Makino Keishi, Tajiri Seiji, Mori Ryosuke, Takada Akira, Hitoshi Yasuyuki, Mukasa Akitake
Department of Neurosurgery, Kumamoto City Hospital, Kumamoto, Japan.
Department of Neurosurgery, Kumamoto Rosai Hospital, Yatsushiro, Japan.
Surg Neurol Int. 2025 Mar 14;16:87. doi: 10.25259/SNI_1094_2024. eCollection 2025.
Limited dorsal myeloschisis (LDM) is a condition in which the separation of the neuroectoderm from the cutaneous ectoderm during primary neural tube formation results in localized disjuncture, causing a continuous cord-like connection and spinal cord tethering. We reported a case of cervical LDM with a wart-like cutaneous appendage that was treated with excision after long-term follow-up.
The patient was an 18-year-old girl. A wart-like cutaneous appendage was noted over the nape of the neck since birth. Computed tomography showed spina bifida in the cervical and thoracic spines, and spinal magnetic resonance imaging (MRI) showed a cervical skin lesion and an enlarged dural sac in the dorsal thoracic spinal cord. At 18 years of age, the patient occasionally experienced numbness in her left hand and was referred to our outpatient clinic due to a new high signal intensity in the dorsal cervical spinal cord on a T2-weighted MRI. The MRI showed that a cord-like object was continuous intradural and dorsal to the spinal cord from a cutaneous lesion in the median cervical region, with a high signal in the same region. Symptomatic cervical spinal cord tethering due to a cord-like material was diagnosed, and the patient underwent resection. During surgery, the tract was removed from the cutaneous lesion into the dura mater as a single mass and untethered in the dorsal spinal cord. The histological diagnosis was a pseudo-dermal sinus tract with no luminal structures or neural tissue present, as the cord-like substance was connective tissue containing small blood vessels. Based on the neuroimaging and pathological findings, the patient was diagnosed with cervical LDM. Neurological symptoms improved postoperatively.
Herein, we reported a case of cervical LDM that was treated after long-term follow-up. The patient's symptoms improved immediately after surgery. Cervical LDMs are rare, and the timing of surgery for LDM should be considered according to the patient's condition.
局限性脊髓背侧裂开(LDM)是一种在原始神经管形成过程中神经外胚层与皮肤外胚层分离导致局部脱节的疾病,会造成连续性索状连接和脊髓栓系。我们报告了一例伴有疣状皮肤附属器的颈段LDM病例,经过长期随访后进行了切除治疗。
患者为一名18岁女孩。自出生以来,其颈部后方可见一个疣状皮肤附属器。计算机断层扫描显示颈椎和胸椎存在脊柱裂,脊髓磁共振成像(MRI)显示颈部皮肤病变以及胸段脊髓背侧硬脊膜囊扩大。18岁时,患者偶尔感到左手麻木,因T2加权MRI显示颈段脊髓背侧出现新的高信号强度而转诊至我们的门诊。MRI显示,一个索状物体从颈中部的皮肤病变处硬膜内且在脊髓背侧连续,同一区域呈高信号。诊断为因索状物质导致的有症状性颈段脊髓栓系,患者接受了切除术。手术过程中,将该条索从皮肤病变处完整切除至硬脑膜,解除了脊髓背侧的栓系。组织学诊断为假性皮样窦道,不存在管腔结构或神经组织,因为该索状物质为含有小血管的结缔组织。根据神经影像学和病理学检查结果,患者被诊断为颈段LDM。术后神经症状有所改善。
在此,我们报告了一例经过长期随访后接受治疗的颈段LDM病例。患者术后症状立即改善。颈段LDM较为罕见,应根据患者情况考虑LDM的手术时机。