Medical School of Nankai University, No. 94, Weijin Road, Naikai District, Tianjin, 300071, People's Republic of China.
Department of Neurosurgery, Chinese PLA General Hospital, No. 28, Fuxing Road, Haidian District, Beijing, 100853, People's Republic of China.
Childs Nerv Syst. 2021 Jan;37(1):235-242. doi: 10.1007/s00381-020-04700-9. Epub 2020 Jun 26.
Pediatric intramedullary spinal cord cavernous malformation (ISCM) is a rare vascular disease with unclear natural history and long-term outcomes. We aim to determine the demographics, hemorrhagic risk, and long-term outcomes of this rare entity.
A retrospective review of clinical data and treatment outcomes of pediatric patients treated with ISCM in our institution from 3/2000 to 3/2017 was conducted. In addition, we performed a systematic review of the literature on pediatric ISCM.
Eighteen consecutive pediatric patients were included, with an average age of 12.9 ± 4.7 years (range: 4-18 years) and 66.7% being male. Locations were equally distributed in cervical and thoracic segments, with mean extension of 1.3 ± 0.7 segments. Clinical manifestation included extremity weakness (n = 15, 83.3%), pain (n = 10, 55.6%), sensory disorders (n = 8, 44.4%), sphincter disturbance (n = 6, 33.3%), muscular atrophy (n = 3, 16.7%), and spinal deformity (n = 1, 5.6%). Most patients presented with acute symptoms (n = 11, 61.1%), and 7 (38.9%) of them had severe neurological deficits. The annual retrospective hemorrhagic risk was 7.7 per patient-year. Two patients received conservative management, with one improved neurologically and the other remained unchanged. Total resection was achieved in 12 (75%) of the 16 surgical cases, with 8 patients (50%) improved their clinical outcomes, 7 patients (43.8%) remained unchanged, and 1 (6.3%) worsened. During follow-up, one patient had relapse of ISCM.
Pediatric ISCM appears to have higher hemorrhage risk than their adult counterparts, and they can benefit from surgery whether in the acute phase of neurological deterioration or after clinical recuperation.
小儿脊髓髓内海绵状血管畸形（ISCM）是一种罕见的血管疾病，其自然病史和长期预后尚不清楚。本研究旨在确定该罕见实体的人口统计学、出血风险和长期预后。
对 2000 年 3 月至 2017 年 3 月期间在我院接受治疗的小儿 ISCM 患者的临床资料和治疗结果进行回顾性分析。此外，我们还对小儿 ISCM 的文献进行了系统回顾。
共纳入 18 例连续的小儿患者，平均年龄为 12.9±4.7 岁（范围：4-18 岁），其中 66.7%为男性。病变部位在颈椎和胸椎段分布均匀，平均扩展 1.3±0.7 个节段。临床表现包括肢体无力（n=15，83.3%）、疼痛（n=10，55.6%）、感觉障碍（n=8，44.4%）、括约肌功能障碍（n=6，33.3%）、肌肉萎缩（n=3，16.7%）和脊柱畸形（n=1，5.6%）。大多数患者表现为急性症状（n=11，61.1%），7 例（38.9%）患者存在严重神经功能缺损。回顾性分析显示，每年的出血风险为 7.7 例/患者年。2 例患者接受了保守治疗，1 例患者神经功能改善，1 例患者无变化。16 例手术患者中，12 例（75%）实现了肿瘤全切除，其中 8 例（50%）患者的临床结局得到改善，7 例（43.8%）患者无变化，1 例（6.3%）患者病情恶化。随访期间，1 例患者复发 ISCM。
小儿 ISCM 的出血风险似乎高于成人，无论是在神经功能恶化的急性期还是在临床恢复后，手术治疗都可能使患者受益。
