{"title":"腭成形术治疗镰形发育不良1例报告及文献复习。","authors":"Kaya Narimatsu, Akihiko Iida, Takanori Kobayashi","doi":"10.1177/1055665621992654","DOIUrl":null,"url":null,"abstract":"<p><p>Campomelic dysplasia (CMPD) is a skeletal disorder resulting from <i>SOX9</i> gene mutations. Palatoplasty is rare due to a high lethality rate in infants from respiratory distress. Our patient had characteristic symptoms of CMPD, including short bowed limbs, macrocephaly, low-set ears, short palpebral fissures, hypertelorism, a flat nasal bridge, a long philtrum, micrognathia, and a cleft palate. We performed a Furlow palatoplasty when the patient was 2 years 9 months of age, after respiratory conditions had stabilized. We reviewed the literature of CMPD cases that underwent palatoplasty and discussed the optimal timing and surgical methods.</p>","PeriodicalId":520794,"journal":{"name":"The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association","volume":" ","pages":"132-136"},"PeriodicalIF":0.0000,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/1055665621992654","citationCount":"0","resultStr":"{\"title\":\"Palatoplasty for the Patient With Campomelic Dysplasia-Report of a Case and Review of the Literature.\",\"authors\":\"Kaya Narimatsu, Akihiko Iida, Takanori Kobayashi\",\"doi\":\"10.1177/1055665621992654\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Campomelic dysplasia (CMPD) is a skeletal disorder resulting from <i>SOX9</i> gene mutations. Palatoplasty is rare due to a high lethality rate in infants from respiratory distress. Our patient had characteristic symptoms of CMPD, including short bowed limbs, macrocephaly, low-set ears, short palpebral fissures, hypertelorism, a flat nasal bridge, a long philtrum, micrognathia, and a cleft palate. We performed a Furlow palatoplasty when the patient was 2 years 9 months of age, after respiratory conditions had stabilized. We reviewed the literature of CMPD cases that underwent palatoplasty and discussed the optimal timing and surgical methods.</p>\",\"PeriodicalId\":520794,\"journal\":{\"name\":\"The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association\",\"volume\":\" \",\"pages\":\"132-136\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.1177/1055665621992654\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1177/1055665621992654\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2021/2/12 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1177/1055665621992654","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2021/2/12 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
Palatoplasty for the Patient With Campomelic Dysplasia-Report of a Case and Review of the Literature.
Campomelic dysplasia (CMPD) is a skeletal disorder resulting from SOX9 gene mutations. Palatoplasty is rare due to a high lethality rate in infants from respiratory distress. Our patient had characteristic symptoms of CMPD, including short bowed limbs, macrocephaly, low-set ears, short palpebral fissures, hypertelorism, a flat nasal bridge, a long philtrum, micrognathia, and a cleft palate. We performed a Furlow palatoplasty when the patient was 2 years 9 months of age, after respiratory conditions had stabilized. We reviewed the literature of CMPD cases that underwent palatoplasty and discussed the optimal timing and surgical methods.