罕见门静脉瘤1例分享!

2018-08-06 佚名 影像园

患者,男,55岁,发现右侧腹股沟区可复性包块10月。

病史

患者,男,55岁,发现右侧腹股沟区可复性包块10月。

查体

腹部平坦,腹软,全腹部无压痛,无肌紧张及反跳痛,右侧腹股沟区可见约3*2cm大小包块,坠入右侧阴囊,质软,手法还纳或平卧休息后可消失,无红肿、压痛,透光试验阴性,按压内环口立位咳嗽时包块不复现。右侧睾丸位于阴囊内,左侧阴囊空虚,未扪及睾丸。

家族史

诉其二哥、儿子及孙子均患有腹股沟疝。

上腹部CT平扫+增强:



多平面重建(MPR)



容积再现(VR)



影像诊断:门静脉瘤

诊断分析:首先,患者以“发现右侧腹股沟区可复性包块10月”就诊,下腹部平扫+增强未发现占位性病变(图像略),于是继续行上腹部CT平扫+增强寻找腹腔内睾丸,未查及睾丸存在,但意外发现门静脉瘤。门静脉瘤平扫征象并不明显,只能发现胰头区的占位,增强扫描门静脉早期肿块呈不均匀性强化,这是由于造影剂部分到达门静脉,未形成浓聚;门静脉晚期,造影剂浓聚并充盈,可见门静脉局限性扩张达4.1cm,门静脉瘤的轮廓被完整的勾画出来,其征象显而易见。MRP和VR重建图像直观地显示瘤体形态、所在部位、载瘤静脉、周边情况等,同时对于手术也具有一个更加直观的引导。

讨论

门静脉瘤(PVA)是一种门静脉系罕见的血管畸形,往往在无症状的体检中偶然发现。门静脉结构特殊,其两端都是毛细血管且无瓣膜。一般认为,门静脉瘤病因有以下几种:先天性、继发于门静脉高压、慢性肝疾病、胰腺炎导致胰腺区血管壁损伤变薄等,但是仍有很多患者病因不明。大多数病人并无明显症状或者只有轻微腹痛,但部分病人可出现黄疸,消化道出血等症状。其并发症包括门静脉血栓形成,瘤体破裂、自发性门体分流,以及瘤体对周围组织的压迫等。按照发生部位可分为肝内型和肝外型门静脉瘤,且以肝外型居多,肝外型好发于脾静脉与肠系膜上静脉汇合处,当肝内和肝外门静脉局限性扩张,直径分别>1.5cm和>2.0cm时,即可诊断为门静脉瘤(本例属于肝外型门静脉瘤,且发生于常见部位,直径约4.1cm)。

彩超诊断门静脉瘤的灵敏度和特异性都比较高,且价格低廉,特别适用于门静脉瘤患者的长期随访。CT、MRI亦可准确诊断,CTA、MRA可获得直观的印象并可确定瘤体的确切位置以及与周围组织的关系,故可为外科医生术前评估提供足够信息。

大多数门静脉瘤的病人多年随访并无明显的症状,且瘤体大小保持稳定。一般认为无症状的病人或无门静脉高压等基础疾病的病人无需手术治疗,只需要定期的随访即可。但有学者提出不同意见,强调预防性手术的必要性,对于那些有症状的病人或是瘤体增加迅速易于发生破裂或血栓形成等并发症危险的病人需要预防性手术。

对于瘤体位于门静脉主干的病人,可作门静脉成形术或门体分流术,这些手术在早期的随访中均可获得较好的效果。对于有门脉高压的病人,建议行门体分流术,它可避免解剖和分离瘤体,大大减少手术的风险性。分流手术可降低门静脉压力防止瘤体的进一步扩张。对于没有门静脉高压的病人,则门静脉成形术更加合适,因为它可以保持门静脉正常血流而且避免了门体分流的一些并发症如肝性脑病的发生率。

手术前可以应用生长抑素降低门静脉压力,使手术中出血易于控制,手术安全性增加且副作用少。目前门静脉手术治疗的报道比较少,且缺乏长期的随访报道。尚需在血管外科长期的临床实践中探索门静脉瘤手术的适应症及最合适的手术方式。需要强调的是,门静脉瘤的治疗因人而异,应根据每个具体患者的病情,瘤体大小,和周围组织的解剖关系来确定。总之,门静脉瘤虽然罕见,但其影像学检查征象明显、典型,容易诊断。彩色多普勒、CTA、MRA等无创检查使门静脉瘤的检出大大提高。其治疗方法应该强调个体化,即根据每一位具体患者的自身情况采取不同的治疗方法。

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    2021-01-13

    我小孩5岁检查出来门静脉瘤 33MM 无症状 想问问看有没有过这样的例子是做手术还是不做呢

    0

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    2018-10-29 fusion
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    2018-08-08 syscxl

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