Elbabaa Samer K, Riggs Angela D, Saad Ali G
Department of Neurosurgery, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, Arkansas 72202, USA.
J Neurosurg Pediatr. 2011 Jul;8(1):103-6. doi: 10.3171/2011.4.PEDS10308.
Tuberous sclerosis complex (TSC) is a genetic neurocutaneous disorder that commonly affects the CNS. The most commonly associated brain tumors include cortical tubers, subependymal nodules, and subependymal giant cell astrocytomas (SEGAs). The authors report an unusual case of recurrent meningitis due to a tuber-containing encephalocele via the posterior wall of the frontal sinus. An 11-year-old girl presented with a history of TSC and previous SEGA resection via interhemispheric approach. She presented twice within 4 months with classic bacterial meningitis. Cerebrospinal fluid cultures revealed Streptococcus pneumoniae. Computed tomography and MR imaging of the brain showed a right frontal sinus encephalocele via a posterior frontal sinus wall defect. Both episodes of meningitis were treated successfully with standard regimens of intravenous antibiotics. The neurosurgical service was consulted to discuss surgical options. Via a bicoronal incision, a right basal frontal craniotomy was performed. A large frontal encephalocele was encountered in the frontal sinus. The encephalocele was herniating through a bony defect of the posterior sinus wall. The encephalocele was ligated and resected followed by removing frontal sinus mucosa and complete cranialization of frontal sinus. Repair of the sinus floor was conducted with fat and pericranial grafts followed by CSF diversion via lumbar drain. Histopathology of the resected encephalocele showed a TSC tuber covered with respiratory (frontal sinus) mucosa. Tuber cells were diffusely positive for GFAP. The patient underwent follow-up for 2 years without evidence of recurrent meningitis or CSF rhinorrhea. This report demonstrates that frontal tubers of TSC can protrude into the frontal sinus as acquired encephaloceles and present with recurrent meningitis. To the authors' knowledge, recurrent meningitis is not known to coincide with TSC. Careful clinical and radiographic follow-up for frontal tubers in patients with TSC is recommended.
结节性硬化症（TSC）是一种常见影响中枢神经系统的遗传性神经皮肤疾病。最常相关的脑肿瘤包括皮质结节、室管膜下结节和室管膜下巨细胞星形细胞瘤（SEGAs）。作者报告了一例罕见病例，一名患有含结节性脑膨出的患者经额窦后壁反复发生脑膜炎。一名11岁女孩有TSC病史，之前通过半球间入路切除过SEGA。她在4个月内两次出现典型的细菌性脑膜炎。脑脊液培养显示为肺炎链球菌。脑部计算机断层扫描和磁共振成像显示通过额窦后壁缺损形成右额窦脑膨出。两次脑膜炎发作均通过静脉抗生素标准方案成功治疗。咨询神经外科以讨论手术方案。通过双冠状切口，进行了右基底额部开颅手术。在额窦中发现一个大的额部脑膨出。脑膨出通过窦后壁的骨缺损突出。结扎并切除脑膨出，随后切除额窦黏膜并对额窦进行完全颅骨化。用脂肪和颅骨膜移植物修复窦底，随后通过腰大池引流进行脑脊液分流。切除的脑膨出的组织病理学显示一个TSC结节被呼吸道（额窦）黏膜覆盖。结节细胞GFAP弥漫性阳性。患者接受了2年随访，没有复发性脑膜炎或脑脊液鼻漏的迹象。本报告表明，TSC的额叶结节可作为后天性脑膨出突入额窦，并表现为反复性脑膜炎。据作者所知，复发性脑膜炎与TSC并不相关。建议对TSC患者的额叶结节进行仔细的临床和影像学随访。
