{"title":"额叶发育不良伴严重肥大的眼眶框截骨术:我们的首次经验","authors":"I. Adnyana, G. Samsarga, Rachel Vania","doi":"10.4103/jclpca.jclpca_4_23","DOIUrl":null,"url":null,"abstract":"Frontonasal dysplasia (FND) is a rare craniofacial deformity, including severe hypertelorism. We herein presented the case of a 6-year-old female patient with FND and Grade III hypertelorism who underwent a four-wall-orbital box osteotomy with a bicoronal approach. Orbital wall osteotomy was performed behind the lacrimal fossa. Central resection was done to allow for medial translocation of the orbits. At the last, the newly positioned orbitals were fixated using wire, absorbable plates, and screws. Neurosurgical assistance with the frontal bone flap before orbital osteotomy helps to leave the cribriform plate intact to preserve the olfactory. Finally, excessive median soft tissue was excised. The patient's hypertelorism improved after surgery. Careful surgical procedures to correct the soft and bony tissue are beneficial treatments for improving the quality of life and general condition of patients with hypertelorism.","PeriodicalId":34294,"journal":{"name":"Journal of Cleft Lip Palate and Craniofacial Anomalies","volume":"161 1","pages":"103 - 106"},"PeriodicalIF":0.0000,"publicationDate":"2023-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Orbital box osteotomy in frontonasal dysplasia with severe hypertelorism: Our first experience\",\"authors\":\"I. Adnyana, G. Samsarga, Rachel Vania\",\"doi\":\"10.4103/jclpca.jclpca_4_23\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Frontonasal dysplasia (FND) is a rare craniofacial deformity, including severe hypertelorism. We herein presented the case of a 6-year-old female patient with FND and Grade III hypertelorism who underwent a four-wall-orbital box osteotomy with a bicoronal approach. Orbital wall osteotomy was performed behind the lacrimal fossa. Central resection was done to allow for medial translocation of the orbits. At the last, the newly positioned orbitals were fixated using wire, absorbable plates, and screws. Neurosurgical assistance with the frontal bone flap before orbital osteotomy helps to leave the cribriform plate intact to preserve the olfactory. Finally, excessive median soft tissue was excised. The patient's hypertelorism improved after surgery. Careful surgical procedures to correct the soft and bony tissue are beneficial treatments for improving the quality of life and general condition of patients with hypertelorism.\",\"PeriodicalId\":34294,\"journal\":{\"name\":\"Journal of Cleft Lip Palate and Craniofacial Anomalies\",\"volume\":\"161 1\",\"pages\":\"103 - 106\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Cleft Lip Palate and Craniofacial Anomalies\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/jclpca.jclpca_4_23\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Cleft Lip Palate and Craniofacial Anomalies","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/jclpca.jclpca_4_23","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
前鼻骨发育不良(FND)是一种罕见的颅面畸形,包括严重的畸形肥大。我们在本文中介绍了一例患有 FND 和 III 度畸形的 6 岁女性患者,她接受了双冠法四壁眶盒截骨术。眼眶壁截骨手术在泪窝后方进行。进行了中央切除,以便眼眶向内侧移位。最后,使用钢丝、可吸收钢板和螺钉固定新定位的眼眶。在进行眼眶截骨术之前,神经外科医生会在额骨瓣上进行辅助,这有助于完整保留楔形骨板,从而保留嗅觉。最后,切除了过多的正中软组织。术后,患者的肥大视力得到了改善。对软组织和骨组织进行仔细的手术矫正,是改善眼球后凸患者生活质量和全身状况的有效治疗方法。
Orbital box osteotomy in frontonasal dysplasia with severe hypertelorism: Our first experience
Frontonasal dysplasia (FND) is a rare craniofacial deformity, including severe hypertelorism. We herein presented the case of a 6-year-old female patient with FND and Grade III hypertelorism who underwent a four-wall-orbital box osteotomy with a bicoronal approach. Orbital wall osteotomy was performed behind the lacrimal fossa. Central resection was done to allow for medial translocation of the orbits. At the last, the newly positioned orbitals were fixated using wire, absorbable plates, and screws. Neurosurgical assistance with the frontal bone flap before orbital osteotomy helps to leave the cribriform plate intact to preserve the olfactory. Finally, excessive median soft tissue was excised. The patient's hypertelorism improved after surgery. Careful surgical procedures to correct the soft and bony tissue are beneficial treatments for improving the quality of life and general condition of patients with hypertelorism.