口腔颌面部动静脉畸形的手术治疗

2019-05-27 吴丽芬 孙艳芳 刘冰 临床口腔医学杂志

患者,女,47岁。以“下颌骨肿瘤”收入院。自诉发现下颌骨前份肿物1月余,无明显疼痛不适。检查:面部不对称,颏部及右侧下颌体部膨隆,面部皮肤颜色、质地未见明显异常。口内33-46区可扪及颌骨隆起,触压痛明显,有博动感,前牙区龈黏膜可见血管扩张。32-44松动Ⅰ~Ⅱ度,叩痛(-)。CBCT示34-46区下颌骨可见骨质密度减低区,边界不清,唇颊侧骨皮质连续性部分中断,呈穿凿样,舌侧骨皮质变薄。行下颌骨节

动静脉畸形(arteriovenous malformations,AVMs),曾称蔓状血管瘤,是由于胚胎时期脉管系统发育异常,导致动、静脉直接吻合形成的血管团块,内含不成熟的动脉和静脉,缺乏毛细血管。动静脉畸形相对少见,在脉管性疾病中仅占1.5%左右。口腔颌面部是好发的部位,占所有动静脉畸形的50%。女性患病略多于男性,约为1.5∶1。颅面部动静脉畸形可累及颊、鼻、耳、上唇及头皮等部位。颌骨动静脉畸形以往被称为颌骨中心性血管瘤,可表现为反复、少量的口腔内自发性出血,活检或简单的拔牙术都可引起严重出血,甚至危及生命。
 
动静脉畸形的治疗,一直是临床上面临的难题与挑战。目前动静脉畸形治疗方法主要有介入栓塞治疗与手术治疗。不恰当的治疗如单纯供血动脉结扎或堵塞不仅达不到治疗效果,反而促进侧支循环形成使病情变得更加复杂。本文对我院2008年1月~2015年6月收治的12例手术治疗的口腔颌面部动静脉畸形病例进行总结,现报道如下。
 
1.临床资料
 
收集武汉大学口腔医院2008年1月~2015年6月住院治疗的口腔颌面部动静脉畸形12例,男6例,女6例,年龄范围10~67岁,平均年龄30岁。其中软组织动静脉畸形6例,颌骨动静脉畸形6例;软组织动静脉畸形主要累及唇、颊、颏部及颌下区;其中2例栓塞治疗后需改善面容为主诉入院,4例以局部搏动性肿物为主诉入院。上颌骨动静脉畸形1例(女性1例,67岁),下颌骨动静脉畸形5例(男1例,女4例,平均年龄32岁)。
 
颌骨动静脉畸形患者均有颌骨渐进性膨隆症状,伴有牙齿不同程度松动,1例有间断出血症状,入院前未行任何治疗。根据病情,术前行多层计算机断层扫描(multislice computed tomography,MSCT)或锥形束计算机断层扫描(cone beam computed tomography,CBCT)。软组织AVMs平扫CT表现异常软组织膨隆,边界不清,质地不均;注射增强剂后,该异常软组织局部明显强化,近似邻近血管密度,回流静脉提前显示(图1)。颌骨AVMsCT可见骨髓腔间隙增大,骨小梁消失,呈单囊或多囊样低密度灶,边界不清(图2A~C)。所有病例均经术后病理学明确诊断,随访时间2.5~10年,平均6.2年。
 
图1 软组织AVMs平扫CT。A:右侧颞部软组织AVMs平扫CT上表现异常软组织占位影,边界不清,质地不均;B:增强CT示右侧颞部占位局部明显强化


图2 典型病例图片。A~C:术前CBCT示下颌骨溶骨性破坏,唇颊侧骨皮质不连续;D:切除的下颌骨;E:术后病理示颌骨动静脉畸形(HE染色,×40);F:术后CBCT示自体腓骨移植修复下颌骨缺损
 
2.治疗方法
 
本组病例中6例软组织动静脉畸形采用病变完全切除术,1例下唇及颏部动静脉畸形切除后使用前臂皮瓣修复缺损。5例下颌骨AVMs采用节段性切除术后行自体骨移植或再植修复缺损。其中3例同期行血管化腓骨移植;1例儿童患者行肋骨移植后局部感染取出移植骨,成年后行髂骨移植;1例行自体病变骨冷冻再植术。1例上颌骨AVMs行患侧上颌骨次全切除术,未行修复重建。结果12例患者中软组织动静脉畸形6例,局灶型4例,弥散型2例。颌骨动静脉畸形6例,其中上颌骨AVMs1例。5例下颌骨AVMs累及范围较大,主要累及前牙区、颏孔周围及磨牙区的下颌骨体部,1例累及下颌升支及体部。随访时间2.5年~10年,平均6.2年,均无复发。
 
治疗后所有患者局部肿胀消退、搏动感消失,面颈部外形明显改善。其中5例下颌骨AVMs患者面部基本对称,对外形修复满意。1例上颌骨AVMs患者未行修复重建,术侧面部凹陷。
 
3.典型病例
 
患者,女,47岁。以“下颌骨肿瘤”收入院。自诉发现下颌骨前份肿物1月余,无明显疼痛不适。检查:面部不对称,颏部及右侧下颌体部膨隆,面部皮肤颜色、质地未见明显异常。口内33-46区可扪及颌骨隆起,触压痛明显,有博动感,前牙区龈黏膜可见血管扩张。32-44松动Ⅰ~Ⅱ度,叩痛(-)。CBCT示34-46区下颌骨可见骨质密度减低区,边界不清,唇颊侧骨皮质连续性部分中断,呈穿凿样,舌侧骨皮质变薄。行下颌骨节段性切除术,同期行血管化腓骨肌皮瓣移植修复下颌骨缺损。术后随访4年,病变无复发,面部外形基本对称(图2)。
 
4.讨论
 
动静脉畸形为先天性病变,尚未发现其具有遗传性。60%的病变在出生时即被发现,在儿童期或成年后方才表现出来。病灶通常随身体发育而呈比例增长,可长期保持稳定,也可因外伤、青春期或孕期体内激素变化及不恰当的治疗在短期内迅速增大。颅面部软组织动静脉畸形表现为局部软组织膨隆,界限不清,表面皮肤颜色正常或呈暗红色。随着血管生成增加,病变逐渐变大,回流静脉压力增高,引起血流动力学改变。可表现为疼痛、溃疡、出血、局部组织破坏、面容损毁等。
 
病灶内可见念珠状或条索状迂曲的粗大而带搏动的血管,皮温高于正常皮肤,可扪及震颤感,听诊有连续性吹风样杂音。颌骨动静脉畸形可扪及颌骨膨隆,伴或不伴周围软组织动静脉畸形。可表现为反复牙周渗血,也可在拔牙或误诊手术时引起严重大出血,常规止血手段难以奏效,常常危及生命。颅面部软组织动静脉畸形常位于体表,通过病史及临床表现可易于诊断。对于位置较深在的病变可借助影像学手段以明确病变的范围及性质。CT对于显示颌骨内病变的位置、范围和边界方面具有优势。
 
MRI可较好显示病变与周围解剖结构的关系。数字减影血管造影(digital subtraction angiography,DSA)是目前诊断颌骨AVM的金标准,但因其创伤大、费用高,一般不作为常规检查手段,仅与介入栓塞治疗配合使用。根据临床特征或影像学表现,动静脉畸形可有多种分类。传统上可分为局灶型及弥散型。局灶型病变由单一滋养动脉供血,病变界限较清晰。弥散型病变由多个滋养血管供血,血管迂曲交错,破坏周围组织使边界不清,治疗难度大,复发率较高。
 
动静脉畸形的治疗一直都是临床上面临的难题与挑战,需要多学科的合作,要求医师具有丰富的临床经验。治疗目的是减少病变对周围组织的破坏,减轻临床症状,减少可能引起生命危险的并发症及引起进一步的面容损毁。目前,动静脉畸形的主要治疗方法包括介入栓塞治疗与手术治疗。
 
介入栓塞治疗前必须行DSA检查,且治疗过程中局部及全身并发症较多见,最好在有相关设备及技术条件的医院由具有介入治疗经验的医师来完成。手术治疗主要用于病变范围较局限、经栓塞治疗后病变缩小局限或介入栓塞后仍难以治愈的病变。一般认为应完整切除病灶,选择界限较清晰的局灶型病变是较为理想的。对于累及范围较大的AVMs可在术前行栓塞治疗,有助于缩小病变、减少术中出血及降低复发的概率。在可能的情况下应避免局部切除,尤其对于弥散型病变,局部切除可导致病变的进一步发展,使后续的治疗变得更加困难。
 
以往认为手术治疗风险高、出血多,且会造成容貌毁损及影响咀嚼功能。随着影像学手段的改进、修复重建技术的进步及动静脉畸形基础研究的发展,以往不能开展的手术得以施行。手术治疗可大大减轻患者症状、改善外观、提高生活质量。Kansy等报道2例单纯行手术治疗病例,9例栓塞联合手术治疗病例术后随访30个月无复发。Richter等报道3例单纯行手术治疗,7例栓塞联合手术治疗的巨大弥散型AVMs,80%患者病变得到极大地改善。本组病例中软组织AVMs中4例病变均为局限型,2例弥散型病变行栓塞治疗使病变缩小后手术治疗,术后平均随访6.2年无复发。口腔颌面部AVMs的栓塞治疗强调超选择,尽量接近病灶,以减少对正常组织的损害。
 
介入栓塞的关键是直接消灭异常血管团,禁忌行供血动脉结扎或堵塞。栓塞治疗目的是治愈病变,缩小病变范围减少并发症的发生及利于随后手术的进行;常用的栓塞材料有聚乙烯醇(polyvinyl alcohol,PVA)颗粒、二氰基丙烯酸正丁脂(N-butyl-2-cyanoacrylate,NBCA)和无水乙醇等。无水乙醇通过细胞脱水,直接破坏血管内皮细胞,使血液蛋白质迅速变性及血栓形成,是目前唯一可治愈动静脉畸形的液体栓塞剂。有学者认为直接穿刺“静脉池”栓塞结合动脉血管内栓塞的“双介入法”是治疗颌骨AVMs的有效方法。介入栓塞治疗属于创伤性操作,可引起一些并发症如出血、栓塞剂误流入肺、局部组织坏死等,应由具有一定介入治疗经验的医师实施。目前,栓塞联合手术治疗仍是口腔颌面部AVMs的推荐疗法。
 
原始出处:

吴丽芬,孙艳芳,刘冰,赵怡芳.口腔颌面部动静脉畸形的手术治疗[J].临床口腔医学杂志,2018(06):366-368.

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