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Wound complications after cleft repair in children with Van der Woude syndrome
Jodi L P Jones1, John W Canady, James T Brookes
1Department of Otolaryngology, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.
Insights
Children with Van der Woude syndrome (VWS) face a higher risk of wound complications after cleft repair surgery compared to those with non-syndromic clefts. This finding suggests a role for IRF6 in surgical wound healing.
Area of Science:
- Genetics
- Pediatric Surgery
- Wound Healing
Background:
- Van der Woude syndrome (VWS) is an autosomal-dominant disorder characterized by cleft lip/palate and lower lip pits, caused by IRF6 mutations.
- Surgical repair is standard for cleft lip/palate, necessitating effective wound healing.
- The role of VWS in post-surgical wound healing outcomes remains unclear.
Purpose of the Study:
- To investigate if children with VWS experience more wound complications after cleft repair than children with non-syndromic cleft lip/palate (NSCLP).
- To compare the severity of wound complications and the number of surgical procedures between VWS patients and NSCLP controls.
- To explore the potential involvement of IRF6 in surgical wound healing processes.
Main Methods:
- A retrospective, case-controlled study design was employed.
- Seventeen children with VWS were compared to 68 matched NSCLP controls.
- Data collected included the presence and severity of wound complications and the number of surgeries from birth to age 10.
Main Results:
- Children with VWS had a significantly higher incidence of wound complications post-cleft repair (8/17) compared to NSCLP controls (13/68; P = 0.02).
- Major complications were more frequent in the VWS group (6/8) than in the control group (9/13; P = 0.04).
- The mean number of surgeries did not differ significantly between groups (3.0 for VWS vs. 2.8 for NSCLP; P = 0.67).
Conclusions:
- Children with VWS exhibit an increased risk of wound complications following cleft repair surgery.
- These findings support a significant role for the IRF6 gene in the biological processes of surgical wound healing.
- Further research into VWS and wound healing could inform surgical strategies and patient management.
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.
