PDF(Pubmed)
Abstract：
BACKGROUND: Rubinstein-Taybi syndrome (RTS) is a rare disorder with a range of congenital anomalies. Although 40% to 60% of patients with RTS have scoliotic deformities, few reports discuss the outcomes of correctional surgery and postoperative care. To raise awareness of the clinical features of RTS and surgical considerations, the authors report on the surgical treatment of a pediatric patient with RTS accompanied by scoliosis.
METHODS: A 14-year-old girl with RTS presented with low back pain associated with progressive scoliosis. Because of jaw hypoplasia, videolaryngoscopy-mediated intubation was chosen. A single-stage T4-L3 posterior corrective fusion with instrumentation was successfully performed. Physical and imaging findings were analyzed up to 2 years after correction. The main thoracic Cobb angle was corrected from 73° to 12° and maintained for 2 years after surgery. The patient\'s low back pain resolved.
CONCLUSIONS: Careful consideration of RTS-associated complications and preoperative planning, including the use of videolaryngoscopy-mediated intubation, anesthesia selection, and postoperative care, proved crucial. Scoliosis may appear in many variations in rare diseases such as RTS. Publication of case reports such as this one is needed to provide detailed information about strategies and considerations for correcting scoliotic deformities in patients with RTS.
METHODS: A 14-year-old girl with RTS presented with low back pain associated with progressive scoliosis. Because of jaw hypoplasia, videolaryngoscopy-mediated intubation was chosen. A single-stage T4-L3 posterior corrective fusion with instrumentation was successfully performed. Physical and imaging findings were analyzed up to 2 years after correction. The main thoracic Cobb angle was corrected from 73° to 12° and maintained for 2 years after surgery. The patient\'s low back pain resolved.
CONCLUSIONS: Careful consideration of RTS-associated complications and preoperative planning, including the use of videolaryngoscopy-mediated intubation, anesthesia selection, and postoperative care, proved crucial. Scoliosis may appear in many variations in rare diseases such as RTS. Publication of case reports such as this one is needed to provide detailed information about strategies and considerations for correcting scoliotic deformities in patients with RTS.
摘要：
背景：Rubinstein-Taybi综合征（RTS）是一种罕见的先天性异常疾病。尽管40％至60％的RTS患者患有脊柱侧弯畸形，很少有报告讨论矫正手术和术后护理的结果。提高对RTS临床特点及手术注意事项的认识,作者报道了1例伴有脊柱侧凸的RTS患儿的手术治疗.
方法：一名患有RTS的14岁女孩出现与进行性脊柱侧凸相关的下背痛。因为下巴发育不全,选择视频喉镜介导的插管。成功进行了带器械的单级T4-L3后路矫正融合。矫正后长达2年的物理和影像学检查结果进行分析。主胸Cobb角由73°校正至12°,术后维持2年。病人的腰痛缓解了。
结论：仔细考虑RTS相关并发症和术前计划，包括使用视频喉镜介导的插管，麻醉选择,和术后护理，证明至关重要。脊柱侧凸可能出现在罕见疾病如RTS的许多变异中。需要发布此类病例报告，以提供有关纠正RTS患者脊柱侧弯畸形的策略和注意事项的详细信息。
方法：一名患有RTS的14岁女孩出现与进行性脊柱侧凸相关的下背痛。因为下巴发育不全,选择视频喉镜介导的插管。成功进行了带器械的单级T4-L3后路矫正融合。矫正后长达2年的物理和影像学检查结果进行分析。主胸Cobb角由73°校正至12°,术后维持2年。病人的腰痛缓解了。
结论：仔细考虑RTS相关并发症和术前计划，包括使用视频喉镜介导的插管，麻醉选择,和术后护理，证明至关重要。脊柱侧凸可能出现在罕见疾病如RTS的许多变异中。需要发布此类病例报告，以提供有关纠正RTS患者脊柱侧弯畸形的策略和注意事项的详细信息。
