超选择性肝动脉栓塞术治疗多囊肝的基础及临床研究

Polycystic liver disease (PLD) is the most common extra-renal manifestation associated with autosomal dominant polycystic kidney disease (ADPKD) and is one of the most rare and difficult diseases. However, the relative symptoms of PLD have become more frequent recently because of lengthened lifespan of ADPKD patients, and patients with PLD often suffer from severe abdominal discomfort, dyspepsia, or dyspnea. PLD has become one of the thorny issues in the clinical medical field. Unfortunately, all of the traditional therapies have certain limits, so additional new approaches are anxiously needed. Transcatheter arterial embolization (TAE) has become an accepted treatment option for patients with symptomatic PLD, because super selective embolization of the hepatic artery branches that supply major hepatic cysts can shrink the cyst and liver size and alleviate the symptoms. But it is still not clear which are the best embolic materials nor what are the exact factors influencing the therapeutic effect of PLD. .We designed this study on the basis of previous clinical studies to investigate the relationship between the different dilution ratio of N‑butyl‑2‑cyanoacrylate (NBCA) by iodized oil and the therapeutic effects, so as to provide a reference for clinical application. We also intend to evaluate the safety and efficacy of TAE of symptomatic PLD by using mixtures of NBCA and iodized oil affiliated with bleomycin. By analyzing the angiography photos of PLD, we try to illustrate the characteristics of the hepatic artery branches of the cystic regions, such as the distribution, variation, and extrahepatic collaterals, et al.

多囊肝（PLD）是常染色体显性遗传性多囊肾病最常见的肾外表现，属于少见疑难疾病。近年随着ADPKD患者寿命延长，与多囊肝相关的症状如腹部严重膨胀不适、消化、呼吸困难等，严重影响了患者的生活质量，是目前临床医学领域的棘手问题之一。传统治疗症状性多囊肝的方法均有一定限度，因此研究和发展新的治疗技术有较大的临床意义。前期研究证明经导管肝动脉栓塞术（TAE）精确栓塞供应肝囊肿的供血动脉，可使肝囊肿显著缩小、缓解临床症状，但目前仍存在何种栓塞材料更为适合、影响疗效的因素不确切等问题。本课题拟在前期临床研究的基础上，比较NBCA胶被碘油不同程度稀释后对栓塞效果的影响，选择出最佳的NBCA胶-碘油乳剂混合比，为临床应用提供参考依据；论证NBCA胶-碘油乳剂联合博来霉素栓塞的安全性和有效性，筛选更适宜的栓塞材料，提高介入治疗的疗效；阐明多囊肝供血动脉的走行、变异、侧枝等规律，为TAE超选择性插管提供参考。

多囊肝（PLD）是常染色体显性遗传性多囊肾病（ADPKD）最常见的肾外表现，属于少见疑难疾病。近年随着ADPKD患者寿命延长，与多囊肝相关的症状如腹部严重膨胀不适、消化、呼吸困难等，严重影响了患者的生活质量，是目前临床医学领域的棘手问题之一。传统治疗症状性多囊肝的方法均有一定限度，因此研究和发展新的治疗技术有较大的临床意义。.主要研究内容及结论：.①动物实验研究部分：进行了组织胶[N-丁基-2-氰基丙烯酸酯（NBCA）]与碘油不同混合比例的动物实验研究。通过实验研究发现NBCA胶被碘油按1:1及1:2稀释时，极易凝集、堵塞微导管，且栓塞术后组织坏死明显，感染等并发症发生率高；而当NBCA胶被碘油稀释至1:6时，栓塞范围不易控制，栓塞效果会减弱。因此，本课题组认为，当NBCA胶被碘油稀释至1:3-1:5范围时，在临床应用中更能好达到栓塞目的，且较为安全、便于操作。.②临床研究部分：进行了肝动脉栓塞术（TAE）栓塞材料的改进-改良研究：创新性使用稀释组织胶（NBCA）-碘油乳剂+低剂量博来霉素用于临床治疗多囊肝，临床证实安全性高，更有助于阻塞肝动脉的微小分支，使囊肿体积进一步缩小，患者症状改善，疗效确切。.③多囊肝的血管造影研究：率先提出多囊肝的囊肿多按肝叶-段分布、仅由肝动脉分支供血（正常肝脏由肝动脉、门静脉双重供血），此特点有利于定向精确栓塞，能最大限度的保留正常肝组织。.④侧枝血管参与多囊肝的规律：本项目组研究发现：除了肝动脉外，膈下动脉、胸廓内动脉、网膜动脉、右肾包膜动脉等体循环侧支均可参与多囊肝的供血，漏栓这些侧枝会影响疗效。.⑤对症状性多囊肝患者的临床特征进行了总结，归纳其在性别、地域分布、民族、年龄、家族遗传等方面的特点。