OBJECTIVE: The purpose of this study was to investigate the surgical, speech, and audiologic outcomes in patients with Van der Woude syndrome (VWS) and compare them to patients with nonsyndromic cleft palate with or without cleft lip (NS-CP ± L) treated at the same institution. DESIGN: Retrospective chart review. SETTING: A single children's hospital at a major academic institution. PATIENTS: The records of 18 patients with VWS who had been treated at a single institution from 1989 to 2017 have been retrospectively examined. Thirty-eight patients with NS-CP ± L who were also treated at the same institution during that same time frame were selected to closely match sex and date of birth. MAIN OUTCOME MEASURES: Demographic, clinical, surgical, and speech pathology data were gathered from medical charts. RESULT: By age 4, 88% of subjects with VWS and 76% of subjects with NS-CP ± L (P = 0.732) had been, or were actively involved in, speech therapy. By age 10, 100% of remaining subjects with VWS and 58% of remaining subjects with NS-CP ± L remained involved in speech therapy (P = 0.027).About 33% of patients with VWS and 16% of patients with NS-CP ± L had a secondary procedure for velopharyngeal dysfunction (VPD) (P = 0.171). CONCLUSION: The VWS group had more than twice the rate of secondary procedures for VPD repair, and a higher rate of continuing involvement for speech therapy at age 10. No differences were found in the rate of participation in speech therapy at or by age 4.
OBJECTIVE: The purpose of this study was to investigate the surgical, speech, and audiologic outcomes in patients with Van der Woude syndrome (VWS) and compare them to patients with nonsyndromic cleft palate with or without cleft lip (NS-CP ± L) treated at the same institution. DESIGN: Retrospective chart review. SETTING: A single children's hospital at a major academic institution. PATIENTS: The records of 18 patients with VWS who had been treated at a single institution from 1989 to 2017 have been retrospectively examined. Thirty-eight patients with NS-CP ± L who were also treated at the same institution during that same time frame were selected to closely match sex and date of birth. MAIN OUTCOME MEASURES: Demographic, clinical, surgical, and speech pathology data were gathered from medical charts. RESULT: By age 4, 88% of subjects with VWS and 76% of subjects with NS-CP ± L (P = 0.732) had been, or were actively involved in, speech therapy. By age 10, 100% of remaining subjects with VWS and 58% of remaining subjects with NS-CP ± L remained involved in speech therapy (P = 0.027).About 33% of patients with VWS and 16% of patients with NS-CP ± L had a secondary procedure for velopharyngeal dysfunction (VPD) (P = 0.171). CONCLUSION: The VWS group had more than twice the rate of secondary procedures for VPD repair, and a higher rate of continuing involvement for speech therapy at age 10. No differences were found in the rate of participation in speech therapy at or by age 4.
Authors: Shinji Kondo; Brian C Schutte; Rebecca J Richardson; Bryan C Bjork; Alexandra S Knight; Yoriko Watanabe; Emma Howard; Renata L L Ferreira de Lima; Sandra Daack-Hirsch; Achim Sander; Donna M McDonald-McGinn; Elaine H Zackai; Edward J Lammer; Arthur S Aylsworth; Holly H Ardinger; Andrew C Lidral; Barbara R Pober; Lina Moreno; Mauricio Arcos-Burgos; Consuelo Valencia; Claude Houdayer; Michel Bahuau; Danilo Moretti-Ferreira; Antonio Richieri-Costa; Michael J Dixon; Jeffrey C Murray Journal: Nat Genet Date: 2002-09-03 Impact factor: 38.330