摘要
门静脉海绵样变(CTPV),特别是成人原发性较少见。本文通过介绍典型的1例原发性门静脉海绵样变病例,结合文献资料复习本病的病因,病理及其影像学及临床诊断方面的理论知识,并通过本例的几个特点:①老年女性;②病史40年以上,无肝病病史;③门静脉扩张呈海绵状血管瘤样改变;④伴有肺循环紊乱及肺动脉高压(门静脉性-肺动脉高压);⑤管壁大量钙化;⑥巨脾并大量钙化;⑦累及邻近器官(左肾);⑧病变范围较广泛。更加进一步提高对门静脉海绵样变特别是巨大海绵状血管瘤样改变和伴有肺循环紊乱及肺动脉高压(门脉性-肺动脉高压)的认识,并结合文献了解临床对门静脉海绵样变的治疗方法及进展。
Cavernous transformation of the portal vein, CTPV, the adult primary stage is specially rare. This article approveing an introduction model ＇s example primary CTPV. Combining Literature information to review the causes of disease,the pathology ,the intervertebral study and the clinical diagnosis aspect theoretical knowledge, and adopting this example several characteristics 1：Old age female. 2： Above medical history 40 years. 3 ： The portal vein expansion assumes the angioma cavernosum type change. 4 ： Is accompanied by the pulmonary circulation disorder and the pulmonary artery high pressure. 5：The blood vessel wails have massive calcifications. 6： Great spleen and massive calcifications. 7：Implicates the adjacent organs（ left kidney）. 8 ： The pathological sco-peis widespread. Further Improves understanding about CTPV Specially the huge angiomacavernosum type changes and about the pulmonary circulation disorderly and the pulmonary artery with high pressure （portal veinpulmonary artery high pressure）, and understands the clinical method of treatment and the progress which about CTPV.
