Abstract：
The two main objectives of this study were to explore the rate of spinal dysraphism within bladder and cloacal exstrophy and to analyze the relationship between spinal dysraphism surgery, including timing of spinal dysraphism surgery, with urological and neurological outcomes.
A prospectively maintained IRB-approved database of pediatric exstrophy patients treated from 1982 to 2021 was retrospectively reviewed for patients with spinal dysraphism. Spinal dysraphism was categorized into the following 7 subtypes: lipoma-based closed defect, myelomeningocele, meningocele, diastematomyelia, myelocystocele, low-lying conus with tethered cord/fatty filum, and sacral bony defect. Other factors assessed included patient demographic characteristics, type of spinal dysraphism procedure, reoperation, complication, presence of other neurological problems (e.g., hydrocephalus, Chiari malformation), neurological status, and urological function.
Analysis revealed that 114/1401 patients had coexisting spinal dysraphism. Of these 114, sufficient records including type of dysraphism were available for 54. Spinal dysraphism was most common within cloacal exstrophy (83.3% [45/54 patients]), followed by cloacal exstrophy variants (9.3% [5/54]), classic bladder exstrophy (3.7% [2/54]), and classic bladder exstrophy variants (3.7% [2/54]). Within spinal dysraphism, lipoma-based closed defects (63.0% [34/54]) and low-lying conus with tethered cord/fatty filum (11.1% [6/54]) were most common. Hydrocephalus and Chiari malformation occurred in 24.1% (13/54) and 11.1% (6/54) of patients. All 13 patients with hydrocephalus underwent shunt placement. Among those who underwent neurosurgical intervention, the complication rate for spinal dysraphism was 14.6% (7/48). Motor function data were available for 41 patients and revealed that motor function declined for 2/41 (4.8%) patients and improved for 6/41 (14.6%) after neurosurgery. There was no statistical difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.
The authors have reported the surgical management and outcomes of patients with exstrophy and coexisting spinal dysraphism (n = 54). In 54 patients, spinal dysraphism was most common in the subset of patients with cloacal exstrophy (83.3%). Lipoma-based closed defects (63.0%) and low-lying conus with tethered cord/fatty filum (11.1%) were the most common, and the rates of hydrocephalus and Chiari malformation were 24.1% and 11.1%, respectively. There was no difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.
A prospectively maintained IRB-approved database of pediatric exstrophy patients treated from 1982 to 2021 was retrospectively reviewed for patients with spinal dysraphism. Spinal dysraphism was categorized into the following 7 subtypes: lipoma-based closed defect, myelomeningocele, meningocele, diastematomyelia, myelocystocele, low-lying conus with tethered cord/fatty filum, and sacral bony defect. Other factors assessed included patient demographic characteristics, type of spinal dysraphism procedure, reoperation, complication, presence of other neurological problems (e.g., hydrocephalus, Chiari malformation), neurological status, and urological function.
Analysis revealed that 114/1401 patients had coexisting spinal dysraphism. Of these 114, sufficient records including type of dysraphism were available for 54. Spinal dysraphism was most common within cloacal exstrophy (83.3% [45/54 patients]), followed by cloacal exstrophy variants (9.3% [5/54]), classic bladder exstrophy (3.7% [2/54]), and classic bladder exstrophy variants (3.7% [2/54]). Within spinal dysraphism, lipoma-based closed defects (63.0% [34/54]) and low-lying conus with tethered cord/fatty filum (11.1% [6/54]) were most common. Hydrocephalus and Chiari malformation occurred in 24.1% (13/54) and 11.1% (6/54) of patients. All 13 patients with hydrocephalus underwent shunt placement. Among those who underwent neurosurgical intervention, the complication rate for spinal dysraphism was 14.6% (7/48). Motor function data were available for 41 patients and revealed that motor function declined for 2/41 (4.8%) patients and improved for 6/41 (14.6%) after neurosurgery. There was no statistical difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.
The authors have reported the surgical management and outcomes of patients with exstrophy and coexisting spinal dysraphism (n = 54). In 54 patients, spinal dysraphism was most common in the subset of patients with cloacal exstrophy (83.3%). Lipoma-based closed defects (63.0%) and low-lying conus with tethered cord/fatty filum (11.1%) were the most common, and the rates of hydrocephalus and Chiari malformation were 24.1% and 11.1%, respectively. There was no difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.
摘要：
目的：本研究的两个主要目的是探讨膀胱和泄殖腔内脊柱发育不良的发生率，并分析脊柱发育不良手术之间的关系。包括脊柱发育不良手术的时机,泌尿系统和神经系统的结果。
方法：回顾性分析了从1982年至2021年接受治疗的儿科外患患者的前瞻性维持IRB批准的数据库，以评估脊柱发育不良患者。脊髓发育不良分为以下7种亚型：基于脂肪瘤的闭合缺损，脊髓膜膨出,脑膜膨出,脊髓纵裂,骨髓囊肿,有系绳/脂肪纤维的低洼圆锥，和骶骨缺损.评估的其他因素包括患者人口统计学特征，脊柱发育不良手术类型，再操作,并发症,其他神经系统问题的存在(例如，脑积水,Chiari畸形)，神经状况,和泌尿系统功能。
结果：分析显示114/1401例患者同时存在脊柱发育不良。在这114个记录中，有54个记录包括发育异常的类型。脊柱发育不良在泄殖腔内最常见（83.3%[45/54例]），其次是泄殖腔exstrophy变体（9.3%[5/54]），经典膀胱外翻（3.7%[2/54]），和经典的膀胱外翻变种（3.7％[2/54]）。在脊髓发育不良中，以脂肪瘤为基础的闭合性缺损(63.0%[34/54])和低地圆锥与绳索/脂肪丝(11.1%[6/54])最为常见。24.1％（13/54）和11.1％（6/54）的患者发生脑积水和Chiari畸形。所有13例脑积水患者均行分流术。在接受神经外科手术的人中，脊柱发育不良的并发症发生率为14.6%(7/48)。41例患者的运动功能数据显示，神经外科手术后2/41（4.8％）患者的运动功能下降，6/41（14.6％）的改善。与神经外科手术和外翻闭合时间相关的下肢运动结果没有统计学差异。
结论：作者报道了患有脊柱发育不良的患者的手术治疗和结果（n=54）。在54名患者中，脊髓发育不良在泄殖腔外翻患者中最常见(83.3%).以脂肪瘤为基础的闭合性缺损(63.0%)和低洼的圆锥系绳/脂肪丝(11.1%)最为常见,脑积水和Chiari畸形的发生率分别为24.1%和11.1%,分别。与神经外科手术和外翻闭合时间相关的下肢运动结果没有差异。
方法：回顾性分析了从1982年至2021年接受治疗的儿科外患患者的前瞻性维持IRB批准的数据库，以评估脊柱发育不良患者。脊髓发育不良分为以下7种亚型：基于脂肪瘤的闭合缺损，脊髓膜膨出,脑膜膨出,脊髓纵裂,骨髓囊肿,有系绳/脂肪纤维的低洼圆锥，和骶骨缺损.评估的其他因素包括患者人口统计学特征，脊柱发育不良手术类型，再操作,并发症,其他神经系统问题的存在(例如，脑积水,Chiari畸形)，神经状况,和泌尿系统功能。
结果：分析显示114/1401例患者同时存在脊柱发育不良。在这114个记录中，有54个记录包括发育异常的类型。脊柱发育不良在泄殖腔内最常见（83.3%[45/54例]），其次是泄殖腔exstrophy变体（9.3%[5/54]），经典膀胱外翻（3.7%[2/54]），和经典的膀胱外翻变种（3.7％[2/54]）。在脊髓发育不良中，以脂肪瘤为基础的闭合性缺损(63.0%[34/54])和低地圆锥与绳索/脂肪丝(11.1%[6/54])最为常见。24.1％（13/54）和11.1％（6/54）的患者发生脑积水和Chiari畸形。所有13例脑积水患者均行分流术。在接受神经外科手术的人中，脊柱发育不良的并发症发生率为14.6%(7/48)。41例患者的运动功能数据显示，神经外科手术后2/41（4.8％）患者的运动功能下降，6/41（14.6％）的改善。与神经外科手术和外翻闭合时间相关的下肢运动结果没有统计学差异。
结论：作者报道了患有脊柱发育不良的患者的手术治疗和结果（n=54）。在54名患者中，脊髓发育不良在泄殖腔外翻患者中最常见(83.3%).以脂肪瘤为基础的闭合性缺损(63.0%)和低洼的圆锥系绳/脂肪丝(11.1%)最为常见,脑积水和Chiari畸形的发生率分别为24.1%和11.1%,分别。与神经外科手术和外翻闭合时间相关的下肢运动结果没有差异。
