Emans Pieter J, van Aalst Jasper, van Heurn Ernest L W, Marcelis Carlo, Kootstra Gauke, Beets-Tan Regina G H, Vles Johannes S H, Beuls Emile A M
Department of Pediatric Surgery, University Hospital Maastricht, Maastricht, The Netherlands.
Neurosurgery. 2006 May;58(5):924-9; discussion 924-9. doi: 10.1227/01.NEU.0000209945.87233.6A.
The Currarino triad, a relatively uncommon hereditary disorder, is often associated with tethered cord and anterior myelomeningocele. Little is known of the implications of these neuroanatomic malformations or of the neurosurgical attitude. The objective of this study is to identify the spinal cord and meningeal malformations associated with the Currarino triad and to discuss the risks and benefits of surgical intervention.
We analyzed the spinal cord malformations and the neurosurgical involvement with the Currarino triad by retrospective chart review.
The Currarino triad neuroanatomic malformations were identified in five patients. The Currarino triad was associated with a tethered cord in three patients, a myelomeningocele in five patients, a syrinx in two patients, a fistula between the colon and spinal canal in two patients, and an Arnold-Chiari Type 1 malformation in one patient.
Full spine imaging is required for all patients diagnosed with the Currarino triad. Magnetic resonance imaging of the head should be performed in every patient with neuroanatomic anomalies. Surgery of an anterior myelomeningocele is not necessarily indicated, only in the rare case in which the space-occupying aspect is expected to cause constipation or problems during pregnancy or delivery. Constipation directly after birth is seen in virtually all patients with the triad. Therefore, constipation cannot be used to diagnose a tethered cord syndrome nor indicate tethered cord release. Fistulas between the spinal canal and colon have to be operated on directly.
库拉里诺三联征是一种相对罕见的遗传性疾病，常与脊髓栓系和前侧脊髓脊膜膨出相关。对于这些神经解剖畸形的影响或神经外科治疗态度知之甚少。本研究的目的是确定与库拉里诺三联征相关的脊髓和脑膜畸形，并讨论手术干预的风险和益处。
我们通过回顾性病历审查分析了库拉里诺三联征患者的脊髓畸形和神经外科治疗情况。
在5例患者中发现了库拉里诺三联征的神经解剖畸形。库拉里诺三联征与3例脊髓栓系、5例脊髓脊膜膨出、2例脊髓空洞症、2例结肠与椎管之间的瘘管以及1例阿诺德 - 基亚里1型畸形相关。
所有诊断为库拉里诺三联征的患者都需要进行全脊柱成像。每例有神经解剖异常的患者都应进行头部磁共振成像检查。前侧脊髓脊膜膨出不一定需要手术，仅在极少数预期占位会导致便秘或妊娠或分娩期间出现问题的情况下才考虑手术。实际上，所有三联征患者出生后都会立即出现便秘。因此，便秘不能用于诊断脊髓栓系综合征，也不能作为脊髓栓系松解术的指征。椎管与结肠之间的瘘管必须直接进行手术治疗。
