Di Rocco C, Iannelli A, Marchese E
Institute of Neurosurgery, Catholic University, Rome, Italy.
Pediatr Neurosurg. 1995;23(3):115-21. doi: 10.1159/000120947.
Between 1980 and 1992, 10 children affected by tuberous sclerosis and intraventricular subependymal giant cell astrocytomas were surgically treated at the Institute of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University of Rome. Nine patients presented with signs and/or symptoms of intracranial hypertension; in all of them the neuroradiological investigations demonstrated the presence of a space-occupying lesion in the region of the foramen of Monro with secondary ventricular dilation. In the remaining patient, a 5-month-old male infant, an intraventricular mass was discovered by means of an ultrasound examination performed after the first epileptic fit. Three patients underwent a ventriculoperitoneal CSF shunt as first surgical procedure; in 2 of them it was subsequently necessary to remove the intraventricular tumor due to the frequent occlusion of the CSF shunt device. Seven subjects underwent the direct surgical excision of the lesion. In all of them the procedure resulted in the control of the associated hydrocephalus. On the basis of such an experience, the authors conclude that the surgical removal of the intraventricular tumors in patients with tuberous sclerosis and hydrocephalus is the most appropriate treatment. In fact, in the series considered here, the removal of the tumor was not accompanied by significant morbidity, and was followed by improvement in clinical conditions. In particular, in cases in whom the occurrence of hydrocephalus was associated with a worsening in the seizure disorder, the tumor removal and the correction of intracranial hypertension was followed by a significant reduction in frequency or even by the disappearance of the seizures. However, in no case presenting with mental impairment was a significant improvement observed in mental performances as a consequence of the surgical treatment.
1980年至1992年间，罗马天主教大学儿科神经外科神经外科研究所对10例患有结节性硬化症和脑室内室管膜下巨细胞星形细胞瘤的儿童进行了手术治疗。9例患者出现颅内高压的体征和/或症状；所有患者的神经放射学检查均显示Monro孔区域存在占位性病变，并伴有继发性脑室扩张。在其余1例患者中，一名5个月大的男婴在首次癫痫发作后通过超声检查发现了脑室内肿块。3例患者首次手术采用脑室腹腔脑脊液分流术；其中2例由于脑脊液分流装置频繁堵塞，随后有必要切除脑室内肿瘤。7例患者接受了病变的直接手术切除。所有患者术后脑积水均得到控制。基于这一经验，作者得出结论，对于患有结节性硬化症和脑积水的患者，手术切除脑室内肿瘤是最合适的治疗方法。事实上，在本文所讨论的病例系列中，肿瘤切除并未伴随明显的并发症，且术后临床状况有所改善。特别是，在脑积水的发生与癫痫发作加重相关的病例中，肿瘤切除和颅内高压的纠正后，癫痫发作频率显著降低甚至消失。然而，在任何存在智力障碍的病例中，均未观察到手术治疗后智力表现有显著改善。
