Choledochal Cyst

胆总管囊肿
  • 文章类型: Journal Article
    背景:先前的分型方法无法为肝外胆总管囊肿(ECC)的手术复杂性提供预测性见解。本研究旨在通过对成像结果的聚类,建立一种新的ECC分类系统。此外,它旨在比较已确定的ECC类型之间的差异,并评估手术难度的水平。
    方法:通过K均值聚类分析对124例患者的影像学数据进行自动分组。根据新分组的特点,进行了纠正和干预,以建立新的分类。人口统计数据,临床表现,手术参数,并发症,再操作,并根据不同类型对预后指标进行分析。还评估了导致手术时间延长的因素。
    结果:ECC的新分类系统:类型A(上段),B型(中段),C型(下段),和D型(整个胆管)。合并症(结石或感染)的发生率差异有统计学意义(P=0.000,P=0.002)。此外,术后胆管炎发生率差异有统计学意义(P=0.046).两组手术时间差异有统计学意义(P=0.001)。年龄,BMI>30,分类,合并结石的存在与手术时间延长显著相关(P=0.002,P=0.000,P=0.011,P=0.011)。
    结论:结论:我们利用机器学习驱动的聚类分析,创造了一种新颖的肝外胆管扩张类型学.这个分类,结合年龄等因素,联合结石发生,肥胖,显著影响腹腔镜胆总管囊肿手术的复杂性,为改进手术治疗提供有价值的见解。
    BACKGROUND: Prior typing methods fail to provide predictive insights into surgical complexities for extrahepatic choledochal cyst (ECC). This study aims to establish a new classification system for ECC through clustering of imaging results. Additionally, it seeks to compare the differences among the identified ECC types and assess the levels of surgical difficulty.
    METHODS: The imaging data of 124 patients were automatically grouped through a K-means clustering analysis. According to the characteristics of the new grouping, corrections and interventions were carried out to establish a new classification. Demographic data, clinical presentations, surgical parameters, complications, reoperation, and prognostic indicators were analyzed according to different types. Factors contributing to prolonged surgical time were also evaluated.
    RESULTS: A new classification system of ECC: Type A (upper segment), Type B (middle segment), Type C (lower segment), and Type D (entire bile duct). The incidences of comorbidities (calculus or infection) were significantly different (P=0.000, P=0.002). Additionally, variations in the incidence of postoperative cholangitis were statistically significant (P=0.046). The operative time was significantly different between groups (P=0.001). Age, BMI > 30, classification, and the presence of combined stones exhibit a significant association with prolonged operative time (P=0.002, P=0.000, P=0.011, P=0.011).
    CONCLUSIONS: In conclusion, our utilization of machine learning-driven cluster analysis has enabled the creation of a novel extrahepatic biliary dilatation typology. This classification, in conjunction with factors like age, combined stone occurrence, and obesity, significantly influences the complexity of laparoscopic choledochal cyst surgery, offering valuable insights for improved surgical treatment.
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  • 文章类型: Journal Article
    胆总管囊肿(CC)由于胚胎学和病因密切相关,可以更好地称为胆道畸形。与巴比特的反流假设相反,损伤和扩张,在所有CC品种中,都不能证明反流是致病因素。胆道系统的高压,否则称为导管高血压,作为解释CC演变的替代方案。我的水果类型,在标准分类中找不到位置,典型的导管高血压假说。因此更接近,深入的回顾将能够突出胆道畸形这一恰当的术语。
    The choledochal cyst (CC) can be better termed as biliary tract malformation because of the close association of embryology and etiology in the causation of CC. Contrary to Babbitt\'s postulation of reflux, damage and dilatation, reflux was not demonstrable as the causative factor in all varieties of CC. High pressure in the biliary system, otherwise termed ductal hypertension, is put forth as an alternative to explain the evolution of CC. The forme fruste type, which does not find a place in the standard classification, typifies the ductal hypertension hypothesis. Hence a closer, in-depth review would be able to highlight this apt terminology of biliary tract malformation.
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  • 文章类型: Journal Article
    背景:基质金属蛋白酶-7(MMP-7)与胆道损伤有关。本研究旨在评估胆总管囊肿(CDC)患儿血清MMP-7与临床特征的关系。
    方法:在2020年6月至2022年7月之间,我们对在我们中心进行了一期明确手术的CDC进行了前瞻性研究。使用酶联免疫吸附测定法测量血清MMP-7。我们评估了血清MMP-7与年龄之间的关系,实验室测试,影像学检查,肝纤维化,MMP-7表达,和穿孔。
    结果:共328个CDCs被纳入研究,血清MMP-7中位数为7.67ng/mL。较高的血清MMP-7与诊断时年龄较小相关(p<0.001),囊肿较大(p<0.001),肝纤维化分期较高(p<0.001),穿孔发生率较高(p<0.01)。肝脏MMP-7主要表达于肝内外胆管上皮细胞。血清MMP-7预测穿孔的受试者工作特征曲线下面积(AUROC)为0.630(p<0.001)。当血清MMP-7与γ-谷氨酰转移酶(GGT)联合时,AUROC增加到0.706(p<0.001)。
    结论:血清MMP-7与CDCs胆道梗阻相关。血清MMP-7高的患者更容易发生严重的肝损伤和胆道损伤,肝纤维化和穿孔的发生率较高。
    BACKGROUND: Matrix metalloproteinase-7 (MMP-7) is associated with biliary injury. This study aimed to evaluate the relationships of serum MMP-7 with clinical characteristics in choledochal cysts (CDC) children.
    METHODS: Between June 2020 and July 2022, we conducted a prospective study of CDCs who underwent one-stage definitive operation at our center. Serum MMP-7 was measured using an enzyme-linked immunosorbent assay. We evaluated the relationships between serum MMP-7 and age, laboratory tests, imaging examinations, liver fibrosis, MMP-7 expression, and perforation.
    RESULTS: A total of 328 CDCs were enrolled in the study, with a median serum MMP-7 of 7.67 ng/mL. Higher serum MMP-7 was correlated with younger age at diagnosis (p < 0.001), larger cyst sizes (p < 0.001), higher liver fibrosis stages (p < 0.001), and higher incidence of perforation (p < 0.01). Liver MMP-7 was mainly expressed in intrahepatic and extrahepatic biliary epithelial cells. The area under the receiver operating characteristic curve (AUROC) was 0.630 (p < 0.001) for serum MMP-7 in predicting perforation. When serum MMP-7 was combined with γ-glutamyl transferase (GGT), the AUROC increased to 0.706 (p < 0.001).
    CONCLUSIONS: Serum MMP-7 was associated with biliary obstruction in CDCs. Patients with high serum MMP-7 were more likely to have severe liver damage and biliary injury, with higher incidences of liver fibrosis and perforation.
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  • 文章类型: Journal Article
    目的是研究小儿胆总管囊肿(CC)患者肝脏的各种组织病理学变化,并与囊肿的表现和类型相关。
    在一项包括所有接受CC切除的儿科患者的前瞻性观察研究中,胆汁淤积(CHS)形式的肝脏组织病理学变化,门静脉炎症(PI),胆管增生(BDP),使用评分系统对纤维化进行研究和分级。对它们进行了年龄分析,性别,症状,和囊肿的类型。
    所有30例CC患者均以CHS的形式在肝脏中表现出不同程度的组织病理学变化,PI,BDP,和肝纤维化。<1年的患者有9/13(69.2%)的囊性变种,>1年的患者有17/17(100%)的梭形变种CC(P<0.001)。<1年的患者常表现为黄疸和肝肿大,>1年的患者表现为腹痛(P<0.002)。与梭形变种相比,囊性变种的肝纤维化和BDP等级更高(P<0.001)。然而,与CHS和PI无显著相关性(P>1.23)。
    在所有CC患者中都可以看到不同等级的肝脏组织病理学变化。CC<1年的患者常出现黄疸,有囊性类型,在组织病理学上有较高程度的肝脏损害。
    UNASSIGNED: The aim is to study the various histopathological changes in the liver in pediatric patients with choledochal cyst (CC) and correlate with the presentation and type of cyst.
    UNASSIGNED: In a prospective observational study including all pediatric patients who underwent CC excision, histopathological changes of the liver in the form of cholestasis (CHS), portal inflammation (PI), bile duct proliferation (BDP), and fibrosis were studied and graded using a scoring system. They were analyzed in relation to age, sex, symptoms, and type of the cyst.
    UNASSIGNED: All 30 patients of CC showed various degrees of histopathological changes in the liver in the form of CHS, PI, BDP, and liver fibrosis. Patients <1 years had 9/13 (69.2%) cystic variety and those >1 years had 17/17 (100%) fusiform variety of CC (P < 0.001). Patients <1 years frequently presented with jaundice and hepatomegaly and those >1 years presented with pain abdomen (P < 0.002). Higher grades of liver fibrosis and BDP were seen in the cystic variety compared to the fusiform variety (P < 0.001). However, no significant association was found with CHS and PI (P > 1.23).
    UNASSIGNED: Histopathological changes in the liver of varying grades are seen in all patients of CC. Patients of CC <1 year presented frequently with jaundice, had the cystic type, and had a higher degree of liver damage on histopathology.
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  • 文章类型: Journal Article
    机器人手术(RS)具有3维视图的优点,光学放大,运动缩放,和改进的人体工程学和自由度。尽管最近在儿科患者中广泛进行RS,仍然有许多限制和模糊的迹象。这项研究的目的是报告我们在单个中心对儿科患者进行RS的早期经验。
    使用daVinciXi手术平台接受RS的患者的电子病历(直观的手术,Inc.)于2019年11月至2021年8月在首尔国立大学儿童医院进行了回顾性审查。中位随访时间为21.0个月(范围,12.3-31.8个月)。进行了一项在线调查,以调查对机器人手术疤痕的满意度。
    54名患者接受了机器人手术(手术时的中位年龄,11.1年[范围,0.1-17.8年])。在我们的医院里,病人有20种不同的机器人手术,包括胆总管囊肿切除和肝空肠吻合术,卵巢肿块切除术,和其他人。中位操作时间和控制台时间为157.5分钟(范围,45-505分钟)和40分钟(范围,11-360分钟),分别。所有病例均未转换为开腹或腹腔镜方法。术后并发症5例。根据一项在线调查,超过一半的患者(60.9%)回答他们对疤痕感到满意。
    我们的早期经验证明了RS在具有一系列诊断和复杂程序的儿童中的安全性和可行性。有了更多的经验,RS可以替代小儿患者的传统开放或腹腔镜手术。需要进一步的研究来阐明小儿RS的适应症。
    UNASSIGNED: Robotic surgery (RS) has the advantages of 3-dimensional view, optical magnification, motional scaling, and improved ergonomics and degree of freedom. Although RS has widely been performed on pediatric patients lately, there are still numerous restrictions and ambiguous indications. The purpose of this study was to report our early experience with RS on pediatric patients at a single center.
    UNASSIGNED: Electronic medical records of patients who underwent RS with the da Vinci Xi surgical platform (Intuitive Surgical, Inc.) in Seoul National University Children Hospital from November 2019 to August 2021 were reviewed retrospectively. The median follow-up was 21.0 months (range, 12.3-31.8 months). An online survey was conducted to investigate satisfaction with robotic surgical scars.
    UNASSIGNED: Fifty-four patients underwent robotic surgeries (median age at operation, 11.1 years [range, 0.1-17.8 years]). In our hospital, patients had 20 different kinds of robotic surgeries, including choledochal cyst excision with hepaticojejunostomy, ovarian mass excision, and others. Median operation time and console time were 157.5 minutes (range, 45-505 minutes) and 40 minutes (range, 11-360 minutes), respectively. All cases were done without conversion into open or laparoscopic methods. Postoperative complications were found in 5 patients. According to an online survey, over half of patients (60.9%) answered that they felt satisfied with scars.
    UNASSIGNED: Our early experience demonstrated the safety and feasibility of RS in children with a range of diagnoses and complicated procedures. With more experience, RS could be an alternative to traditional open or laparoscopic operations in pediatric patients. Further studies are needed to clarify indications of pediatric RS.
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  • 文章类型: Case Reports
    巨大的胆总管囊肿很少见,所以关于最佳手术治疗方法的数据很少。我们在这里介绍,一例巨大的胆总管囊肿经腹腔镜切除成功。一名37岁女性,表现为右上腹痛和轻度黄疸。在检查中,她有一个右上腹部肿块,在影像学上观察到是一个巨大的IVa型胆总管囊肿,测量约129毫米×190毫米。她的血液检查结果显示肝功能异常。我们成功地进行了囊肿的腹腔镜切除术,病人恢复良好,术后八天出院,没有任何并发症。我们希望分享此罕见病例的经验,并为将来诊断和使用腹腔镜切除术治疗巨大胆总管囊肿提供一些临床依据。
    Giant choledochal cysts are rare, and so little data exist on the best surgical treatment method. We present here, a case of a giant choledochal cyst that was successfully excised by laparoscopic resection. A 37-year-old female presented with right upper abdominal pain and mild jaundice. On examination she had a right upper abdominal mass which on imaging was observed to be a giant choledochal cyst of type IVa, measuring approximately 129 mm × 190 mm. Her blood test results showed abnormal liver function. We successfully performed laparoscopic resection of the cyst, the patient recovered well and was discharged from hospital eight days post-operation without any complications. We wish to share the experience of this rare case and provide some clinical basis for future diagnosis and use of laparoscopic resection in the treatment of giant choledochal cysts.
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  • 文章类型: Journal Article
    目的:分析磁共振(MR)对囊性胆道闭锁(CBA)和胆总管囊肿(CC)的鉴别能力。
    方法:对2010年1月至2023年7月诊断为CBA或CCI/IV型的婴儿(≤1岁)进行回顾性分析。比较CBA和CC组的MR影像学特征。分析二元逻辑回归和受试者工作特征曲线下面积(AUC)以鉴定CBA。
    结果:63例CBA患者(中位年龄,30天)和172例CC患者(中位年龄,包括60天)。胆囊(GB)壁厚(截止,1.2mm)显示98.4%的灵敏度和100%的特异性(AUC,0.998).MR-三角线厚度(MR-TCT)(截止,4.1mm)显示出100%的灵敏度和95.9%的特异性(AUC,0.986).胆管环可视化显示96.8%的灵敏度和100%的特异性(AUC,0.984)。近端胆管(PBD)直径(截止,1.3毫米)显示92.1%的灵敏度和95.3%的特异性(AUC,0.977)。囊壁厚度(截止,1mm)显示77.8%的灵敏度和95.3%的特异性(AUC,0.942)。GB壁厚>1.2mm和MR-TCT>4.1mm的组合,GB壁厚>1.2mm和回路可视化,GB壁厚>1.2mm,和囊肿壁厚度>1毫米显示100%的灵敏度和100%的特异性(AUC,1.000)。
    结论:磁共振成像特征可用于识别CBA和CC,以及GB壁厚和MR-TCT的组合,或循环可视化,或囊肿壁厚度,具有完美的诊断价值。
    结论:对CBA和CC进行早期准确的鉴别至关重要,但是目前的方法依赖于固有的主观超声。MRI上的胆道特征允许客观,准确的诊断。
    OBJECTIVE: To analyze the ability of magnetic resonance (MR) to identify cystic biliary atresia (CBA) and choledochal cyst (CC).
    METHODS: Infants (≤ 1 year old) who were diagnosed with CBA or CC type I/IV from January 2010 to July 2023 were retrospectively reviewed. Imaging characteristics on MR were compared between the CBA and CC groups. Binary logistic regression and the area under the receiver operating characteristic curve (AUC) were analyzed for the identification of CBA.
    RESULTS: Sixty-three patients with CBA (median age, 30 days) and 172 patients with CC (median age, 60 days) were included. Gallbladder (GB) wall thickness (cutoff, 1.2 mm) showed 98.4% sensitivity and 100% specificity (AUC, 0.998). MR-triangular cord thickness (MR-TCT) (cutoff, 4.1 mm) showed 100% sensitivity and 95.9% specificity (AUC, 0.986). The bile duct loop visualization showed 96.8% sensitivity and 100% specificity (AUC, 0.984). Proximal bile duct (PBD) diameter (cutoff, 1.3 mm) showed 92.1% sensitivity and 95.3% specificity (AUC, 0.977). Cyst wall thickness (cutoff, 1 mm) showed 77.8% sensitivity and 95.3% specificity (AUC, 0.942). The combination of GB wall thickness > 1.2 mm and MR-TCT > 4.1 mm, GB wall thickness > 1.2 mm and loop visualization, GB wall thickness > 1.2 mm, and cyst wall thickness > 1 mm showed 100% sensitivity and 100% specificity (AUC, 1.000).
    CONCLUSIONS: Imaging characteristics on MR might be used to identify CBA and CC, and the combination of GB wall thickness and MR-TCT, or loop visualization, or cyst wall thickness, has a perfect diagnostic value.
    CONCLUSIONS: Early and accurate differentiation of CBA and CC is essential, but current methods rely on inherently subjective ultrasound. Biliary features on MRI allow for an objective, accurate diagnosis.
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  • 文章类型: Journal Article
    背景:胆总管囊肿伴穿孔(CC伴穿孔)很少发生,早期诊断和及时的治疗方案是治疗合并穿孔的关键。本研究旨在预测穿孔CC的发生。
    方法:所有1111例患者2011年1月至2022年10月在我们医院接受了胆总管囊肿手术。我们进行了单变量和多变量逻辑回归分析,以筛选预测穿孔CC的独立预测因素。在此基础上建立了一个列线图。使用受试者工作特征(ROC)曲线评估列线图的预测性能,校准图,和决策曲线分析(DCA)曲线。
    结果:胆总管囊肿穿孔患儿的年龄主要集中在1至3岁之间。Logistic回归分析表明,年龄,丙氨酸氨基转移酶,谷氨酰转肽酶,C反应蛋白,呕吐,黄疸,腹胀,腹泻与预测胆总管囊肿穿孔的发生有关。ROC曲线,校准图,和DCA曲线分析曲线表明,列线图具有很大的判别能力和校准能力,以及显著的临床效用。
    结论:CC伴穿孔的年龄主要集中在1~3岁之间。建立了预测胆总管囊肿穿孔的列线图。
    BACKGROUND: Choledochal cyst with perforation (CC with perforation) rarely occurs, early diagnosis and timely treatment plan are crucial for the treatment of CC with perforation. This study aims to forecast the occurrence of CC with perforation.
    METHODS: All 1111 patients were conducted, who underwent surgery for choledochal cyst at our hospital from January 2011 to October 2022. We conducted univariate and multivariate logistic regression analysis to screen for independent predictive factors for predicting CC with perforation, upon which established a nomogram. The predictive performance of the nomogram was evaluated using receiver operating characteristic (ROC) curves, calibration plots, and decision curve analysis (DCA) curves.
    RESULTS: The age of children with choledochal cyst perforation is mainly concentrated between 1 and 3 years old. Logistic regression analysis indicates that age, alanine aminotransferase, glutamyl transpeptidase, C-reactive protein, vomiting, jaundice, abdominal distension, and diarrhea are associated with predicting the occurrence of choledochal cyst perforation. ROC curves, calibration plots, and DCA curve analysis curves demonstrate that the nomogram has great discriminative ability and calibration, as well as significant clinical utility.
    CONCLUSIONS: The age of CC with perforation is mainly concentrated between 1 and 3 years old. A nomogram for predicting the perforation of choledochal cyst was established.
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  • 文章类型: Review
    背景:胆道上皮内瘤变(BilIN),胆管癌的非侵入性前体,可以表现为恶性转化。由于胆管癌(CCA)可能由于胆管和胆囊的慢性炎症而进展,胆总管囊肿被认为是CCA的前兆。然而,Bilin在儿童中很少被报道,到目前为止。
    方法:我们回顾了患者的医疗记录(<18岁,n=329),从2008年至2022年在Asan医学中心接受了胆总管囊肿切除术。在15例患者中诊断出BilIN。随后对人口统计学进行了分析,外科手术,临床课程,以及这些患者的结果。进行亚组分析和多因素logistic回归检验以确定影响BilIN发生的因素。
    结果:纳入本研究的患者平均年龄为40.1±47.6个月。在15名患者中,诊断出各种等级的Bilin。TodaniI型在80%的患者中普遍存在。手术时的中位年龄为17个月。在平均63.3±94.0个月的随访中,未观察到不良事件,如残余胰内胆总管和肝内胆管结石或胆管癌,表明到目前为止是有利的结果。
    结论:儿童胆总管囊肿可能进展为BilIN。这些结果可以强调早期和全面切除胆总管囊肿的重要性。包括相关病变的切除切缘,以及对患有BilIN或有BilIN风险的患者进行更彻底的术后监测。
    BACKGROUND: Biliary intraepithelial neoplasia (BilIN), a noninvasive precursor of cholangiocarcinoma, can manifest malignant transformation. Since cholangiocarcinoma (CCA) may progress due to chronic inflammation in the bile ducts and gallbladder, choledochal cysts are considered a precursor to CCA. However, BilIN has rarely been reported in children, to date.
    METHODS: We reviewed medical records of patients (< 18 years of age, n = 329) who underwent choledochal cyst excision at Asan Medical Center from 2008 to 2022. BilIN was diagnosed in 15 patients. Subsequent analyses were performed of the demographics, surgical procedures, clinical course, and outcomes in these patients. Subgroup analysis and multivariate logistic regression test were performed to identify factors influencing BilIN occurrence.
    RESULTS: The mean age of the patients included in our study was 40.1 ± 47.6 months. In 15 patients, BilIN of various grades was diagnosed. Todani type I was prevalent in 80% of the patients. The median age at surgery was 17 months. During a mean follow-up of 63.3 ± 94.0 months, no adverse events such as stone formation in the remnant intrapancreatic common bile duct and intrahepatic duct or cholangiocarcinoma were observed, indicating a favorable outcome until now.
    CONCLUSIONS: The potential progression of choledochal cysts to BilIN in children was demonstrated. These results could underscore the importance of early and comprehensive excision of choledochal cysts, including resection margins for associated lesions and more thorough postoperative surveillance in patients with or at risk of BilIN.
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  • 文章类型: Journal Article
    腹腔镜和机器人手术是对外科医生手眼协调能力的挑战,这需要不断的练习。传统的导师培训正在逐步转向基于各种模式的模拟训练。腹腔镜和机器人胆道吻合术是肝胆外科中的一项重要而困难的手术。我们构建并优化了可重复使用的模块化3D打印胆总管囊肿模型。这项研究的目的是验证该优化模型区分具有不同熟练程度的外科医生的能力以及重复练习的好处。共有12名不同级别的外科医生参加了这项研究。记录手术完成时间和OSATS评分。通过李克特量表对模型进行了验证。在使用该模型进行腹腔镜或机器人胆肠吻合术之前,向外科医生显示了步骤和内容。不同经验水平的外科医生在对该模型进行腹腔镜胆肠吻合术时表现出不同的水平。重复训练可显著缩短腹腔镜胆肠吻合术的时间,提高不同经验水平的外科医生的手术成绩。同时,初步结果表明,外科医生在家用机器人平台上的表现与他们的腹腔镜技能基本一致。该模型可以区分具有不同经验水平的外科医生,并可以通过反复练习提高手术技能。值得注意的是,为了得出更可靠的结论,将来应该收集更多的受试者,做更多的实验。
    Laparoscopic and robotic surgery is a challenge to the surgeon\'s hand-eye coordination ability, which requires constant practice. Traditional mentor training is gradually shifting to simulation training based on various models. Laparoscopic and robotic bilioenteric anastomosis is an important and difficult operation in hepatobiliary surgery. We constructed and optimized the reusable modular 3D-printed models of choledochal cyst. The aim of this study was to verify the ability of this optimized model to distinguish between surgeons with different levels of proficiency and the benefits of repeated practice. A total of 12 surgeons with different levels participated in the study. Operation completion time and OSATS score were recorded. The model was validated by Likert scale. Surgeons were shown the steps and contents before performing laparoscopic or robotic bilioenteric anastomosis using the model. Surgeons with different levels of experience showed different levels when performing laparoscopic bilioenteric anastomosis on this model. Repeated training can significantly shorten the time of laparoscopic bilioenteric anastomosis and improve the operation scores of surgeons with different levels of experience. At the same time, preliminary results have shown that the performance of surgeons on the domestic robotic platform was basically consistent with their laparoscopic skills. This model may distinguish surgeons with different levels of experience and may improve surgical skills through repeated practice. It is worth noting that in order to draw more reliable conclusions, more subjects should be collected and more experiments should be done in the future.
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