Barth P G, de Vries L S, Nikkels P G J, Troost D
Department of Neuropathology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
Neuropediatrics. 2008 Feb;39(1):1-7. doi: 10.1055/s-2008-1077049.
We report a case of congenital brainstem disconnection including the second detailed autopsy. A full-term newborn presented with irreversible apnoea and died on the fifth day. MRI revealed disconnection of the brainstem. The autopsy included a series of transverse sections of the mesencephalon, medulla oblongata and bridging tissue fragments. A fragile tube walled by mature brainstem tissue could be reconstructed. It enveloped a cylinder of fluid within the ventral pons extending to the mesencephalon and the lower brainstem. The aqueduct was patent and outside the lesion. The basilar artery was represented by a tiny median vessel. The ventral and lateral parts of the posterior brainstem were surrounded by heterotopic glial tissue. The olivary nucleus was absent and the cerebellar dentate nucleus was dysplastic. Considering the maturity of the remaining parts of the pons, the onset of structural decline is likely to be close to the time of birth. Probable causes are progressively insufficient perfusion through an hypoplastic basilar artery, and obstructed venous drainage through an abnormal glial barrier surrounding the posterior brainstem. The morphological findings can be characterized as a syrinx, known from disorders in which brainstem or spinal cord are damaged by a combination of mechanical and circulatory factors.
我们报告一例先天性脑干离断病例，包括第二次详细尸检。一名足月儿出现不可逆呼吸暂停，于第五天死亡。磁共振成像（MRI）显示脑干离断。尸检包括一系列中脑、延髓及桥接组织碎片的横切面。可以重建一个由成熟脑干组织构成管壁的脆弱管道。它包裹着一个位于脑桥腹侧延伸至中脑和下脑干的充满液体的圆柱体。导水管通畅且位于病变之外。基底动脉由一条细小的正中血管代表。脑干后部的腹侧和外侧部分被异位神经胶质组织包围。橄榄核缺失，小脑齿状核发育异常。考虑到脑桥其余部分的成熟度，结构衰退的起始可能接近出生时间。可能的原因是通过发育不全的基底动脉灌注逐渐不足，以及通过围绕脑干后部的异常神经胶质屏障导致静脉引流受阻。形态学发现可被描述为一种空洞，这在脑干或脊髓因机械和循环因素共同受损的疾病中较为常见。
