新生儿幼年性巨大毛细血管瘤1例报告

2019-01-04 唐东方 尤金良 李伟 中风与神经疾病杂志

男婴,13d,出生时发现左侧颞枕有大小约4.0 cm×5.0 cm类圆形包块,后逐渐增大。患儿饮食较好,无发热,无抽搐,可自行进乳,尿便正常。查体:皮肤红润,前囟平坦、呼吸平稳,腹部平坦,四肢肌张力及反射正常,左侧颞枕部可见一大小约5.0 cm×7.0 cm包块,包块中心有深红色不规则皮肤(见图1)。包块突出皮肤表面部分活动度较好,按压柔软,包块内未触及硬性结节。

血管瘤是婴幼儿最常见的良性肿瘤,但是新生儿的发病率较低。尤其是头皮巨大血管瘤的报道甚少。在此我们报道1例新生儿巨大头皮血管瘤并复习相关文献,以期提高对相关疾病的认识。
 
1.病例资料
 
男婴,13d,出生时发现左侧颞枕有大小约4.0 cm×5.0 cm类圆形包块,后逐渐增大。患儿饮食较好,无发热,无抽搐,可自行进乳,尿便正常。查体:皮肤红润,前囟平坦、呼吸平稳,腹部平坦,四肢肌张力及反射正常,左侧颞枕部可见一大小约5.0 cm×7.0 cm包块,包块中心有深红色不规则皮肤(见图1)。包块突出皮肤表面部分活动度较好,按压柔软,包块内未触及硬性结节。

图1 左侧颞枕类圆形包块
 
影像学检查:(1)CT:各部脑结构未见异常;脑室、脑池的位置、大小及密度均未见异常;中线结构无移位。左侧颞顶枕皮下见不规则团块状软组织密度影,大小约5.3 cm×1.8 cm×4.3 cm,CT值约31HU,边界尚清(见图2)。(2)局部软组织浅表彩超:左侧头部指示部位皮下可见一实质性略强回声光团,边界尚清,内部回声欠均,可见管道样回声;肿物内可见丰富血流信号。
 
图2 头部CT扫描
 
实验室检验未见明显异常。完善相关检验、检查后,患儿全麻下行头皮肿物切除术,肿物位于皮下,实性,质地软,呈不规则形,有包膜,与周围组织轻度粘连,血运丰富,肿物内有暗红色胶冻状内容物,肿物大小约6.5 cm×4.5 cm×1.5 cm。术后病理诊断为幼年性毛细血管瘤(见图3)。术后患儿恢复良好出院。
 
图3 病理为幼年性毛细血管瘤
 
2.讨论
 
血管瘤是婴幼儿最常见的良性肿瘤,新生儿的发病率为1%~2%,1岁左右发病率上升至12%。血管瘤的发病率有种族差异,白人的发病率高于其他种族,黑人的发病率最低,女性发病率高于男性。1863年Virchow以镜下血管结构为基础,提出单纯性血管瘤、海绵状血管瘤和蔓状血管瘤的分类概念。Wegner于1877年提出淋巴血管瘤的概念,此后单纯性血管瘤又成了毛细血管瘤的同义词,同样有着相似毛细血管结构的先天性毛细血管畸形也被归属到毛细血管瘤中。血管瘤按照其生长特点及临床特点可分为增殖期、消退期和消退完成期。婴幼儿血管瘤通常在出生后1w~1m内出现,随后血管瘤快速增长,体积增大,在经过一个始发的快速增殖期后,血管瘤逐渐稳定,不再继续快速增殖,肿瘤内细胞成分减少,并逐渐被纤维脂肪组织所取代。血管瘤是血管生长及修复的紊乱,其主要的特征是毛细血管的内皮细胞增生。
 
毛细血管内皮细胞与血管瘤的病程变化密切相关,毛细血管内皮细胞的凋亡在血管瘤的自然消退过程中起重要作用。毛细血管瘤可分布于全身,相对好发于面颈部。其界限清楚,呈鲜红或紫红色,表面高低不等,多高出皮肤,皮下的部分因分布较深而呈青紫色,质地较韧,常可扪及皮下肿块。当血管腔表面内皮细胞发生无异形性增生,内皮细胞增生显著、但血管结构未成熟或完全闭塞时,即为幼年型毛细血管瘤(juvenile capillary hemangioma)。
 
幼年性毛细血管瘤在血管瘤中发病率较低,常在出生几日内发现,也可表现为出生数周内保持红斑点而后逐渐增大,常突出皮肤表面,色泽鲜红似“草莓状”,又称草莓状毛细血管瘤,表面有分叶状,多为单发性。血管瘤在诊断过程中可采用彩色多普勒超声、CT、MRI等。使用彩色多普勒超声检查血管瘤时,血管瘤在高频灰阶超声图像上均表现为一中等偏低回声的实质性团块,呈类圆形或不规则形,边界清楚,内呈网格状。
 
CT平扫肿瘤呈结节状、分叶状、管状或团块状低密度病变。增强扫描后血管成分显著强化,可呈扭曲血管状,而非血管成分不强化或轻度强化。MRIT1WI上肿瘤呈略高信号或等信号,T2WI上呈显著高信号,其中夹杂不均匀信号,病理上代表了脂肪、纤维组织、平滑肌、血栓、静脉石和钙化。CT和MRI对血管瘤均可做出定性诊断,但MRI在显示血管瘤内特征性的血管及血管成分间的脂肪纤维组织方面较CT优越。
 
血管瘤的治疗方法多样,可选择治疗方法的现主要有等待观察、药物治疗、激光治疗及手术治疗。在选择治疗方法时,涉及多方面因素,应根据具体情况而定,目前尚无一种治疗方法适用于所有情况。非重要部位的增殖期血管瘤,如体积较小,或处于生长稳定期,未对美观和功能造成重要影响,可定期随访观察,处于消退期的血管瘤可以定期随访观察。激光主要适用于表浅、面积较小且生长缓慢或已停止的血管瘤,可以阻断其进入快速增殖期。对难治性、多发性的婴幼儿血管瘤,口服激素是加速其自然消退的首选方法。体外细胞培养研究表明,糖皮质激素能非常明显地抑制血管瘤血管内皮细胞的增殖。
 
对激光治疗或口服激素治疗效果欠佳,并且血管瘤已处于消退期的患者,可采用激素或平阳霉素瘤内注射治疗,总有效率可达94.5%。而对于身体隐蔽部位的中、小型婴幼血管瘤可采用5%咪喹莫特局涂,其用药方便,且全身反应及副作用较少。冷冻疗法曾被用于浅表血管瘤的治疗,并取得了一定效果,但由于其并发症多见,现已较少用于血管瘤的治疗。手术治疗血管瘤的适应证较广泛,对其他方法无效的病灶都可以考虑手术切除或行后期修复。因大部分血管瘤具有自我消退的特点,手术治疗被不少学者认为是最后采取的方法,但近年有学者对手术切除的血管瘤患者进行回顾并随访,对其疗效进行分析,手术切除血管瘤其总体有效率80.61%,且<3岁年龄组治愈率为90.24%。
 
血管瘤瘤体随着病程的延长和患儿的生长发育逐渐增大,部分类型血管瘤病变深度加深,影响治疗和预后。皮肤浅表部位血管瘤在局部皮肤会有较明显变化,影响美观,对患儿造成心理压力。近年来,心理因素对患儿身心发育的影响也越来越受到重视,儿童在18~24m时开始出现自我意识,完整的自我身体形象意识在3岁时形成,早期适当治疗,不仅可以较少因血管瘤带来的身体损害,也可以缓解患儿及家属的心理压力。
 
本例患儿出生时即发现肿物位于左侧颞枕部皮下,约5.0 cm×4.0 cm大小,并在2w内迅速增大至7.0 cm×5.0 cm。该患儿随访6m,术后患儿恢复良好且无明显并发症,肿物无复发。因此,对于体积较大,反复发生溃疡、感染、出血、瘢痕,伴有其他并发症或动态观察过程中逐渐增大的表浅幼年性毛细血管瘤,若无手术禁忌,可考虑手术治疗,大部分手术治疗术后恢复良好,并发症亦少见。
 
原始出处:

唐东方,尤金良,李伟,陈趟,李文鹏,王育波,张显峰.新生儿幼年性巨大毛细血管瘤1例报告[J].中风与神经疾病杂志,2018,35(01):81-82.

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  2. [GetPortalCommentsPageByObjectIdResponse(id=1975773, encodeId=b92e19e577380, content=<a href='/topic/show?id=544e63e06be' target=_blank style='color:#2F92EE;'>#毛细血管瘤#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=63706, encryptionId=544e63e06be, topicName=毛细血管瘤)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4cea26, createdName=yxch36, createdTime=Sun Jun 02 12:02:00 CST 2019, time=2019-06-02, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=369653, encodeId=7aa33696534f, content=这个是不是随年龄增长会长大啊?, beContent=null, objectType=article, channel=null, level=null, likeNumber=63, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/08/04/0723196b0475bd13fcb558f0517dbad7.jpg, createdBy=44622104218, createdName=apan小潘潘, createdTime=Sun Jul 14 12:34:22 CST 2019, time=2019-07-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1694858, encodeId=013916948581f, content=<a href='/topic/show?id=a48a63e0086' target=_blank style='color:#2F92EE;'>#毛细血管#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=50, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=63700, encryptionId=a48a63e0086, topicName=毛细血管)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=0ab329781157, createdName=wshxjq, createdTime=Mon Oct 21 16:02:00 CST 2019, time=2019-10-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=358492, encodeId=90be35849212, content=新生儿幼年性巨大细血管瘤。, beContent=null, objectType=article, channel=null, level=null, likeNumber=76, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160806/IMG57A5698BDB0244113.jpg, createdBy=32a91358059, createdName=jyzxjiangqin, createdTime=Fri Jan 18 07:05:08 CST 2019, time=2019-01-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1348277, encodeId=f75313482e727, content=<a href='/topic/show?id=1eaf89e26d3' target=_blank style='color:#2F92EE;'>#血管瘤#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=35, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=89726, encryptionId=1eaf89e26d3, topicName=血管瘤)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=fc8528, createdName=clmlylxy, createdTime=Sun Jan 06 11:02:00 CST 2019, time=2019-01-06, status=1, ipAttribution=)]
    2019-07-14 apan小潘潘

    这个是不是随年龄增长会长大啊?

    0

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    2019-01-18 jyzxjiangqin

    新生儿幼年性巨大细血管瘤。

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