Tyagi Gaurav, Sikaria Abhay, Birua Gyani Jail Singh, Beniwal Manish, Srinivas Dwarakanath
Department of Neurosurgery, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, India.
J Cerebrovasc Endovasc Neurosurg. 2022 Sep;24(3):262-266. doi: 10.7461/jcen.2022.E2021.08.001. Epub 2022 Feb 28.
Multiple intracranial cavernomas are rare and occur mostly in familial cases. Clinical presentation with simultaneous rupture of two or more lesions has only been reported in four cases to date. A 15-year-old boy presented with simultaneous right frontal and superior vermian hematomas with hydrocephalus. The patient underwent a ventriculoperitoneal shunt, and his magnetic resonance imaging (MRI) revealed multiple cavernomas with bleed in the above-mentioned locations. The patient underwent a midline suboccipital craniotomy and excision of the cavernoma. The supratentorial lesions were left in situ in lieu of small size, no history of seizures, mass effect, or other neurological deficits. The patient recovered well from surgery with significant improvement in truncal ataxia. He remained asymptomatic for supratentorial lesions at follow-up. Cavernomas should be considered as differential diagnoses in cases of multiple intraparenchymal hemorrhages, especially in pediatric patients. The surgical management should be rationalized based on the lesion location, the eloquence of the surrounding parenchyma, mass effect, and the risks of re-rupture. Due to the rarity of multiple simultaneous hemorrhages, the management of multiple cavernomas remains controversial. The patient's relatives can be screened with MRI to rule out the familial form of the disease. Strict clinical and radiological follow-up is a must in such patients.
多发性颅内海绵状血管瘤较为罕见,多发生于家族性病例。迄今为止,仅有4例报告了两个或更多病灶同时破裂的临床表现。一名15岁男孩出现右侧额叶和小脑蚓部上份同时出血并伴有脑积水。患者接受了脑室腹腔分流术,其磁共振成像(MRI)显示在上述部位有多个伴出血的海绵状血管瘤。患者接受了枕下中线开颅术并切除了海绵状血管瘤。幕上病灶因体积小、无癫痫病史、无占位效应或其他神经功能缺损而未予处理。患者术后恢复良好,躯干共济失调明显改善。随访时幕上病灶无症状。对于多发性脑实质内出血病例,尤其是儿科患者,应考虑海绵状血管瘤作为鉴别诊断。手术治疗应根据病灶位置、周围实质的功能区、占位效应和再破裂风险进行合理规划。由于多个病灶同时出血的情况罕见,多发性海绵状血管瘤的治疗仍存在争议。可对患者亲属进行MRI筛查以排除家族性疾病形式。对此类患者必须进行严格的临床和影像学随访。