Tuft Mia, Berger Ylva Østby, Marthinsen Pål Bache, Due-Tønnessen Bernt Johan, Frič Radek
National Centre for Rare Epilepsy-related Disorders, Oslo University Hospital, P.O. Box 0495, N-0424, Oslo, Norway.
Institute of Psychology, University of Oslo, Oslo, Norway.
Childs Nerv Syst. 2025 Mar 4;41(1):126. doi: 10.1007/s00381-025-06779-4.
Subependymal giant cell astrocytomas (SEGA) are present in patients with tuberous sclerosis complex (TSC), occasionally requiring surgical removal. The study aimed to analyze the results from our series of children undergoing surgery for SEGA.
We retrospectively identified children with TSC undergoing resection of SEGA at Oslo University Hospital between 1982 and 2016. Patient charts, radiological images, epilepsy, and neuropsychological reports were reviewed.
Out of 208 patients with TSC, 18 (9%) underwent resection of SEGA. Due to missing data, we could only analyze results from 14 surgeries in 11 children (median age 6 years, range 0-19; male/female ratio 2.7:1). The tumours were bilateral in four (36%) patients. The tumour diameter was a median of 19 mm (10-104 mm). The surgical approach was transcortical in eight (57%) and transcallosal in six surgeries (43%). Gross total resection was achieved in 12 (86%) of surgeries. There was no mortality or major morbidity related to surgery except for one case of chronic subdural hematoma, but out of two patients with ventriculoperitoneal shunts, one developed shunt infection, and both experienced shunt failures during the follow-up. During the follow-up (median 11 years, range 1-21), three patients (27%) underwent repeated surgery. We could not document any significant impact of the surgery on patients' cognitive functioning or the grade of epilepsy.
Resection of SEGA in children with TSC was associated with a low complication rate. We could not document any impact of surgery on patients' cognitive functioning or grade of epilepsy. However, the neuropsychological data were limited in most cases. Neuropsychological assessment should be performed before the surgery and be a part of follow-up after surgery.
室管膜下巨细胞星形细胞瘤（SEGA）见于结节性硬化症（TSC）患者，偶尔需要手术切除。本研究旨在分析我们一系列接受SEGA手术的儿童的手术结果。
我们回顾性确定了1982年至2016年间在奥斯陆大学医院接受SEGA切除术的TSC患儿。查阅了患者病历、放射影像、癫痫及神经心理学报告。
在208例TSC患者中，18例（9%）接受了SEGA切除术。由于数据缺失，我们仅能分析11例儿童14次手术的结果（中位年龄6岁，范围0 - 19岁；男女比例2.7:1）。4例（36%）患者的肿瘤为双侧性。肿瘤直径中位值为19毫米（10 - 104毫米）。8例（57%）手术采用经皮质入路，6例（43%）采用经胼胝体入路。12例（8%）手术实现了全切除。除1例慢性硬膜下血肿外，无手术相关的死亡或严重并发症，但在2例接受脑室腹腔分流术的患者中，1例发生分流感染，且2例在随访期间均出现分流失败。在随访期间（中位时间11年，范围1 - 21年），3例（27%）患者接受了再次手术。我们无法证明手术对患者认知功能或癫痫发作程度有任何显著影响。
TSC患儿SEGA切除术的并发症发生率较低。我们无法证明手术对患者认知功能或癫痫发作程度有任何影响。然而，大多数情况下神经心理学数据有限。术前应进行神经心理学评估，并作为术后随访的一部分。
