<abstract xmlns="http://www.w3.org/1999/xhtml">

<sec><h3>Background</h3><p>Van der Woude syndrome (VWS) is a rare congenital malformation characterized by lower lip pits among patients with a lip and/or palate cleft. It is transmitted by an autosomal dominant inheritance with variable expressivity.</p></sec>
<sec><h3>Methods</h3><p>The study group consisted of 24 consecutive patients (13 males and 11 females) with VWS operated on at a single center between 2009 and 2022. They suffered from: bilateral cleft lip and palate – 6 patients; unilateral cleft lip and palate – 9 patients; cleft lip – 1 patient; and isolated cleft palate – 8 patients.</p></sec>
<sec><h3>Results</h3><p>In 16 (66%) cases pits of lower lip occurred on both side of midline, while in 8 (34%) the pits were detected unilaterally. The primary cleft repairs were performed according to one-stage principle at the mean age of 8.6 months (SD 1.4, range 6–12). In all patients lower lip pits repairs were performed after the primary cleft repairs as a separate procedure at the mean age of 37 months (SD 11.3 range 14–85). The mean number of all primary repairs of the syndrome—both cleft defect and lower lip pits repairs—was 2.46. Nine patients (37.5%) required additional secondary corrections of the lower lip due to the poor aesthetic post-operative outcome.</p></sec>
<sec><h3>Conclusions</h3><p>The frequent need for secondary corrections of residual lower lip deformities indicates the considerable difficulties in obtaining a satisfactory outcome of the repairs to lip pits caused by VWS. The average number of the primary surgical interventions in evaluated material remained low.</p></sec>
</abstract>

BackgroundVan der Woude syndrome (VWS) is a rare congenital malformation characterized by lower lip pits among patients with a lip and/or palate cleft. It is transmitted by an autosomal dominant inheritance with variable expressivity.
MethodsThe study group consisted of 24 consecutive patients (13 males and 11 females) with VWS operated on at a single center between 2009 and 2022. They suffered from: bilateral cleft lip and palate – 6 patients; unilateral cleft lip and palate – 9 patients; cleft lip – 1 patient; and isolated cleft palate – 8 patients.
ResultsIn 16 (66%) cases pits of lower lip occurred on both side of midline, while in 8 (34%) the pits were detected unilaterally. The primary cleft repairs were performed according to one-stage principle at the mean age of 8.6 months (SD 1.4, range 6–12). In all patients lower lip pits repairs were performed after the primary cleft repairs as a separate procedure at the mean age of 37 months (SD 11.3 range 14–85). The mean number of all primary repairs of the syndrome—both cleft defect and lower lip pits repairs—was 2.46. Nine patients (37.5%) required additional secondary corrections of the lower lip due to the poor aesthetic post-operative outcome.
ConclusionsThe frequent need for secondary corrections of residual lower lip deformities indicates the considerable difficulties in obtaining a satisfactory outcome of the repairs to lip pits caused by VWS. The average number of the primary surgical interventions in evaluated material remained low.

Surgical Treatment of Lip Pits in Van der Woude Syndrome: A Preliminary Retrospective Study of 24 Patients

Department of Maxillo-facial Surgery, Clinic of Pediatric Surgery

Journal of Mother and Child

This work is licensed under the Creative Commons Attribution 4.0 International License.

BackgroundVan der Woude syndrome (VWS) is a rare congenital malformation characterized by lower lip pits among patients with a lip and/or palate cleft. It is...

Surgical Treatment of Lip Pits in Van der Woude Syndrome: A Preliminary Retrospective Study of 24 Patients

Article Category: Case Study

Pubblicato online: 19 giu 2024

Pagine: 45 - 50

Ricevuto: 16 mar 2024

Accettato: 23 mag 2024

DOI: https://doi.org/10.34763/jmotherandchild.20242801.d-24-00020

Parole chiave
Van der Woude syndrome, cleft lip and/or palate, primary cleft repair, lip pits

© 2024 Łukasz Wieprzowski et al., published by Sciendo

This work is licensed under the Creative Commons Attribution 4.0 International License.

Surgical Treatment of Lip Pits in Van der Woude Syndrome: A Preliminary Retrospective Study of 24 Patients

Article Category: Case Study

Pubblicato online: 19 giu 2024

Pagine: 45 - 50

Ricevuto: 16 mar 2024

Accettato: 23 mag 2024

DOI: https://doi.org/10.34763/jmotherandchild.20242801.d-24-00020

Parole chiaveVan der Woude syndrome, cleft lip and/or palate, primary cleft repair, lip pits

© 2024 Łukasz Wieprzowski et al., published by Sciendo

This work is licensed under the Creative Commons Attribution 4.0 International License.

Parole chiave
Van der Woude syndrome, cleft lip and/or palate, primary cleft repair, lip pits