Department of Neurosurgery, Juntendo University Shizuoka Hospital, Izunokuni City, Shizuoka, Japan.
Department of Pediatrics, Juntendo University Shizuoka Hospital, Izunokuni City, Shizuoka, Japan.
Childs Nerv Syst. 2023 Aug;39(8):2045-2051. doi: 10.1007/s00381-023-06012-0. Epub 2023 Jul 1.
Diffuse villous hyperplasia of the choroid plexus (DVHCP) and choroid plexus papilloma (CPP) are rare benign tumors usually diagnosed as a result of progressive hydrocephalus, especially in childhood. We present the case of a Japanese boy diagnosed with progressive hydrocephalus due to DVHCP.
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.
脉络丛绒毛状增生（DVHCP）和脉络丛乳头状瘤（CPP）是罕见的良性肿瘤，通常由于进行性脑积水而被诊断出来，尤其是在儿童中。我们报告了一例因 DVHCP 导致进行性脑积水的日本男孩病例。
病例：一名 2 岁 3 个月大的日本男孩被发现运动发育迟缓（相当于 1 岁 2 个月），头围增大至 51cm，超出+1.5 个标准差，前囟未完全闭合。磁共振成像（MRI）显示双侧脉络丛呈小叶状肿大，从三角区延伸至侧脑室体部和下角。行内镜下脉络丛电凝术以降低脑脊液形成率。
病理和临床均诊断为 DVHCP。术后，患者无并发症进展，如脑脊液漏。尽管脑室扩大持续存在，但前囟凹陷，头围停止扩张。
文献中报道的双侧 DVHCP 和 CPP 病例较少。我们遇到了一例使用微创内镜技术治疗因 DVHCP 引起的脑积水的有效脉络丛电凝病例。这也表明了 DVHCP 与 9p 染色体获得之间的关联。
