Abstract:
Caudal regression syndrome is a form of segmental spinal dysgenesis involving the caudal spinal column, ranging from segmental coccygeal agenesis to extensive thoracolumbar agenesis with varying degrees of spinal cord dysgenesis. A majority of caudal regression cases are sporadic but maternal pre-gestational diabetes mellitus is an important risk factor. Imaging is an integral part of management of caudal regression syndrome. Antenatal diagnosis on obstetric ultrasound and evaluation with fetal MRI is ideal. Early postnatal diagnosis and/or detailed evaluation with MRI is essential for early management to improve outcomes. Pang classification categorizes caudal regression syndrome into two categories based on the position of the conus while Renshaw classification is based on the degree of vertebral column agenesis. Caudal regression syndrome may be associated with several additional anomalies, both spinal and extraspinal. A number of genitourinary and gastrointestinal anomalies have been described in association with caudal regression syndrome. The field of view of MRI of the lumbosacral spine in caudal regression syndrome needs to be extended to visualize the retroperitoneal structures without the use of a saturation band. Syndromic associations may be suspected, and additional imaging performed, based on findings of extended field of view MRI of the spine. Associated sacral masses and filar abnormalities need to be identified and may also require surgical treatment. The multisystem nature of this disease necessitates a multimodality approach to the evaluation and management of caudal regression syndrome with close cooperation between pediatric neuroradiologists and body radiologists as well as multiple clinical teams. Appropriate early management with surgical correction as necessary can significantly improve prognosis and survival in caudal regression syndrome.
摘要:
尾端回归综合征是一种涉及尾端脊柱的节段性脊柱发育不全,从节段尾骨发育不全到广泛的胸腰椎发育不全,并伴有不同程度的脊髓发育不全。大多数尾部消退病例是散发性的,但孕妇孕前糖尿病是一个重要的危险因素。影像学是治疗尾回归综合征不可或缺的一部分。产前诊断产科超声和胎儿MRI评估是理想的。产后早期诊断和/或MRI的详细评估对于早期治疗以改善结局至关重要。Pang分类根据圆锥的位置将尾回归综合征分为两类,而Renshaw分类根据脊柱发育不全的程度。尾端回归综合征可能与几个额外的异常有关,脊柱和脊柱外.已经描述了许多泌尿生殖系统和胃肠道异常与尾消退综合征有关。在不使用饱和带的情况下,需要扩展尾回归综合征的腰骶椎MRI视野以可视化腹膜后结构。可能怀疑综合征关联,和额外的成像,基于脊柱扩展视野MRI的发现。相关的骶骨肿块和丝异常需要识别,也可能需要手术治疗。这种疾病的多系统性质需要采用多模态方法来评估和管理尾回归综合征,并在儿科神经放射科医生和身体放射科医生以及多个临床团队之间密切合作。适当的早期治疗和必要的手术矫正可以显着改善尾消退综合征的预后和生存率。