Anne-Marie Datcu, David Thornberg, Anna Booth, Daniel J Sucato
{"title":"比尔斯综合症患者的脊柱畸形手术可以有效实施,但存在翻修手术的风险。","authors":"Anne-Marie Datcu, David Thornberg, Anna Booth, Daniel J Sucato","doi":"10.1007/s43390-024-00993-x","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong>Beals syndrome or congenital contractual arachnodactyly is a rare disorder characterized by multiple joint contractures, Marfanoid body habitus and crumpled ears and has been associated with scoliosis. This study reports a clinical series of patients with Beals syndrome who have had surgical treatment for their spinal deformity.</p><p><strong>Methods: </strong>A retrospective review of all patients at a single institution who had a genetically-confirmed diagnosis of Beals syndrome and had surgical treatment for their scoliosis were reviewed for surgical outcome and complications.</p><p><strong>Results: </strong>There were eight patients who had surgery at an average age of 11.5 years, four were female and four had cardiovascular abnormalities requiring treatment. The preoperative coronal Cobb was 82.3° which improved to 42.1°(46.8% correction), and 46.5° (43.5% correction) at final follow-up. Preoperative halo-gravity-traction was used in three patients. Three patients had a posterior instrumentation and fusion (PSFI), 2 a combined anterior/PSFI, 1 had tethering, 1 with PSFI with posteriorly-approached discectomy, and 1 with a PSFI and vertebral column resection. One of the eight patients had a critical intraoperative neuromonitoring event but was normal following appropriate responses and no patient awoke with neurologic deficits. Two had an unplanned return to the operating room for implant dislodgement and each had a successful revision.</p><p><strong>Conclusions: </strong>Scoliosis associated with Beals syndrome can have large curves at the time of surgery and require a variety of surgical approaches to achieve a good result. Revision surgery with return to the operating room is necessary in 25% of patients.</p>","PeriodicalId":21796,"journal":{"name":"Spine deformity","volume":" ","pages":""},"PeriodicalIF":1.6000,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Spine deformity surgery in patients with Beals syndrome can be effectively performed but does risk revision surgery.\",\"authors\":\"Anne-Marie Datcu, David Thornberg, Anna Booth, Daniel J Sucato\",\"doi\":\"10.1007/s43390-024-00993-x\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Purpose: </strong>Beals syndrome or congenital contractual arachnodactyly is a rare disorder characterized by multiple joint contractures, Marfanoid body habitus and crumpled ears and has been associated with scoliosis. This study reports a clinical series of patients with Beals syndrome who have had surgical treatment for their spinal deformity.</p><p><strong>Methods: </strong>A retrospective review of all patients at a single institution who had a genetically-confirmed diagnosis of Beals syndrome and had surgical treatment for their scoliosis were reviewed for surgical outcome and complications.</p><p><strong>Results: </strong>There were eight patients who had surgery at an average age of 11.5 years, four were female and four had cardiovascular abnormalities requiring treatment. The preoperative coronal Cobb was 82.3° which improved to 42.1°(46.8% correction), and 46.5° (43.5% correction) at final follow-up. Preoperative halo-gravity-traction was used in three patients. Three patients had a posterior instrumentation and fusion (PSFI), 2 a combined anterior/PSFI, 1 had tethering, 1 with PSFI with posteriorly-approached discectomy, and 1 with a PSFI and vertebral column resection. One of the eight patients had a critical intraoperative neuromonitoring event but was normal following appropriate responses and no patient awoke with neurologic deficits. Two had an unplanned return to the operating room for implant dislodgement and each had a successful revision.</p><p><strong>Conclusions: </strong>Scoliosis associated with Beals syndrome can have large curves at the time of surgery and require a variety of surgical approaches to achieve a good result. Revision surgery with return to the operating room is necessary in 25% of patients.</p>\",\"PeriodicalId\":21796,\"journal\":{\"name\":\"Spine deformity\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":1.6000,\"publicationDate\":\"2024-10-28\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Spine deformity\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1007/s43390-024-00993-x\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Spine deformity","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1007/s43390-024-00993-x","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Spine deformity surgery in patients with Beals syndrome can be effectively performed but does risk revision surgery.
Purpose: Beals syndrome or congenital contractual arachnodactyly is a rare disorder characterized by multiple joint contractures, Marfanoid body habitus and crumpled ears and has been associated with scoliosis. This study reports a clinical series of patients with Beals syndrome who have had surgical treatment for their spinal deformity.
Methods: A retrospective review of all patients at a single institution who had a genetically-confirmed diagnosis of Beals syndrome and had surgical treatment for their scoliosis were reviewed for surgical outcome and complications.
Results: There were eight patients who had surgery at an average age of 11.5 years, four were female and four had cardiovascular abnormalities requiring treatment. The preoperative coronal Cobb was 82.3° which improved to 42.1°(46.8% correction), and 46.5° (43.5% correction) at final follow-up. Preoperative halo-gravity-traction was used in three patients. Three patients had a posterior instrumentation and fusion (PSFI), 2 a combined anterior/PSFI, 1 had tethering, 1 with PSFI with posteriorly-approached discectomy, and 1 with a PSFI and vertebral column resection. One of the eight patients had a critical intraoperative neuromonitoring event but was normal following appropriate responses and no patient awoke with neurologic deficits. Two had an unplanned return to the operating room for implant dislodgement and each had a successful revision.
Conclusions: Scoliosis associated with Beals syndrome can have large curves at the time of surgery and require a variety of surgical approaches to achieve a good result. Revision surgery with return to the operating room is necessary in 25% of patients.
期刊介绍:
Spine Deformity the official journal of the?Scoliosis Research Society is a peer-refereed publication to disseminate knowledge on basic science and clinical research into the?etiology?biomechanics?treatment?methods and outcomes of all types of?spinal deformities. The international members of the Editorial Board provide a worldwide perspective for the journal's area of interest.The?journal?will enhance the mission of the Society which is to foster the optimal care of all patients with?spine?deformities worldwide. Articles published in?Spine Deformity?are Medline indexed in PubMed.? The journal publishes original articles in the form of clinical and basic research. Spine Deformity will only publish studies that have institutional review board (IRB) or similar ethics committee approval for human and animal studies and have strictly observed these guidelines. The minimum follow-up period for follow-up clinical studies is 24 months.