Akutsu Nobuyuki, Koyama Junji, Kawamura Atsufumi, Nagashima Tatsuya, Taniguchi Masaaki, Kohmura Eiji
Department of Neurosurgery, Hyogo Prefectural Kobe Children's Hospital, 1-6-7 Minatojima-minamimachi, Chuo-ku, Kobe, 650-0047, Japan.
Department of Neurosurgery, Kobe University Graduate School of Medicine, Kobe, Japan.
Childs Nerv Syst. 2018 May;34(5):991-994. doi: 10.1007/s00381-018-3719-8. Epub 2018 Jan 11.
There are very few reports in the literature associating in hydrocephalus in osteopetrosis. As a complication of shunt procedure, there are two reports on shunt malfunction due to osseous overgrowth at the burr hole in patients with osteopetrosis. We herein report a case of osteopetrosis with hydrocephalus that was successfully treated with endoscopic third ventriculostomy (ETV).
At 5 months of age, a male patient presented with developmental delay. Head computed tomography (CT) demonstrated triventricular hydrocephalus with a cerebellar tonsillar herniation. At 7 months of age, he underwent suboccipital decompression with decompression of the foramen magnum. The hydrocephalus did not improve postoperatively, and the patient was transferred to our hospital. At 12 months of age, the hydrocephalus was successfully treated with ETV. The postoperative period was uneventful. Postoperative CT demonstrated an improvement in the ventricle size.
The etiology of hydrocephalus in osteopetrosis is not completely understood; however, there have been several reports in which ETV was effective. ETV should be considered the treatment of choice for hydrocephalus in osteopetrosis, as it avoids the characteristic shunt complications that can occur in patients with osteopetrosis.
文献中很少有关于骨硬化症合并脑积水的报道。作为分流手术的一种并发症，有两篇关于骨硬化症患者因骨孔处骨质过度生长导致分流故障的报道。我们在此报告一例骨硬化症合并脑积水患者，经内镜下第三脑室造瘘术（ETV）成功治疗。
一名男性患者5个月大时出现发育迟缓。头部计算机断层扫描（CT）显示三脑室脑积水伴小脑扁桃体疝。7个月大时，他接受了枕下减压术及枕大孔减压术。术后脑积水未改善，患者转至我院。12个月大时，ETV成功治疗了脑积水。术后过程顺利。术后CT显示脑室大小有所改善。
骨硬化症中脑积水的病因尚未完全明确；然而，有几篇报道表明ETV是有效的。ETV应被视为骨硬化症患者脑积水的首选治疗方法，因为它避免了骨硬化症患者可能出现的典型分流并发症。
