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。

IF 1.6 Q3 CLINICAL NEUROLOGY Spine deformity Pub Date : 2024-10-28 DOI:10.1007/s43390-024-00993-x
Anne-Marie Datcu, David Thornberg, Anna Booth, Daniel J Sucato
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引用次数: 0

摘要

目的:比尔斯综合征或先天性挛缩性蛛网膜挛缩症是一种罕见的疾病,以多发性关节挛缩、马凡诺德体型和皱缩耳为特征,并与脊柱侧弯有关。本研究报告了一系列因脊柱畸形而接受手术治疗的比尔斯综合征患者的临床资料:方法:对一家医疗机构中所有经基因确诊为比尔斯综合征并接受脊柱侧弯手术治疗的患者进行回顾性研究,以了解手术效果和并发症:共有八名患者接受了手术治疗,平均年龄为11.5岁,其中四名为女性,四名患有需要治疗的心血管异常。术前冠状面Cobb为82.3°,术后改善至42.1°(矫正率46.8%),最后随访时为46.5°(矫正率43.5%)。有三名患者在术前使用了半重力牵引。3名患者进行了后路器械植入和融合术(PSFI),2名患者进行了前路/PSFI联合术,1名患者进行了拴系术,1名患者进行了PSFI和后路椎间盘切除术,1名患者进行了PSFI和椎体切除术。八名患者中有一名患者术中出现了严重的神经监测事件,但在采取适当的应对措施后恢复正常,没有患者醒来时出现神经功能缺损。两名患者因植入物脱落而意外返回手术室,但都成功进行了翻修:结论:与比尔斯综合征相关的脊柱侧弯在手术时可能会有较大的弯曲,需要采用多种手术方法才能达到良好的效果。25%的患者需要返回手术室进行翻修手术。
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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.

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来源期刊
CiteScore
3.20
自引率
18.80%
发文量
167
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.
期刊最新文献
18th International Congress on Early Onset Scoliosis and the Growing Spine : November 13-15, 2024 Scottsdale, Arizona, USA. A team approach to improve outcomes in pediatric scoliosis surgery: a review of the current literature. Research trends of biomechanics in scoliosis from 1999 to 2023: a bibliometric analysis. Is the information provided by large language models valid in educating patients about adolescent idiopathic scoliosis? An evaluation of content, clarity, and empathy : The perspective of the European Spine Study Group. What imaging does my AIS patient need? A multi-group survey of provider preferences.
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