Morioka Takato, Murakami Nobuya, Suzuki Satoshi O, Nakamura Ryoko, Mizoguchi Masahiro
Department of Neurosurgery, Fukuoka Children's Hospital, Fukuoka, Fukuoka, Japan.
Department of Neuropathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Fukuoka, Japan.
NMC Case Rep J. 2021 Apr 2;8(1):51-55. doi: 10.2176/nmccrj.cr.2020-0073. eCollection 2021 Apr.
Subpial lipomas, which are also known as nondysraphic intramedullary spinal cord lipomas, are not associated with spinal dysraphism resulting from the failed primary neurulation. Retained medullary cord (RMC) is a newly defined entity of closed spinal dysraphism that originates from the late arrest of secondary neurulation. We treated a 6-year-old boy presented with myoclonus of the lower limbs, who had subpial lipoma at the lumbar cord, just rostral to the low-lying conus, which was tethered by a cord-like structure (C-LS) continuous from the conus and extending to the dural cul-de-sac. Following cord untethering from C-LS and minimal debulking of the lipoma, the myoclonus was improved. Histological examination of C-LS revealed a large central canal-like structure in the neuroglial core and the diagnosis of RMC was made. Subpial lipomas can be incidentally coexistent with spinal dysraphism resulting from the failed secondary neurulation, such as RMC.
软膜下脂肪瘤，也被称为非神经管闭合不全性脊髓内脂肪瘤，与原发性神经管形成失败导致的脊柱神经管闭合不全无关。残留脊髓（RMC）是一种新定义的闭合性脊柱神经管闭合不全实体，起源于继发性神经管形成的晚期停滞。我们治疗了一名6岁出现下肢肌阵挛的男孩，其腰髓处有软膜下脂肪瘤，恰好在低位圆锥头侧，该脂肪瘤被一个从圆锥延续并延伸至硬膜盲端的索状结构（C-LS）束缚。在将脊髓从C-LS松解并对脂肪瘤进行最小程度的减容后，肌阵挛得到改善。对C-LS的组织学检查显示神经胶质核心中有一个大的中央管样结构，从而做出了RMC的诊断。软膜下脂肪瘤可偶然与继发性神经管形成失败导致的脊柱神经管闭合不全同时存在，如RMC。
