Miyamoto Junichi, Tatsuzawa Kazunori, Sasajima Hiroyasu, Mineura Katsuyoshi
Department of Neurosurgery, Kyoto Prefectural University Graduate School of Medicine, Kyoto, Japan.
Neurol Med Chir (Tokyo). 2010;50(12):1116-8. doi: 10.2176/nmc.50.1116.
A 1-year 9-month-old girl presented with achondroplasia. Serial magnetic resonance (MR) imaging demonstrated mild compression of the medulla oblongata by the occipital bone, macrocrania, and progressive hydrocephalus. Cerebrospinal fluid (CSF) flow study using MR imaging clearly demonstrated CSF flow disturbance at the cervicospinal junction. Foramen magnum decompression was performed for her hydrocephalus and compressed medulla. Postoperative CSF flow study demonstrated improvement of CSF flow at the craniocervical junction. The patient has remained in a stable condition for 7 months postoperatively. Achondroplasia represents hydrocephalus or medullary compression caused by narrowed foramen magnum, which can result in sudden death in some infants. Surgical indications and methods for hydrocephalus combined with achondroplasia remain controversial because the natural history of the hydrocephalus has remained unclear. CSF flow study using MR imaging can provide useful information regarding the surgical indication and methods for the treatment of hydrocephalus combined with achondroplasia.
一名1岁9个月大的女童患软骨发育不全。系列磁共振成像显示枕骨对延髓有轻度压迫、巨头畸形和进行性脑积水。利用磁共振成像进行的脑脊液流动研究清楚地显示了颈髓交界处的脑脊液流动障碍。为治疗她的脑积水和受压延髓，实施了枕骨大孔减压术。术后脑脊液流动研究显示颅颈交界处的脑脊液流动有所改善。术后7个月患者病情一直稳定。软骨发育不全可导致因枕骨大孔狭窄引起的脑积水或延髓受压，这在一些婴儿中可能导致猝死。由于脑积水的自然病程尚不清楚，软骨发育不全合并脑积水的手术指征和方法仍存在争议。利用磁共振成像进行的脑脊液流动研究可为软骨发育不全合并脑积水的手术指征和治疗方法提供有用信息。
