门静脉海绵样变性

门静脉海绵样变性 患者，男，21岁，既往体健，常规查体发现门脉系统病变，查肝功无异常 图1 quickly. (23) Gao Changhai night rescued compatriots in January 1943, the Japanese control after Su Jiahu along the highway, to intensify guerrilla "raids". One day, the Japanese army from Wuzhen area caught 53 "Shina" is held in spring qiaotu farm 图 2 图 3 quickly. (23) Gao Changhai night rescued compatriots in January 1943, the Japanese control after Su Jiahu along the highway, to intensify guerrilla "raids". One day, the Japanese army from Wuzhen area caught 53 "Shina" is held in spring qiaotu farm 图 4 (图 5 quickly. (23) Gao Changhai night rescued compatriots in January 1943, the Japanese control after Su Jiahu along the highway, to intensify guerrilla "raids". One day, the Japanese army from Wuzhen area caught 53 "Shina" is held in spring qiaotu farm 图 6 图 7 quickly. (23) Gao Changhai night rescued compatriots in January 1943, the Japanese control after Su Jiahu along the highway, to intensify guerrilla "raids". One day, the Japanese army from Wuzhen area caught 53 "Shina" is held in spring qiaotu farm 图 8 图片分析:门静脉主干正常形态消失，代以多条管状血管结构，呈杂乱的网络样回声。彩色血流显示:病变区见丰富的静脉血流信号。 诊断:门静脉海绵样变性。 病因:肝门静脉主干和分支均缺乏静脉瓣，易发生阻塞。阻塞后血流受阻，肝门静脉周围的侧支和旁路形成，使门静脉代偿性再通，肝门静脉正常结构消失，变狭窄.增厚，故呈团状强回声，内有不规则相通的管状无回声。其内见红蓝相间的彩色血流，呈扭曲状，为低速平直的静脉频谱。 鉴别诊断:肝Ca，肝Ca彩色血流不呈扭曲状，内见动脉频谱。 门静脉海绵样变: 原因:据文献报道,继发性门脉海绵变形致病因素多为门脉癌栓、血栓,其次为门脉周围纤维组织炎,脾切除术后及各种凝血疾病,消化系统感染疾病及外界压迫。儿童门静脉海绵样变性主要是由于肝门部及其分支部 门静脉管腔的缺失!狭窄或闭锁所致"病因可能与下列因素有关:先天畸形,患儿同时合并其他先天畸形,如心血管!肾脏!胃肠道及卵巢等[1];新生儿脐部感染引起脐静脉炎,累及门静脉系统,导致门静脉闭塞和门静脉周围侧支静脉形成门静脉的血管瘤"门静脉海绵样变性分为原发及继发两种,后者多发生在门静脉急性栓塞后,而前者是由于门静脉主干和它的属支先天性发育异常所致. 临床现: 儿童起病急,常以食管胃底静脉曲张,上消化道出血常为首发症状"因其侧支循环quickly. (23) Gao Changhai night rescued compatriots in January 1943, the Japanese control after Su Jiahu along the highway, to intensify guerrilla "raids". One day, the Japanese army from Wuzhen area caught 53 "Shina" is held in spring qiaotu farm 建立不完善,出血量大80%以上患儿在1~6岁出现首次食管胃底静脉曲张破裂出血,此外还有脾肿大!脾功能亢进,黄疸(继发于出血!感染!交通静脉压迫胆总管!肝细胞缺血性损伤等)!小肠静脉血栓形成!肺栓塞等"肝脏无肿大及病变,其形态及组织学检查一般是正常的,肝功能良好"但因肝血流灌注不足,肝体积可较正常为小,肝功能也可有不同程度的异常. 继发性门静脉海绵样变常有原发病表现和门静脉高压等表现. 本例超声表现为:肝门区蜂窝状无回声,且内可见红蓝相间的血流通过,脾大,符合门静脉海绵样变表现. 具体原因需排除血液疾病(脾大表现)后,才考虑为先天异常. 支持楼上战友们的观点:原发性门静脉海绵样变性并门静脉高压形成。 原发性门静脉海绵样变性是指门静脉结构先天性发育异常，门脉管腔缺失、狭窄或闭锁，取而代之的是肠系膜和肝静脉之间的静脉丛异常增生的血管网。 诊断依据: 一.临床:年轻(21岁)，无症状(常规查体发现，肝功无异常)。 二.超声表现: 1.门静脉主干及各级分支失去正常管径结构，被大量呈蜂窝状、蚓团状红蓝相间的血流信号代替。 2.脾大，脾门部脾静脉及肠系膜上静脉增宽，属门静脉高压的表现。 3.除了右叶小囊肿外，肝脏实质没有异常改变。 本例累及门静脉主干及各级分支，病变范围如此之广，比较少见。 鉴别: 继发性门静脉海绵样变性。继发性门静脉海绵样变性由门静脉栓塞所致(占54%)，其中57%为癌栓。本例患者较年轻，没有原发病灶，并且累及范围如此之广，故不考虑。 quickly. (23) Gao Changhai night rescued compatriots in January 1943, the Japanese control after Su Jiahu along the highway, to intensify guerrilla "raids". One day, the Japanese army from Wuzhen area caught 53 "Shina" is held in spring qiaotu farm