Department of pediatric neurosurgery, University of Lille, Lille University Hospital, Lille, France.
Department of pediatric neurosurgery, University of Lille, Lille University Hospital, Lille, France.
Neurochirurgie. 2023 Sep;69(5):101473. doi: 10.1016/j.neuchi.2023.101473. Epub 2023 Jul 22.
Non-dysraphic intramedullary spinal cord lipomas (NDSCL) represent 1% of spinal cord tumors. They are less frequent than dysraphic spinal cord lipomas and clinical presentation is unspecific. There are no guidelines on surgical management.
We report three observations of NDSCL in children, focusing on the clinical presentation, surgical management and postoperative outcome.
The patients, one female and two males, aged from 5 months to 10 years presented with neurological deterioration, pain, spinal rigidity and in two cases, a subcutaneous mass. Spinal MRI found intradural lipomas without spina bifida, located in the cervico-thoracic area in all cases. The lipoma extended to the medulla oblongata in two cases and was in the lumbar region in the third. These lipomas were massive, requiring decompression surgery. Surgery confirmed the lipoma to be subpial. We performed debulking of the lipoma without attempting total resection, and with or without dural plasty and laminoplasty, followed by minerva cast in two cases, and avoidance of standing in the youngest. Satisfactory recovery occurred in all three cases. After a follow-up between 4 months and 9 years, the outcome was favorable in all cases, and no patient presented with secondary spinal deformation or lipoma progression.
NDSCL is a rare entity, which often manifests with progressive pain and neurological deficits. In our experience, partial resection with or without dural plasty and laminoplasty has been associated with satisfactory postoperative outcomes and no recurrence of symptoms. We should be attentive to the risk of postoperative spinal deformity in these young patients.
非脊膜脊髓内脂肪瘤(NDSCL)占脊髓肿瘤的 1%。它们比脊膜脊髓脂肪瘤少见,临床表现不特异。目前尚无关于手术治疗的指南。
我们报告了 3 例儿童 NDSCL 的病例,重点介绍了临床表现、手术治疗和术后结果。
3 例患者为 1 女 2 男,年龄 5 个月至 10 岁,表现为神经功能恶化、疼痛、脊柱僵硬,其中 2 例有皮下肿块。脊髓 MRI 发现无脊膜裂的硬脊膜内脂肪瘤,均位于颈胸段。2 例脂肪瘤延伸至延髓,第 3 例位于腰椎。这些脂肪瘤巨大,需要减压手术。手术证实脂肪瘤位于软膜下。我们进行了脂肪瘤的部分切除术,未尝试完全切除,同时行或不行硬脑膜修补和椎板成形术,随后在 2 例中使用 Minerva 铸型,在最小的患者中避免站立。3 例患者均获得满意的恢复。随访 4 个月至 9 年,所有患者的结果均良好,无患者出现继发性脊柱畸形或脂肪瘤进展。
NDSCL 是一种罕见的疾病,常表现为进行性疼痛和神经功能缺损。根据我们的经验,部分切除术结合或不结合硬脑膜修补和椎板成形术可获得满意的术后结果,且无症状复发。对于这些年轻患者,应注意术后脊柱畸形的风险。