O'Neill Nora, Cook Danielle, Verhofste Bram, Smith John, Emans John B
Director Emeritus, Division of Spine Surgery, Department of Orthopaedic Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Department of Orthopaedics, Primary Children's Hospital, University of Utah, Salt Lake City, UT, USA.
Spine Deform. 2022 May;10(3):717-725. doi: 10.1007/s43390-021-00453-w. Epub 2022 Jan 9.
Although scoliosis and kyphosis have been associated with Williams Syndrome (WS), no previous literature has reported on surgical treatment for early onset scoliosis (EOS) in WS. The aim of this case series is to report on the outcomes of spine deformity surgery in patients with EOS and WS and any perioperative anesthetic or cardiovascular complications.
One multicenter database was queried for all patients with WS who underwent growth-friendly (GF) treatment before age 12 between 2000 and 2017. Demographics, surgical, and growth-friendly data were queried. Radiographs were measured for curve magnitude, T1-T12 length, and T1-S1 length.
Seven patients were analyzed (3 males, 4 females). Patients were at a median age of 2.8 years at initial surgery with median follow-up 3.6 years (range 2.0-12 years) after index surgery. The initial surgical treatments were as follows: 2 traditional growing rods (TGR), 2 magnetically controlled growing rods (MCGR), and 3 vertical expandable prosthetic titanium ribs (VEPTR). The median duration of growth-friendly treatment was 5.0 years (range, 2.6-10.4 years) with a median number of 9 device lengthenings. The median improvement in coronal curve magnitude from preoperative to most recent follow-up was 19° (range, 54°-9°). Three patients have completed GF treatment: one underwent definitive fusion, and two are under observation with apparent spontaneous fusion and retain the original GF implants. No peri-operative anesthetic or cardiovascular complications occurred.
Few studies have reported on surgical outcomes in WS patients with EOS. In this case series, 6/7 patients experienced curve improvement with growth-friendly spine instrumentation. This study suggests that growth-friendly instrumentation for severe EOS in WS can be used for control of spinal deformity while allowing for further growth. Associated complications were typical of distraction-based EOS surgical treatment. There were 62 total procedures with general anesthesia, but no perioperative cardiac complications occurred.
虽然脊柱侧凸和后凸与威廉姆斯综合征（WS）有关，但此前尚无文献报道WS早期发作性脊柱侧凸（EOS）的手术治疗情况。本病例系列的目的是报告EOS和WS患者脊柱畸形手术的结果以及任何围手术期麻醉或心血管并发症。
查询一个多中心数据库，以获取2000年至2017年间12岁前接受生长友好型（GF）治疗的所有WS患者的信息。查询人口统计学、手术和生长友好型数据。测量X线片的侧弯度数、T1-T12长度和T1-S1长度。
分析了7例患者（3例男性，4例女性）。患者初次手术时的中位年龄为2.8岁，初次手术后的中位随访时间为3.6年（范围2.0 - 12年）。初次手术治疗如下：2例采用传统生长棒（TGR），2例采用磁控生长棒（MCGR），3例采用垂直可扩张人工钛肋（VEPTR）。生长友好型治疗的中位持续时间为5.0年（范围2.6 - 10.4年），中位延长器械次数为9次。从术前到最近一次随访，冠状面侧弯度数的中位改善为19°（范围54° - 9°）。3例患者已完成GF治疗：1例接受了确定性融合，2例在观察中，有明显的自发融合，保留了原来的GF植入物。未发生围手术期麻醉或心血管并发症。
很少有研究报道WS合并EOS患者的手术结果。在本病例系列中，6/7的患者通过生长友好型脊柱器械治疗侧弯得到改善。本研究表明，生长友好型器械用于WS中严重EOS的治疗，可用于控制脊柱畸形，同时允许进一步生长。相关并发症是基于撑开的EOS手术治疗的典型并发症。共有62例手术采用全身麻醉，但未发生围手术期心脏并发症。
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