Lenriot J P, Gigot J F, Ségol P, Fagniez P L, Fingerhut A, Adloff M
Department of Surgery, Centre Hospitalier Longjumeau, France.
Ann Surg. 1998 Aug;228(2):159-66. doi: 10.1097/00000658-199808000-00003.
To review the features of adult patients undergoing surgery for bile duct cysts, focusing on the anatomy of the biliary tree as well as the long-term outcome.
Bile duct cysts (BDCs) are uncommon in Western countries, and the majority of reported cases originate from Asia. Japanese authors have emphasized the frequent association of extra- and intrahepatic bile duct dilatations, but grading of patients based on Todani's classification is often hindered by the absence of an accurate definition of types IC and IVA cysts. Moreover, despite the increasing use of extrahepatic cyst excision, little is known about the long-term outcome in patients with intrahepatic bile duct involvement.
Forty-two adult patients with BDC were treated between 1980 and 1992 in 17 institutions of the French Associations for Surgical Research. Clinical presentation, radiologic presurgical evaluation, and surgical procedures were analyzed. The long-term postsurgical outcome was derived from patient charts, attending physicians, or direct patient contact.
Twelve patients (30%) had recurrent abdominal pain or jaundice from childhood. Seven (17%) had undergone prior cystenterostomy. Twenty-one (50%) had a Todani-type IVA cyst with extra- and intrahepatic bile duct involvement. Of these, nine had segmental, exclusively left-sided intrahepatic bile duct dilatation. Biliary carcinoma was encountered in five patients (12%). Extrahepatic cyst excision with a Roux-en-Y hepaticojejunostomy was performed in 34 patients with type I or IV cysts. The overall operative mortality rate was 2.4%. Long-term results were clearly correlated with cyst type: during a mean follow-up of 8.4 years, 11 of 12 patients (92%) treated by cyst excision for type I cyst remained free of symptoms, whereas 31% of patients who underwent surgery for type IV cyst had episodic or severe cholangitis with intrahepatic stones.
In patients with BDC, particular attention must be given to the associated intrahepatic bile duct dilatations. We propose a modification of Todani's classification to distinguish cystic, segmental, and fusiform dilatations of the intrahepatic biliary tree in type IV cysts. In patients with segmental left intrahepatic cystic dilatations, combined left liver lobectomy and extrahepatic cyst excision is suggested to decrease late postsurgical biliary complications.
回顾接受胆管囊肿手术的成年患者的特征，重点关注胆管树的解剖结构以及长期预后。
胆管囊肿（BDC）在西方国家并不常见，大多数报道的病例来自亚洲。日本学者强调肝外和肝内胆管扩张经常同时存在，但基于托达尼分类法对患者进行分级时，常因缺乏对IC型和IVA 型囊肿的准确定义而受到阻碍。此外，尽管肝外囊肿切除术的应用越来越多，但对于肝内胆管受累患者的长期预后了解甚少。
1980年至1992年间，法国外科研究协会的17家机构对42例成年BDC患者进行了治疗。分析了临床表现、术前影像学评估和手术方式。术后长期预后来自患者病历、主治医生或直接与患者接触。
12例患者（30%）自幼反复出现腹痛或黄疸。7例（17%）曾接受过囊肿肠吻合术。21例（50%）为托达尼IVA型囊肿，伴有肝外和肝内胆管受累。其中，9例为节段性、仅左侧肝内胆管扩张。5例患者（12%）发生胆管癌。34例I型或IV型囊肿患者接受了肝外囊肿切除并 Roux-en-Y 肝空肠吻合术。总体手术死亡率为2.4%。长期结果与囊肿类型明显相关：在平均8.4年的随访期间，12例接受I型囊肿切除治疗的患者中有11例（92%）无症状，而接受IV型囊肿手术的患者中有31%出现发作性或严重胆管炎并伴有肝内结石。
对于BDC患者，必须特别关注相关的肝内胆管扩张。我们建议对托达尼分类法进行修改，以区分IV型囊肿中肝内胆管树的囊性、节段性和梭形扩张。对于节段性左侧肝内囊性扩张患者，建议联合左肝叶切除术和肝外囊肿切除术，以减少术后晚期胆道并发症。
