Tubbs R Shane, Soleau Scott, Custis James, Wellons John C, Blount Jeffrey P, Oakes W Jerry
Department of Cell Biology, University of Alabama at Birmingham, Birmingham, USA.
Childs Nerv Syst. 2004 Jul;20(7):459-61. doi: 10.1007/s00381-004-0948-9. Epub 2004 Apr 2.
Tectal beaking is a common finding in children born with a myelomeningocele. Quantitation of the presence or absence of this finding correlated with normal or abnormal eye movements is lacking in the medical literature.
We prospectively evaluated 50 children in our multidisciplinary spina bifida clinic. Each child was observed for the presence or absence of nystagmus at rest or with extraocular movement. Grades of nystagmus and severity of tectal beaking (Types I-III) on MRI were then correlated.
Overwhelmingly, children with greater dysmorphology of the mesencephalic tectum were most likely to have nystagmus. Higher grades of nystagmus were associated with Type III tectal beaking.
We have demonstrated that there is a correlation between the presence and severity of nystagmus and the severity of tectal beaking and have quantified this association in patients with the Chiari II malformation. This information may act as a guide in predicting which children with the Chiari II malformation will have significant nystagmus. These data may aid the clinician in the interpretation of oculomotor abnormalities in this cohort of patients.
顶盖喙状畸形是脊髓脊膜膨出患儿的常见表现。医学文献中缺乏对该表现的有无与正常或异常眼球运动之间相关性的定量研究。
我们对多学科脊柱裂诊所的50名儿童进行了前瞻性评估。观察每个儿童在静息状态或眼球运动时是否存在眼球震颤。然后将MRI上眼球震颤的分级与顶盖喙状畸形的严重程度（I - III型）进行相关性分析。
绝大多数中脑顶盖形态异常更严重的儿童最有可能出现眼球震颤。较高等级的眼球震颤与III型顶盖喙状畸形相关。
我们已经证明眼球震颤的存在和严重程度与顶盖喙状畸形的严重程度之间存在相关性，并对Chiari II型畸形患者的这种关联进行了量化。这些信息可作为预测哪些Chiari II型畸形儿童会出现明显眼球震颤的指南。这些数据可能有助于临床医生解释该组患者的眼球运动异常情况。
