Winsauer Andrew G, Thornberg David C, Rodriguez Stephen M, Poppino Kiley F, Ramo Brandon A
Orthopedics. 2023 Jul-Aug;46(4):e223-e229. doi: 10.3928/01477447-20230207-07. Epub 2023 Feb 10.
We sought to examine the modern surgical treatment of spinal deformity associated with sister imprinting disorders, Prader-Willi syndrome (PWS) and Angelman syndrome (AS), with emphasis on the specific complications encountered in these patient populations. Fifteen patients with PWS and 5 patients with AS who underwent surgical intervention for spinal deformity between 2000 and 2018 were identified. Postoperative complications were classified using the modified Clavien-Dindo-Sink (CDS) system and further categorized into specific subtypes including excessive drainage, dehiscence, implant failure, infection, and delayed wound healing. Perioperative and final follow-up radiographic data were analyzed. Mean age at surgery was 12.9 years (range, 4-21 years) with mean follow-up of 46.1 months (range, 1-145 months). There were postoperative complications in 17 patients (85%). Ten major complications (CDS ≥ 3) occurred in 9 patients (45%). These included 5 infections requiring reoperation, 1 seroma requiring drainage, 2 severe cervical-thoracic deformities requiring reoperation, 1 implant failure requiring reoperation, and 1 death secondary to fungal sepsis and thromboembolic disease. Eight additional patients (40%) had minor complications (CDS 1 or 2). Eight intraoperative complications occurred in 5 patients (25%), including loss of neuromonitoring signals and cerebrospinal fluid leaks. Surgical intervention for scoliosis in PWS and AS continues to have high complication rates secondary to medical and behavioral comorbidities found in these patient populations. The exact etiology of the high complication rates encountered cannot be definitively stated, but both syndromes frequently present with a number of unique features that may predispose patients to develop surgical complications. [. 2023;46(4):e223-e229.].
我们试图研究与姐妹印记障碍、普拉德-威利综合征（PWS）和安吉尔曼综合征（AS）相关的脊柱畸形的现代外科治疗方法，重点关注这些患者群体中遇到的特定并发症。确定了2000年至2018年间因脊柱畸形接受手术干预的15例PWS患者和5例AS患者。术后并发症采用改良的Clavien-Dindo-Sink（CDS）系统进行分类，并进一步分为特定亚型，包括引流过多、裂开、植入物失败、感染和伤口愈合延迟。分析了围手术期和最终随访的影像学数据。手术时的平均年龄为12.9岁（范围4 - 21岁），平均随访时间为46.1个月（范围1 - 145个月）。17例患者（85%）出现术后并发症。9例患者（45%）发生了10例主要并发症（CDS≥3）。其中包括5例需要再次手术的感染、1例需要引流的血清肿、2例需要再次手术的严重颈胸段畸形、1例需要再次手术的植入物失败以及1例因真菌败血症和血栓栓塞性疾病导致的死亡。另外8例患者（40%）出现轻微并发症（CDS 1或2）。5例患者（25%）发生了8例术中并发症，包括神经监测信号丢失和脑脊液漏。由于这些患者群体中存在的内科和行为合并症，PWS和AS患者脊柱侧弯的手术干预并发症发生率仍然很高。所遇到的高并发症发生率的确切病因尚不能明确说明，但这两种综合征都经常表现出一些独特的特征，这些特征可能使患者易发生手术并发症。[. 2023;46(4):e223 - e229.]
