儿童多发性海绵状瘤同时幕上和幕下出血的外科治疗

G. Tyagi, Abhay Sikaria, G. Birua, M. Beniwal, Dwarakanath Srinivas
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引用次数: 0

摘要

多发性颅内海绵状瘤是罕见的,主要发生在家族性病例中。到目前为止,只有四例病例报告了同时出现两个或多个病变破裂的临床表现。一名15岁男孩同时出现右额和上朱部血肿并伴有脑积水。患者接受了脑室-腹腔分流术,他的磁共振成像(MRI)显示上述位置有多处海绵状瘤出血。患者接受了枕下中线开颅术和海绵状瘤切除术。幕上病变留在原位,而不是体积小、没有癫痫发作史、肿块效应或其他神经系统缺陷。患者手术后恢复良好,躯干共济失调明显改善。在随访中,他仍然没有幕上病变的症状。海绵状瘤应被视为多发性实质内出血的鉴别诊断,尤其是在儿科患者中。手术治疗应根据病变部位、周围软组织的清晰度、肿块效应和再次破裂的风险进行合理化。由于多发性同时出血的罕见性,多发性海绵体瘤的治疗仍然存在争议。可以用核磁共振成像对患者亲属进行筛查,以排除这种疾病的家族形式。对此类患者必须进行严格的临床和放射学随访。
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Surgical management of simultaneous supra- and infratentorial hemorrhages in a pediatric patient with multiple cavernomas
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.
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