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圆锥内髓内婴儿型血管瘤伴有限性背侧脊髓裂:病例报告

Limited dorsal myeloschisis associated with intramedullary infantile hemangioma in the conus medullaris: illustrative case.

作者信息

Nakanishi Yoko, Kunihiro Noritsugu, Umaba Ryoko, Matsusaka Yasuhiro, Inoue Takeshi, Sakamoto Hiroaki

机构信息

Departments of1Pediatric Neurosurgery, and.

2Department of Neurological Surgery, Wakayama Medical University, Wakayama, Japan.

出版信息

J Neurosurg Case Lessons. 2023 Apr 24;5(17). doi: 10.3171/CASE22359.

Abstract

BACKGROUND

Limited dorsal myeloschisis (LDM) and intramedullary infantile hemangioma rarely coexist in the spinal cord.

OBSERVATIONS

The authors describe the case of a 3-month-old girl who, despite lacking neurological symptoms or signs, had a cigarette burn-like mark at the lumbosacral area and skin dimpling in the gluteal area. Magnetic resonance imaging showed a low-set conus due to a thickened filum and an abnormal subcutaneous stalk connected to the conus medullaris. In combination with the skin lesions, these findings strongly implied nonsaccular-type LDM. An intramedullary mass in the conus medullaris was also shown on magnetic resonance imaging and was homogenously enhanced with isointensity on T1- and T2-weighted images. We prophylactically untethered the spinal cord and partially removed the intramedullary mass, which had no clear borders, for a safe surgical dissection. Histologically, the intramedullary mass was an infantile hemangioma, and the subcutaneous stalk was a lesion associated with LDM. The patient remained neurologically intact after surgery, and then 2 years later, there was spontaneous regression of the residual tumor.

LESSONS

Although rare, nonsaccular type LDM may appear concurrently with intramedullary infantile hemangioma at the conus medullaris. The authors present a possible mechanism behind this concurrent presentation in the same area.

摘要

背景

有限性背侧脊髓纵裂(LDM)与髓内婴儿型血管瘤在脊髓中很少同时存在。

观察结果

作者描述了一名3个月大女孩的病例,尽管她没有神经症状或体征,但在腰骶部有类似香烟烫伤的痕迹,臀部有皮肤凹陷。磁共振成像显示由于终丝增厚导致圆锥低位,以及一个与脊髓圆锥相连的异常皮下蒂。结合皮肤病变,这些发现强烈提示为非囊状型LDM。磁共振成像还显示脊髓圆锥内有一个髓内肿块,在T1加权和T2加权图像上呈等强度均匀强化。为了安全地进行手术切除,我们预防性地松解了脊髓并部分切除了边界不清的髓内肿块。组织学检查显示,髓内肿块为婴儿型血管瘤,皮下蒂为与LDM相关的病变。患者术后神经功能保持完好,2年后残余肿瘤自发消退。

经验教训

尽管罕见,但非囊状型LDM可能与脊髓圆锥内的髓内婴儿型血管瘤同时出现。作者提出了在同一区域这种同时出现情况背后的一种可能机制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05e3/10550677/b9769c0b4202/CASE22359f1.jpg

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