Bustamante-Vidales Jesús Carlos, Kleriga-Grossgere Enrique, Zambito-Brondo Gerardo Francisco, García-Moreno Carla María
Instituto Mexicano de Neurociencias, Hospital Angeles Lomas, Huixquilucan, Estado de México, Mexico.
Cir Cir. 2010 May-Jun;78(3):251-4.
Idiopathic transdural spinal cord herniation (ISCH) is a rare entity with postsurgical and post-trauma forms. ISCH is often omited in the preoperative evaluation. It often affects the thoracic segment and presents clinically as a rare cause of progressive myelopathy or Brown-Séquard syndrome, whose diagnosis is established by magnetic resonance imaging (MRI). We report on this rare entity due to its difficult diagnosis, making optimal management difficult.
We present the cases of two patients with ISCH who were misdiagnosed and operated on in other spinal segments without reaching an accurate diagnosis. In our institution, patients with clinical suspicion were evaluated by imaging studies in order to rule out other pathologies. Laminectomy was performed on the involved levels, reducing herniation and with the placement of a synthetic spinal patch to the duramater.
ISCH has been attributed to congenital weakness of the duramater or the dural ventral duplication with herniation through the inner layer due to continuous pressure from cerebrospinal fluid that pushes the marrow out of the subdural space. It is estimated that presurgical diagnosis is done only in one third of the cases, confirmed by MRI. Surgery is performed on patients with symptom progression. Surveillance in those patients with mild symptoms is recommended. Treatment may allow recovery of the neurological deficit, improving motor affection in 80% of patients and sensory affection in 35%.
特发性经硬膜脊髓疝（ISCH）是一种罕见疾病，有术后和创伤后两种类型。ISCH在术前评估中常被遗漏。它常累及胸段，临床上表现为进行性脊髓病或布朗 - 塞卡尔综合征的罕见病因，其诊断通过磁共振成像（MRI）确定。由于其诊断困难，导致最佳治疗难以实施，我们特此报告这一罕见疾病。
我们介绍了两名ISCH患者的病例，他们被误诊并在其他脊髓节段进行了手术，未能得到准确诊断。在我们机构，对临床怀疑的患者进行了影像学检查，以排除其他病变。对受累节段进行了椎板切除术，减轻了疝出，并在硬脑膜上放置了合成脊髓补片。
ISCH被认为是由于硬脑膜先天性薄弱或硬脑膜腹侧重复，在脑脊液持续压力作用下，髓质通过内层疝出，导致硬膜下间隙增宽。据估计，仅三分之一的病例在术前通过MRI确诊。对症状进展的患者进行手术治疗。建议对症状轻微的患者进行监测。治疗可能使神经功能缺损恢复，80%的患者运动功能得到改善，35%的患者感觉功能得到改善。
