Lal Richa, Prasad D K V, Krishna Phani, Sikora Sadiq S, Poddar Ujjal, Yachha S K, Kumari Niraj
Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow, 226014 UP, India.
Pediatr Surg Int. 2007 Aug;23(8):773-8. doi: 10.1007/s00383-007-1948-0. Epub 2007 Jun 14.
The purpose of this study is to classify biliary atresia (BA) with a "cyst at porta" according to the cholangiographic anatomy and to define management strategy and outcome in each group. A cyst at porta was identified in 13 of 58 babies (22.4%) with BA at first presentation. The cholangiographic anatomy was classified as; Group A (n = 7), type III BA with extrahepatic cyst; Group B (n = 2), type I or II BA with extrahepatic biliary cyst; and Group C (n = 4), type I or II BA with both extrahepatic and intrahepatic biliary cysts. The remaining 45 patients were comprised of type III BA without a cyst. A Kasai's portoenterostomy (PE) was performed for all Group A patients. Groups B and C were treated by hepaticojejunostomy (n = 5) or portoenterostomy (n = 1). All 45 patients with type III BA without a cyst were treated by a Kasai's PE. The median age at surgery was 92 days (ranges 28-342 days). There were three early post-operative deaths, all in patients with type III BA without cyst. Overall 18/55 (32.7%) patients achieved a jaundice free state. In Group A, 5/7 (71.4%) patients had bile flow, 2/7 (28.6%) are anicteric and 2/7(28.6%) had 1-2 episodes of post-operative cholangitis. In Group B, both patients are anicteric and none had post-operative cholangitis. In Group C, all four babies had bile flow but, significant morbidity because of recurrent severe cholangitis. Only one patient reached a jaundice free state. Of the remaining 42 patients with type III BA without a cyst, 27 (64.3%) had bile flow, 13 (31%) became jaundice free and 14 (33.3%) have had 1-2 episodes of post-operative cholangitis. In conclusion, thirteen of 58 (22.4%) babies with BA had a "cyst at porta" at first presentation in this series. The outcome was most satisfactory in type I BA without intrahepatic cystic dilatation (Group B) in terms of achieving a jaundice free state and freedom from recurrent cholangitis. However, intrahepatic biliary cysts (Group C) were associated with recurrent severe cholangitis and a poor eventual outcome despite a good initial bile flow. The outcome in type III BA with extrahepatic cyst was comparable to type III BA without cyst.
本研究的目的是根据胆管造影解剖结构对伴有“肝门部囊肿”的胆道闭锁（BA）进行分类，并确定每组的治疗策略和预后。在58例初诊为BA的婴儿中，有13例（22.4%）发现有肝门部囊肿。胆管造影解剖结构分为：A组（n = 7），III型BA伴肝外囊肿；B组（n = 2），I型或II型BA伴肝外胆管囊肿；C组（n = 4），I型或II型BA伴肝外和肝内胆管囊肿。其余45例患者为无囊肿的III型BA。所有A组患者均行了Kasai肝门空肠吻合术（PE）。B组和C组分别接受了肝管空肠吻合术（n = 5）或肝门空肠吻合术（n = 1）。所有45例无囊肿的III型BA患者均接受了Kasai PE手术。手术时的中位年龄为92天（范围28 - 342天）。术后有3例早期死亡，均为无囊肿的III型BA患者。总体而言，55例患者中有18例（32.7%）达到了无黄疸状态。A组中，5/7（71.4%）的患者有胆汁引流，2/7（28.6%）无黄疸，2/7（28.6%）有1 - 2次术后胆管炎发作。B组的2例患者均无黄疸，且均未发生术后胆管炎。C组的4例婴儿均有胆汁引流，但因复发性严重胆管炎导致明显的发病率。只有1例患者达到了无黄疸状态。在其余42例无囊肿的III型BA患者中，27例（64.3%）有胆汁引流，13例（31%）无黄疸，14例（33.3%）有1 - 2次术后胆管炎发作。总之，在本系列中，58例BA婴儿中有13例（22.4%）初诊时有“肝门部囊肿”。就达到无黄疸状态和无复发性胆管炎而言，I型BA无肝内囊性扩张（B组）的预后最为满意。然而，肝内胆管囊肿（C组）尽管初始胆汁引流良好，但与复发性严重胆管炎和最终不良预后相关。伴有肝外囊肿的III型BA的预后与无囊肿的III型BA相当。
