PDF(Pubmed)
Abstract：
BACKGROUND: Unilateral cranial nerve (CN) VI, or abducens nerve, palsy is rare in children and has not been reported in association with Chiari malformation type 1 (CM1) in the absence of other classic CM1 symptoms.
METHODS: A 3-year-old male presented with acute incomitant esotropia consistent with a unilateral, left CN VI palsy and no additional neurological symptoms. Imaging demonstrated CM1 without hydrocephalus or papilledema, as well as an anterior inferior cerebellar artery (AICA) vessel loop in the immediate vicinity of the left abducens nerve. Given the high risk of a skull base approach for direct microvascular decompression of the abducens nerve and the absence of other classic Chiari symptoms, the patient was initially observed. However, as his palsy progressed, he underwent posterior fossa decompression with duraplasty (PFDD), with the aim of restoring global cerebrospinal fluid dynamics and decreasing possible AICA compression of the left abducens nerve. Postoperatively, his symptoms completely resolved.
CONCLUSIONS: In this first reported case of CM1 presenting as a unilateral abducens palsy in a young child, possibly caused by neurovascular compression, the patient\'s symptoms resolved after indirect surgical decompression via PFDD.
METHODS: A 3-year-old male presented with acute incomitant esotropia consistent with a unilateral, left CN VI palsy and no additional neurological symptoms. Imaging demonstrated CM1 without hydrocephalus or papilledema, as well as an anterior inferior cerebellar artery (AICA) vessel loop in the immediate vicinity of the left abducens nerve. Given the high risk of a skull base approach for direct microvascular decompression of the abducens nerve and the absence of other classic Chiari symptoms, the patient was initially observed. However, as his palsy progressed, he underwent posterior fossa decompression with duraplasty (PFDD), with the aim of restoring global cerebrospinal fluid dynamics and decreasing possible AICA compression of the left abducens nerve. Postoperatively, his symptoms completely resolved.
CONCLUSIONS: In this first reported case of CM1 presenting as a unilateral abducens palsy in a young child, possibly caused by neurovascular compression, the patient\'s symptoms resolved after indirect surgical decompression via PFDD.
摘要：
背景：单侧颅神经（CN）VI，或者外展神经，麻痹在儿童中很少见，在没有其他经典CM1症状的情况下，尚未报告与Chiari畸形1型（CM1）相关。
方法：一名3岁男性出现急性相伴性内斜视，左侧CNVI麻痹，无其他神经系统症状。影像学显示CM1无脑积水或乳头水肿，以及左外展神经附近的小脑前下动脉（AICA）血管环。鉴于颅底入路直接进行展神经微血管减压的风险很高，并且没有其他经典的Chiari症状，最初观察到患者。然而,随着他的麻痹进展,他接受了后颅窝减压和硬脑膜成形术（PFDD），目的是恢复整体脑脊液动力学并减少对左展神经的AICA压迫。术后,他的症状完全缓解。
结论：在第一例CM1病例中，表现为幼儿单侧外展麻痹，可能是神经血管压迫造成的,通过PFDD间接手术减压后，患者的症状得以缓解。
方法：一名3岁男性出现急性相伴性内斜视，左侧CNVI麻痹，无其他神经系统症状。影像学显示CM1无脑积水或乳头水肿，以及左外展神经附近的小脑前下动脉（AICA）血管环。鉴于颅底入路直接进行展神经微血管减压的风险很高，并且没有其他经典的Chiari症状，最初观察到患者。然而,随着他的麻痹进展,他接受了后颅窝减压和硬脑膜成形术（PFDD），目的是恢复整体脑脊液动力学并减少对左展神经的AICA压迫。术后,他的症状完全缓解。
结论：在第一例CM1病例中，表现为幼儿单侧外展麻痹，可能是神经血管压迫造成的,通过PFDD间接手术减压后，患者的症状得以缓解。
