Case: A 2-year and 3-month-old Japanese boy was found to have delayed motor development (equivalent to 1 year and 2 months old), an enlarged head circumference of 51 cm within + 1.5 standard deviation (S.D.), and incomplete closure of the anterior fontanel. The magnetic resonance imaging (MRI) showed lobular enlargement of the bilateral choroid plexuses extending from the trigone to the body and inferior horn of the lateral ventricle. The endoscopic choroid plexus coagulation surgery was performed to reduce the CSF formation rate.
DVHCP was diagnosed both pathologically and clinically. Postoperatively, the patient progressed without complications, such as cerebrospinal fluid leakage. Although ventricular enlargement persisted, the anterior fontanel recessed, and the expansion of the head circumference stopped.
Few cases of bilateral DVHCP and CPP have been reported in the literature. We encountered a case in which effective choroid plexus coagulation was performed for hydrocephalus due to DVHCP using less invasive endoscopic technique. It also represented an association between DVHCP and the gain of chromosome 9p.
方法:案例:发现一名2岁3个月大的日本男孩运动发育延迟(相当于1岁2个月大),在+1.5标准偏差(S.D.)内51厘米的扩大头围,前font门不完全闭合。磁共振成像(MRI)显示双侧脉络丛的小叶增大,从三角区延伸到身体和侧脑室的下角。内镜下进行脉络丛凝固手术以降低脑脊液形成率。
结果:DVHCP在病理和临床上均得到诊断。术后,患者进展无并发症,如脑脊液漏。尽管心室扩大持续存在,前fontanel凹陷,头围的扩张停止了。
结论:文献中报道了很少的双侧DVHCP和CPP病例。我们遇到了一个案例,其中使用侵入性较小的内窥镜技术对DVHCP引起的脑积水进行了有效的脉络丛凝固术。它还代表了DVHCP与染色体9p的获得之间的关联。