and interocular distances, and horizontal axis of the eye will be measured. - Vertical orbital dystopia: Vertical position of the orbits/ orbital floor will be analysed on CT/CBCT (bone) and (3D-)photographs (soft tissue). - Midface hypoplasia: The advancement of the midface due to surgery or growth, will be studied on cephalometry calculating SNA. On lateral photographs facial divergence will be analyzed drawing a line from the glabella to the upperlip, to the vertex of the soft-tissue chin. - Additional peri-orbital deformities will be assessed on standardized photographs according to the Versnel score. Movements of the soft tissue and bone structures will be measured on pre- and postoperative CT/CBCT in millimetres. Relapse and growth. Relapse will be evaluated in the operated patients. Growth will be analyzed using X-ray and photographs at 0, 4, 9 and 18 ye | — |
Secondary
| Measure | Time frame |
|---|---|
| Postoperative satisfaction of the surgeons will be analysed using a panel. Photographs pre-operatively, 1 year postoperatively and at the age of 18-21 years will be analysed. Surgeons will score surgical outcomes using a VAS. With the VAS hypertelorism, vertical orbital dystopia and midface hypoplasia, the overall appearance, the frontal, orbital, nose and midface region will be judged. Postoperative satisfaction of the patients/parents will be analyzed. Using a patient related expectation measurements (PREM) pre-operative expectations are investigated. Using a patient related outcome measurements (PROM) post-operative satisfaction is investigated. Children will be asked questions on teasing. When the patient is younger than 12 years old, or mentally retarded, parents will fill in the questionnaire. When the patient is capable of completing the questionnaires we request the patient to fill in the questionnaires himself/herself. For measurement of the facial/peri-orbital proportions of patients with syndromal craniosynostosis (Crouzon, Apert) and CFNS without hypertelorism (Control population I), distances on photographs will be measured using Photoshop (2D photographs) or Maxilim (3D-photographs). From a frontal view the midface is divided in 5 regions: the interocular distance, the ocular expanses and the distances between the lateral canthus to the medial part of ear. We will evaluate the osseous proportions with CT/CBCT. Analysis will be done in Maxilim (CT, CBCT) and outcome will be described in millimetres and/or ratios. This will be done in Control population I (if these are available) and II. With this method, the height and width of the orbit will be measured, and the interdacryon distance determined in millimetres. All these measurements have been performed in Study population I and IIA pre-operatively and postoperatively. Then comparison with aged matched controls with syndromal craniosynostosis (Crouzon, Apert) and CFNS without | — |
Countries
Netherlands
Outcome results
None listed