Branchi Vittorio, Weismüller Tobias J, Zhou Taotao, Henn Jonas, Semaan Alexander, Glowka Tim R, Gonzalez-Carmona Maria, Strassburg Christian, Kalff Jörg C, Manekeller Steffen, Matthaei Hanno
Klinik und Poliklinik für Allgemein‑, Viszeral‑, Thorax- und Gefäßchirurgie, Universitätsklinikum Bonn, Venusberg-Campus 1, 53127, Bonn, Deutschland.
Medizinische Klinik und Poliklinik I, Universitätsklinikum Bonn, Venusberg-Campus 1, 53217, Bonn, Deutschland.
Chirurg. 2021 Feb;92(2):148-157. doi: 10.1007/s00104-020-01197-5.
In recent years substantial progress has been made in the treatment, surveillance and understanding of the pathogenesis of primary sclerosing cholangitis (PSC); however, in most cases liver transplantation (LTX) is still the only curative option for cancer or end-stage liver disease (ELD). In rare cases a partial liver resection is a possible curative treatment of a PSC-associated cholangiocellular carcinoma (CCC). These operations represent a significant additional burden for PSC patients.
Due to the rarity of PSC detailed studies regarding hepato-pancreato-biliary (HPB) surgery are lacking. The aim of this study was to analyze the surgical indications and prognosis of PSC patients.
A single center retrospective cohort study from 1990 to 2020 was carried out. In this study patients with PSC were included and investigated with respect to factors associated with surgery and the prognosis.
As a consequence of PSC-associated conditions, in 62 patients (36%) a major HPB operation or explorative laparotomy was necessary. The prevalence of chronic inflammatory bowel disease was significantly higher in these patients (P < 0.019). An LTX was carried out in 46 patients (73%) because of ELD. A liver resection (LR) was performed in 8 patients (11%) and 9 patients only underwent an explorative laparotomy. The overall survival in the LTX subgroup was significantly longer than patients who underwent LR and explorative laparotomy (258 months; 95% confidence interval, CI 210-306 months vs. 88 months; 95% CI 16-161 months vs. 13 months; 95% CI 3-23 months; p < 0.05, respectively).
The majority of patients with PSC have to be operated on because of the disease with substantial risks for morbidity and mortality. Curative treatment options are lacking, thus underlining the need for effective early detection and treatment strategies for PSC-CCC.
近年来,在原发性硬化性胆管炎(PSC)的治疗、监测及发病机制的理解方面取得了重大进展;然而,在大多数情况下,肝移植(LTX)仍是治疗癌症或终末期肝病(ELD)的唯一治愈选择。在极少数情况下,部分肝切除术是治疗PSC相关胆管细胞癌(CCC)的一种可能的治愈性治疗方法。这些手术给PSC患者带来了巨大的额外负担。
由于PSC病例罕见,缺乏关于肝胰胆(HPB)手术的详细研究。本研究的目的是分析PSC患者的手术指征和预后。
进行了一项1990年至2020年的单中心回顾性队列研究。本研究纳入了PSC患者,并对与手术及预后相关的因素进行了调查。
由于PSC相关疾病,62例患者(36%)需要进行大型HPB手术或剖腹探查术。这些患者中慢性炎症性肠病的患病率显著更高(P<0.019)。46例患者(73%)因ELD接受了肝移植。8例患者(11%)进行了肝切除术(LR),9例患者仅接受了剖腹探查术。肝移植亚组的总生存期明显长于接受LR和剖腹探查术的患者(258个月;95%置信区间,CI 210 - 306个月 vs. 88个月;95%CI 16 - 161个月 vs. 13个月;95%CI 3 - 2三个月;P<0.05)。
大多数PSC患者因该病需要进行手术,存在较高的发病和死亡风险。缺乏治愈性治疗选择,因此强调了对PSC-CCC进行有效早期检测和治疗策略的必要性。