Abstract
Background:
Van der Woude syndrome is synonymous with a severe phenotypic expression of cleft lip and palate. Traditionally the severity of unilateral cleft lip has been associated to the width of a cleft. We examine the presentation of the unilateral cleft lip in Van der Woude syndrome, with a focus on the lateral lip element.
Methods:
This is a retrospective, observational study of patients with a unilateral cleft lip and palate, a diagnosis of Van der Woude syndrome and pre-operative clinical images treated at The Royal Children’s Hospital, Melbourne, Australia; Royal Victoria Infirmary, Newcastle upon Tyne, UK or Menino Jesus Municipal Hospital, São Paulo, Brazil. Patient images were classified into different levels of lateral element hypoplasia according to the Melbourne Classification.
Results:
Eight patients with Van der Woude syndrome and unilateral cleft lip and palate were identified across the three hospital sites. All eight patients had left sided unilateral cleft lip and palate and judge to have Melbourne Classification Type 2 tissue hypoplasia, with shortened vertical lip height and reduced vermilion volume involving the lateral lip element.
Conclusions:
This cohort of children with unilateral cleft lip and palate in the setting of Van der Woude syndrome presented with a left sided preponderance and significant hypoplasia of the lateral lip element. It is important to observe the presence of hypoplasia when attempting to balance any cleft lip repair. Technical nuances are presented which may assist in improving appearance outcomes.
Van der Woude syndrome is synonymous with a severe phenotypic expression of cleft lip and palate. Traditionally the severity of unilateral cleft lip has been associated to the width of a cleft. We examine the presentation of the unilateral cleft lip in Van der Woude syndrome, with a focus on the lateral lip element.
Methods:
This is a retrospective, observational study of patients with a unilateral cleft lip and palate, a diagnosis of Van der Woude syndrome and pre-operative clinical images treated at The Royal Children’s Hospital, Melbourne, Australia; Royal Victoria Infirmary, Newcastle upon Tyne, UK or Menino Jesus Municipal Hospital, São Paulo, Brazil. Patient images were classified into different levels of lateral element hypoplasia according to the Melbourne Classification.
Results:
Eight patients with Van der Woude syndrome and unilateral cleft lip and palate were identified across the three hospital sites. All eight patients had left sided unilateral cleft lip and palate and judge to have Melbourne Classification Type 2 tissue hypoplasia, with shortened vertical lip height and reduced vermilion volume involving the lateral lip element.
Conclusions:
This cohort of children with unilateral cleft lip and palate in the setting of Van der Woude syndrome presented with a left sided preponderance and significant hypoplasia of the lateral lip element. It is important to observe the presence of hypoplasia when attempting to balance any cleft lip repair. Technical nuances are presented which may assist in improving appearance outcomes.
| Original language | English |
|---|---|
| Journal | Journal of Craniofacial Surgery |
| Early online date | 10 Apr 2025 |
| DOIs | |
| Publication status | E-pub ahead of print - 10 Apr 2025 |
Bibliographical note
Publisher Copyright:© 2025 by Mutaz B. Habal, MD.