Wound Complications After Cleft Repair in Children With Van der Woude Syndrome
Wound Complications After Cleft Repair in Children With Van der Woude Syndrome
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DOI：
10.1097/scs.0b013e3181ec6aad
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发表时间：
2010-09-01
影响因子：
0.9
通讯作者：
Dunnwald, Martine
中科院分区：
文献类型：
--
作者：
Jones, Jodi L. P.;Canady, John W.;Dunnwald, Martine
货车德沃德综合征（VWS; OMIM 119300）是一种常染色体显性疾病，与唇和/或腭裂和下唇凹陷有关，由干扰素调节因子6（IRF 6）突变引起。唇腭裂儿童的标准做法是手术修复，这需要适当的伤口愈合。我们检验了这样一个假设，即VWS患儿在唇腭裂修复术后比非综合征性唇腭裂（NSCLP）患儿更容易出现伤口并发症。此外，我们假设患有VWS的儿童有更多的外科手术。进行了一项回顾性病例对照研究。对17名VWS儿童和68名匹配的NSCLP对照组进行了评分，以评估唇腭裂修复后伤口并发症的存在、并发症的严重程度和0至10岁的手术次数。在17例VWS患儿中，8例出现伤口并发症。在68例对照组中，13例出现伤口并发症（P = 0.02）。在VWS组的8例伤口并发症中，6例为严重并发症，而在对照组的13例并发症中，9例为严重并发症（P = 0.04）。伤口并发症多为瘘，发生于孤立性腭裂和双侧唇裂。VWS组的平均手术次数为3.0，而对照组为2.8（P = 0.67）。我们的研究表明，与NSCLP儿童相比，VWS儿童在腭裂修复术后发生伤口并发症的风险增加。此外，这些数据支持IRF 6在伤口愈合中的作用。
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.