CAJ | 학술논문

目的探讨延髓血管母细胞瘤(HB)临床特点和诊治策略,提高手术治愈率,减少并发症和死亡率.方法回顾性分析2002至2012年上海长征医院神经外科手术治疗的12例延髓血管母细胞瘤的临床表现、影像学特征、诊治经过和手术效果.结果主要症状为头痛、躯体麻木和肢体肌力下降.磁共振发现病变均为单发.位于延髓近脑桥1例,延髓中部7例,延颈交界4例；位于软膜下9例,髓内3例；3例有囊性变.5例行数字剪影血管造影(DSA),供血动脉主要为小脑后下动脉和小脑前下动脉分支,1例行术前栓塞.完整切除11例,1例仅行电灼.术后症状改善8例,加重2例,死亡2例.随访3个月～ 10年,McCormick神经功能Ⅰ～Ⅱ级,1例术后7年复发.结论位于延髓中下段髓内、囊性HB和中小型延髓背侧HB手术安全性较高,术后神经功能可获改善,手术应作为一线治疗选择.延桥交界和位于延髓背侧的大型HB(≥3 cm)手术风险大,术后可能出现呼吸衰竭、神经源性肺水肿和脑积水等,手术须慎重.
목적 탐토연수혈관모세포류(HB)림상특점화진치책략,제고수술치유솔,감소병발증화사망솔.방법 회고성분석2002지2012년상해장정의원신경외과수술치료적12례연수혈관모세포류적림상표현、영상학특정、진치경과화수술효과.결과 주요증상위두통、구체마목화지체기력하강.자공진발현병변균위단발.위우연수근뇌교1례,연수중부7례,연경교계4례；위우연막하9례,수내3례；3례유낭성변.5례행수자전영혈관조영(DSA),공혈동맥주요위소뇌후하동맥화소뇌전하동맥분지,1례행술전전새.완정절제11례,1례부행전작.술후증상개선8례,가중2례,사망2례.수방3개월～ 10년,McCormick신경공능Ⅰ～Ⅱ급,1례술후7년복발.결론 위우연수중하단수내、낭성HB화중소형연수배측HB수술안전성교고,술후신경공능가획개선,수술응작위일선치료선택.연교교계화위우연수배측적대형HB(≥3 cm)수술풍험대,술후가능출현호흡쇠갈、신경원성폐수종화뇌적수등,수술수신중.
Objective To explore the clinical characteristics,diagnostic strategies and surgical techniques of hemangioblastoma (HB) in medulla oblongata.Methods The clinical and radiological characteristics,therapeutic processes and outcomes of 12 HB cases treated at our department from 2002 to 2012 were studied by retrospective analysis.Results Headache,somatic numbness and limb muscle weakness were the major symptoms of oblongata HB.Magnetic resonance imaging before surgery revealed a total of 12 single tumors.Among these tumors,upper (n =1),middle (n =7) and lower (n =4) parts of medulla oblongata were involved.The locations were surface (n =9) and intramedullary (n =3).Three tumors had cyst.Digital subtraction angiography (DSA) was performed on 5 cases and it revealed that the main blood supply arteries of tumors were branches of posterior inferior cerebellar artery (PICA) and anterior inferior cerebellar artery (AICA).One case underwent pre-surgical embolism during angiography.Eleven tumors were totally resected and 1 was fulgurized.Symptoms improved (n =8) and worsened (n =2).And two patients died.All survivors were followed up for 3 months to 10 years and had a McCormick functional grading of Ⅰ-Ⅱ.One case relapsed 7 year later.Conclusion For Cystic HB,small or medium sized substantial HB in middle and lower part of oblongata,surgical removal is often safe and symptoms may be lessened.It can be used as a first-line treatment.For large (≥3 cm) substantial HB or HB in upper part of oblongata,serious postoperative complications such as respiratory failure,neurogenic pulmonary edema or acute obstructive hydrocephalus may occur.Thus surgical resection should be prudently considered and possible consequences thoroughly discussed with the patients.