Tubbs R Shane, Wellons III John C, Grabb Paul A, Oakes W Jerry
Department of Cell Biology, University of Alabama at Birmingham, Birmingham, AL, USA.
Pediatr Neurosurg. 2003 Jul;39(2):104-7. doi: 10.1159/000071322.
We report two cases of children with occult spinal dysraphism who were also found to have many associated brain anomalies seen in the Chiari II malformation. No previous report has commented on the possible association between the Chiari II malformation and the 'closed' form of neural tube defect. One child had symptoms referable to pathology at the craniocervical junction. Neither child had cutaneous stigmata associated with occult spinal dysraphism over the caudal midline spine. These cases, although seemingly rare, lend support to the theories that the association between the Chiari II malformation and patients with myelomeningoceles is due to dysgenesis of the rostral and caudal neural tube. These cases are also important as refutation of earlier theories that link these two entities by proposing that the Chiari II malformation is due to overdrainage of cerebrospinal fluid at the site of myelomeningocele.
我们报告了两例隐匿性脊柱裂患儿，他们还被发现有许多与Chiari II型畸形相关的脑部异常。此前尚无报告评论Chiari II型畸形与神经管缺损“闭合”形式之间可能存在的关联。其中一名儿童有颅颈交界区病变相关的症状。两名儿童在尾侧中线脊柱上均无与隐匿性脊柱裂相关的皮肤体征。这些病例虽然看似罕见，但支持了以下理论，即Chiari II型畸形与脊髓脊膜膨出患者之间的关联是由于头端和尾端神经管发育异常所致。这些病例也很重要，因为它们反驳了早期将这两种情况联系起来的理论，即认为Chiari II型畸形是由于脊髓脊膜膨出部位脑脊液过度引流所致。
