Van Der Woude in Unilateral Cleft Lip: Phenotypic Patterns and Surgical Adaptations.

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. The authors 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 preoperative 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 3 hospital sites. All 8 patients had left-sided unilateral cleft lip and palate and Melbourne Classification type 2 tissue hypoplasia, with shortened vertical lip and red vermillion height.

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 type 2 hypoplasia of the lateral lip element. It is important to observe the presence of hypoplasia in the lateral lip element when attempting to balance any cleft lip repair. Technical nuances are presented, which may assist in improving appearance outcomes.