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Wound complications after cleft repair in children with Van der Woude syndrome
Journal article   Open access   Peer reviewed

Wound complications after cleft repair in children with Van der Woude syndrome

Jodi L P Jones, John W Canady, James T Brookes, George L Wehby, Jamie L'Heureux, Brian C Schutte, Jeffrey C Murray and Martine Dunnwald
The Journal of craniofacial surgery, Vol.21(5), pp.1350-1353
09/2010
DOI: 10.1097/SCS.0b013e3181ec6aad
PMCID: PMC3018692
PMID: 20856020
url
https://www.ncbi.nlm.nih.gov/pmc/articles/3018692View
Open Access

Abstract

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 after cleft repair than children with nonsyndromic 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 the presence of wound complications after cleft repair, for the severity of complications, and for number of surgeries from age 0 to 10. Of the 17 children with VWS, 8 had wound complications. Of 68 controls, 13 had wound complications (P = 0.02). Of 8 wound complications in the VWS group, 6 were major, whereas of 13 complications in the control group, 9 were major (P = 0.04). Most 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 with 2.8 in the control group (P = 0.67). Our studies suggest that children with VWS have an increased risk for wound complications after cleft repair compared with children with NSCLP. Furthermore, these data support a role for IRF6 in wound healing.
 Humans Cleft Palate - surgery Child, Preschool Infant Male Postoperative Complications - therapy Cleft Palate - genetics Postoperative Complications - epidemiology Wound Healing Case-Control Studies Syndrome Cleft Lip - genetics Surgical Wound Dehiscence - therapy Female Retrospective Studies Surgical Wound Dehiscence - epidemiology Cleft Lip - surgery

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