Shuman R M
Child Neurology, Inc, Mishawaka, IN 46545-3509, USA.
Semin Pediatr Neurol. 1995 Sep;2(3):220-6. doi: 10.1016/s1071-9091(05)80033-6.
The Chiari malformations form a group of abnormalities that are pathogenetically interrelated. The most important member of the group is the Chiari type II malformation, known as the Arnold-Chiari malformation. Its cardinal features are the myelomeningocele in the thoraco-lumbar spine, the venting of the intracranial cerebrospinal fluid through the central canal, the hypoplasia of the posterior fossa, the herniation of hindbrain into the cervical spinal canal, and the compressive damage to cranial nerves. Some of the abnormalities are progressive, and thus treatable. Limitation of progression may improve outcomes. The challenges to our treatment programs involve early diagnosis, delivery by Caesarean section, emergent closure of the neural plaque and prophylaxis of hydrocephalus, anticipatory prevention of the neurological compression syndromes, multidisciplinary teams, and age-appropriate interventions.
Chiari畸形是一组在发病机制上相互关联的异常情况。该组中最重要的成员是Chiari II型畸形，即Arnold-Chiari畸形。其主要特征包括胸腰椎的脊髓脊膜膨出、颅内脑脊液通过中央管排出、后颅窝发育不全、后脑疝入颈椎管以及对颅神经的压迫性损伤。其中一些异常是进行性的，因此是可治疗的。限制病情进展可能会改善预后。我们治疗方案面临的挑战包括早期诊断、剖宫产分娩、紧急闭合神经斑块和预防脑积水、预期预防神经压迫综合征、多学科团队以及适合年龄的干预措施。
