Nievelstein R A, Valk J, Smit L M, Vermeij-Keers C
Department of Diagnostic Radiology, Free University Hospital, Amsterdam, The Netherlands.
AJNR Am J Neuroradiol. 1994 Jun;15(6):1021-9.
To evaluate the spectrum of developmental anomalies observed in patients with the caudal regression syndrome and relate them to the pathogenesis of this syndrome.
Nineteen children with caudal regression were investigated with MR.
The level of vertebral agenesis varied from T-11 to S-5. In 9 of the 19 children the characteristic high-ending wedge-shaped cord terminus was observed. A separation of the anterior and posterior spinal roots of the cauda equina was observed in 9 patients. Four patients had a tethered spinal cord, in 1 in combination with a wedge-shaped cord terminus.
The pathogenesis of the caudal regression syndrome can be divided into two kinds: there is usually a disturbance of the primary neurulation process; in other cases there is a derailment of the process of degeneration and differentiation of an initially normally developed primary and secondary neural tube. MR aids understanding of the morphology and pathogenesis of congenital malformations involved (including the associated anomalies of the genitourinary and gastrointestinal systems), but other studies are still necessary to determine the exact mechanism of this syndrome.
评估尾椎退化综合征患者中观察到的发育异常谱，并将其与该综合征的发病机制相关联。
对19例尾椎退化患儿进行了磁共振成像（MR）检查。
椎体发育不全的水平从T-11至S-5不等。在19例患儿中的9例观察到特征性的高位终末楔形脊髓末端。在9例患者中观察到马尾神经前后根分离。4例患者存在脊髓栓系，其中1例合并楔形脊髓末端。
尾椎退化综合征的发病机制可分为两类：通常存在原发性神经胚形成过程的紊乱；在其他情况下，最初正常发育的原发性和继发性神经管的退化和分化过程出现异常。磁共振成像有助于理解所涉及的先天性畸形的形态和发病机制（包括泌尿生殖系统和胃肠道系统的相关异常），但仍需要其他研究来确定该综合征的确切机制。
