先天性胆管扩张症研究进展
2018-04-03郭意男焦娇郭秋芳叶卫江
郭意男 焦娇 郭秋芳 叶卫江
先天性胆管扩张症(congenital biliary dilation,CBD)是一种较为少见的胆道疾病,多见于婴幼儿,男女发病比例约为1∶3[1]。东方国家较西方国家发病率高[2]。大多数CBD患者在儿童期发病,尚有25%的患者至成人期才被确诊,且成人CBD病例呈增多趋势[3-4]。本文就CBD研究进展综述如下。
1 病因
CBD的发病机制至今仍未完全阐明。除了Caroli病明确是由于位于染色体6p12的PKHD1基因变异所致外,其余各型CBD致病因素并不明确。目前被广泛接受的发病机制是胰胆管合流异常(pancreaticobiliary maljunction,PBM)[5-6]。PBM指解剖学上的胰管与胆管在十二指肠壁外合流的先天性畸形,由于十二指肠乳头Oddi括约肌无法控制合流部而发生胰液与胆汁互相混合及逆流,最终导致胆道及胰腺各种病理变化的发生。后天性因素如肿瘤、胆石、乳头炎等引起者不在此列。此外还有两种不同的学说:胆管上皮异常增殖学说与神经发育异常学说。前者认为胚胎时期胆管发生过程中其上皮增殖异常导致胆管各处的上皮增生速度不均匀所致;后者认为由于病变部位神经细胞的缺陷所致的胆管扩张类似于巨结肠的改变。
2 临床表现
2.1临床症状及体征CBD的临床表现多样,其主要症状为腹痛(78%)、呕吐(36%)、黄疸(22%)、发热(22%)等[7],其主要体征为肝脏肿大及腹部包块。CBD的临床表现还与首次发病年龄及胆道扩张的形态有关。在新生儿或婴儿期首发并且胆道囊状扩张的患儿往往表现为黄疸及腹部包块;在幼儿期首发并且胆道梭型或者圆柱形扩张的患儿往往表现为腹痛[1]。
2.2并发症CBD的并发症主要有肝内外胆管结石、胆管炎、急慢性胰腺炎、胆道系统恶性肿瘤和肝硬化等[8]。CBD患者胆道结石总体发生率为17.9%[7],胆总管较容易发生结石,不同部位的结石占比分别为:胆囊结石占12.7%,胆总管结石占65.8%,肝内胆管结石占21.5%[9]。成人CBD患者更易并发胆总管结石,成人和儿童的发生率分别为24.1%、9.0%[10]。CBD患者并发急性胰腺炎的概率成人和儿童不尽相同,成年人为10.5%~56%[11-12],儿童为23%[13]。胆道系统恶性肿瘤是CBD最为严重的并发症,成年患者发病率为21.6%[10]。CBD发生癌变时并无特异性的临床表现,仍以上腹疼痛、发热、黄疸和腹部肿块为主,部分患者可有体质量下降。Lee等[14]认为,对于老年CBD患者,如有黄疸和肝功能损害应该高度怀疑癌变的可能。15岁或者年龄更小的患儿并发胆道系统恶性肿瘤的概率尚无数据统计,但已有报道发现患儿并发胆管癌或胆囊癌[15-16]。
3 诊断与分型
3.1诊断CBD的诊断主要依赖于影像学检查。影像学检查发现其有先天性胆道系统的异常扩张伴或不伴随PBM,除外后天性因素如肿瘤、胆石、乳头炎等引起的胆道系统异常扩张,即可诊断。
3.2影像学检查(1)B超:B超能够发现扩张的胆总管、肝内胆管以及增厚的胆囊内壁。且由于其简便及无创的特性,能够为诊断CBD提供重要证据;但B超无法明确患者是否存在PBM。(2)内镜逆行胰胆管成像(endoscopic retrograde cholangiopancreatography,ERCP)及磁共振胰胆管成像(magnetic resonance cholangiopancreatography,MRCP):ERCP及MRCP对于诊断CBD均有重要地位,两者均能够清楚地显示肝外胆道系统,并且能够明确是否存在PBM,对诊断CBD的灵敏度均较高。MRCP对于诊断CBD的灵敏度为38%~100%[17-23],其对于诊断成年人及儿童PBM的灵敏度分别为82%~100%[19,21-22,24]、40%~80%[17,23-27]。在诊断PBM方面,MRCP与ERCP的灵敏度无明显差异。然而,对于那些较短的胰胆管共同通道的患者及婴幼儿,ERCP较MRCP能够更好地判断扩张情况。但由于ERCP为有创性检查,成功率及成像效果与操作者的经验密切相关,并且诱发胰腺炎的风险可高达87.5%[28-29]。因此,MRCP被认为是诊断CBD的首选辅助检查。
3.3实验室检查CBD患者无症状时,实验室指标往往也正常。仅当患者出现症状时,患者的血淀粉酶、肝酶、胆酶、胆红素等指标有不同程度的异常。
图1 Todani分型
3.4分型CBD的分型方法较多,目前最为常用的是由Alonso-Lej等[30]提出,并由Todani等[31-32]在此基础上进行多次修订、补充和完善的分型方法。该分型方法考虑到存在肝内胆管扩张、多发性扩张以及是否存在PBM等因素,分为5型(Todani分型)。但我国学者董家鸿等[33]认为Todani分型未能区分复杂的肝内胆管囊状扩张病变的病理类型,对肝外胆管囊状扩张病变的分型显得繁复且易于混淆,有一定的局限性,影响其对治疗的指导作用,并提出了董氏分型。董氏分型的提出对于CBD的临床治疗决策和手术方法选择具有明确的指导作用,但其不能够区分是否存在PBM。在临床实践中仍需两者结合使用。
3.4.1Todani分型(图1)Ⅰ型(胆总管扩张):Ⅰa型胆总管囊状扩张,常合并PBM;Ⅰb型胆总管局限性扩张,不伴有PBM;Ⅰc型肝外胆管弥漫性梭状扩张,伴有PBM。Ⅱ型:胆总管憩室样扩张,不伴有PBM。Ⅲ型:胆总管十二指肠壁内段扩张,又称为胆总管末端囊肿,通常不伴PBM。Ⅳ型(胆管多发性扩张):Ⅳa型肝内外胆管多发性囊状扩张,通常伴有PBM;Ⅳb型仅肝外胆管多发囊性扩张,是否合并PBM尚不确定。Ⅴ型:肝内胆管单发或多发性囊状扩张,又称为Caroli病,不伴有PBM。3.4.2董氏分型(图2)A型:周围肝管型肝内胆管囊状扩张,为局限于肝脏周围肝管的多发性囊状扩张病变也就是Caroli病。A1型:囊状扩张病变局限分布于部分肝段。A2型:囊状扩张病变弥漫分布于全肝。B型:中央肝管型肝内胆管囊状扩张,为局限于肝内胆管树主干肝管的囊状扩张病变。B1型:单侧肝叶中央肝管囊状扩张。B2型:病变同时累及双侧肝叶主肝管及左、右肝管汇合部。C型:肝外胆管型胆管囊状扩张,为左、右肝管汇合部远端的囊状扩张,仅累及胆总管或肝总管,其包括了Todani分型的Ⅰ、Ⅱ、Ⅳb型。C1型:病变未累及胰腺段胆管。C2型:病变累及胰腺段胆管。D型:肝内外胆管型胆管囊状扩张,为中央肝管和肝外胆管的囊状扩张,其包括了Todani分型的IVa型。D1型:病变累及单侧肝叶中央肝管和肝外胆管。D2型:病变累及双侧肝叶中央肝管和肝外胆管。E型:壶腹胆管型胆管囊状扩张,为局限于胆总管壶腹部的囊状扩张,其包括了Todani分型的Ⅲ型。
图2 董氏分型
4 治疗
成人CBD主要采用外科手术治疗。鉴于CBD患者有胆管恶性变可能,建议成人及有症状的婴幼儿一旦明确诊断应及早行手术治疗;对于无症状的婴幼儿,为避免术后缝合口破裂及吻合口狭窄,建议待患儿3~6个月或更大后再行手术治疗[34]。
4.1囊外引流术目前主张对于合并急性化脓性炎症、严重阻塞性黄疸以及囊肿穿孔等情况的CBD患者暂时行急诊外引流术,以缓解急性梗阻性及胆道感染造成的感染性休克等危重情况,等待炎症消退,全身情况好转后再进一步做囊肿切除手术。常见外引流术方法是行剖腹切开胆总管或囊肿置T管或蕈状引流管引流,此法可能造成腹腔一定的黏连,但一般不影响切除囊肿和胆道重建手术。另外,ERCP技术已经成熟,也可作为此类急症患者临时的过渡措施。
4.2囊肿彻底切除胆道重建术自1966年有学者首次报道了囊肿切除+Roux-en-Y空肠吻合术,Flanigan[35]、Yamaguchi[36]相继报道囊肿切除+胆肠吻合的经验,认为该方式可彻底消除囊腔、改善引流、明显减少手术的并发症,并且可以预防癌变,至今依然是最为推崇的手术方式。其主要指导思想是囊肿切除、胆道重建及胰胆分流。(1)囊肿切除:有报道称,残留的囊肿在术后远期(>15年)仍然有相对高的恶变率,因此建议尽可能做到彻底切除囊肿[37]。但由于成人CBD患者囊肿周围有明显的炎症,彻底切除囊肿有时较为困难,对于这类病例,Lilly[38]在1978年提出可以采用囊内切除,即残留部分与门脉相邻的后壁,该术式被命名为Lilly术式。采用此法的理论依据是囊肿本身的危害是潜在的癌变性,而癌变只起源于黏膜,因此只要消除黏膜层,就能达到预防癌变的目的。(2)胆道重建:胆道重建分为肝管十二指肠吻合术和Roux-en-Y肝管-空肠吻合术两种。其中典型的术式为Roux-en-Y肝管-空肠吻合术。对于两种手术方式孰优孰劣,有荟萃分析表明Roux-en-Y肝管-空肠吻合术与肝管十二指肠吻合术相比,除了能够明显减少内容物的回流及反流性胃炎的发生外,在胆汁外漏、胆管炎、吻合口狭窄、术后肠梗阻、再次手术率、住院时间上没有明显差异[39-40]。基于以上事实,我国及日本等东方国家仍推荐Roux-en-Y肝管-空肠吻合术作为胆道重建的首选方式。(3)胰胆分流:胰胆分流手术可以显著降低伴有PBM的CBD患者恶变率[41]。
4.3腹腔镜手术由于腹腔镜技术的普及及经验的积累,微创治疗CBD越来越受到临床重视[42]。相比传统的手术,腹腔镜手术具有减轻术后疼痛、缩短住院天数以及拥有更为清晰的手术视野等优势[43-44]。也有研究认为腹腔镜手术发生术后并发症的概率更低[45-46]。有学者认为,对于那些囊肿已经穿孔、多次进行肝脏手术以及合并严重肝功能异常的患者是腹腔镜治疗CBD的禁忌证[47]。总之,随着腹腔镜技术的普及,腹腔镜治疗CBD已成为常态,有望取代传统的开腹手术成为CBD的首选治疗方法[44,48]。
4.4肝移植自1997年美国Schiano等[49]报道了1例35岁的女性Caroli病患者行原位肝移植术的手术效果,指出原位肝移植术是晚期Caroli病的唯一有效治疗途径。肝移植除了适用于晚期Caroli病外,还适用于肝内胆管多发囊性扩张,病变弥漫累及全肝或无法单纯手术,胆管炎、肝内胆管结石、黄疸反复发作,出现门静脉高压症相关并发症的CBD患者。也有学者认为肝移植术能完整切除患肝,包括有恶变可能的患肝,从根本上消除病因[50-51]。而常规的手术治疗如肝叶切除术或囊肿切除术等也不能从根本上解决肝内胆管上皮异常增生和囊性扩张的病理改变,胆道仍有受到胆汁、炎症长期刺激而发生癌变的可能。Harring等[52]报道了140例Caroli病患者行肝移植术后受体及移植物1、3、5、10年的存活率分别为89%、83%、81%、78%及82%、75%、71%、68%。Millwala等[53]统计了104例Caroli病行肝移植的患者资料,其5年存活率可达77%,预后良好。我国于2000年首次对2例Caroli病患者行背驮式肝移植,术中各项血液动力学指标平稳,术后恢复快,影响患者生活近20年的发热、腹痛、黄疸均消失,术后1个月均顺利出院[54]。目前临床不主张对Caroli病患者行预防性原位肝移植术[55-56]。对于病变广泛分布于双侧肝叶的、弥漫性的CBD,肝移植是有效的最终选择。
4.5CBD癌变治疗术前已确诊或术中冷冻病理学检查证实已发生癌变的CBD患者应按胆管癌的治疗原则进行处理[57]。然而实际临床工作中,只有不到10%的CBD癌变患者在诊断时能够行根治性切除术[58]。
4.6术后并发症术后早期并发症包括缝合破裂、切口处出血、急性胰腺炎、胰瘘、消化道出血、肠梗阻等,大多数由于手术经验不足造成,较罕见。术后晚期并发症包括胆管炎、肝内胆管结石、胰腺结石、胰腺炎及残余扩张胆管癌等。胆管炎及肝内胆管结石往往由于吻合口狭窄、肝内胆管狭窄、残余的肝内胆管扩张等引起的胆汁淤积所致[59],其中肝内胆管结石发生率约10%[60-62]。报道称术后并发胆管癌的发生率约为0.7%[63],但由于报道例数较少不能作为发病率的依据,其余各并发症均无准确的发病率。
4.7其他囊肿内引流术在早期被广泛应用于临床。囊肿内引流手术可分为囊肿十二指肠吻合术和囊肿空肠吻合术两种方式。其操作简单,可暂时缓解CBD的临床症状,但由于术后肠液很容易反流入囊肿及胆道,可诱发反复的胆道逆行感染、胆管结石生成、胆肠吻合口狭窄等远期并发症。更为严重的是内引流术后囊肿恶变率有明显增高的趋势,因此目前不主张为CBD患者行该手术[38,64]。
5 小结
综上所述,CBD患者的发病机制目前尚未完全阐明,随着辅助检查技术的进步,CBD的诊断也日趋明确,其分型也不断得到完善并富有临床治疗指导意义。囊肿切除、胆道重建及胰胆分流是CBD手术的指导思想。Roux-en-Y肝管-空肠吻合术仍是目前胆道重建的首选手术方式。但由于有部分患者会并发术后远期并发症,因此,对CBD术后患者需进行长期乃至终生的随访观察。
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