Wound Complications Following Cleft Repair in Children with Van der Woude Syndrome

Van der Woude Syndrome (VWS, OMIM #119300) is an autosomal dominant condition associated with clefts of the lip and/or palate and lower lip pits and is caused by mutations in Interferon Regulatory Factor 6 ( IRF6 ). The standard of practice for children born with cleft lip/palate is surgical repair, which requires proper wound healing. We tested the hypothesis that children with VWS are more likely to have wound complications following cleft repair than children with non-syndromic cleft lip/palate (NSCLP). Furthermore, we hypothesized that children with VWS have more surgical procedures. A retrospective, case-controlled study was performed. Seventeen children with VWS and 68 matched controls with NSCLP were scored for presence of wound complications following cleft repair, severity of complications and number of surgeries from ages 0–10. Of the 17 children with VWS, 8 had wound complications. Thirteen of 68 controls had wound complications (P=0.02). Six of eight wound complications in the VWS group were major with nine of 13 complications in the control group major (P=0.04). The majority of wound complications were fistulae and occurred in isolated cleft palate and bilateral cleft lip. The mean number of surgeries in the VWS group was 3.0 compared to 2.8 in the control group (P=0.67). Our studies suggest that children with VWS have an increased risk for wound complications following cleft repair compared to children with NSCLP. Furthermore the data supports a role for IRF6 in wound healing.