{"title":"Popliteal Pterygium Syndrome with a Family History of Van der Woude Syndrome: A Case Report.","authors":"Jiten Kumar Mishra, Shamendra Anand Sahu, Aparajita Saha, Abi Sindhuja, Jalaz Joezer Rahmi, Abhijith Valsalan","doi":"10.4103/jwas.jwas_173_23","DOIUrl":null,"url":null,"abstract":"<p><p>Popliteal pterygium syndrome (PPS) is a rare inherited disorder involving the face, limbs, and genitalia. The most prominent and handicapping deformity associated with this syndrome is Popliteal pterygium. Popliteal pterygium is a contracture band that extends from ischial tuberosity to calcaneum. It is a dense fibrous band difficult to correct surgically. We report a case of PPS with her two other family members diagnosed with Van der Woude syndrome. Single-stage surgical release is ineffective due to shortened neurovascular and surrounding soft tissues. Multimodality treatment with surgical release and motivated parents may help these children to rehabilitate.</p>","PeriodicalId":73993,"journal":{"name":"Journal of the West African College of Surgeons","volume":"14 4","pages":"445-449"},"PeriodicalIF":0.0000,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11412588/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the West African College of Surgeons","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/jwas.jwas_173_23","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/7/18 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Popliteal pterygium syndrome (PPS) is a rare inherited disorder involving the face, limbs, and genitalia. The most prominent and handicapping deformity associated with this syndrome is Popliteal pterygium. Popliteal pterygium is a contracture band that extends from ischial tuberosity to calcaneum. It is a dense fibrous band difficult to correct surgically. We report a case of PPS with her two other family members diagnosed with Van der Woude syndrome. Single-stage surgical release is ineffective due to shortened neurovascular and surrounding soft tissues. Multimodality treatment with surgical release and motivated parents may help these children to rehabilitate.