Medium-term outcomes after laparoscopic revision of laparoscopic Kasai portoenterostomy in patients with biliary atresia

Medium-term outcomes after laparoscopic revision of laparoscopic Kasai portoenterostomy in patients with biliary atresia | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Research Medium-term outcomes after laparoscopic revision of laparoscopic Kasai portoenterostomy in patients with biliary atresia Yi Ji, Xuepeng Zhang, Siyuan Chen, Yanan Li, Kaiying Yang, Jiangyuan Zhou, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-70828/v2 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 30 Apr, 2021 Read the published version in Orphanet Journal of Rare Diseases → Version 2 posted 9 You are reading this latest preprint version Show more versions Abstract Objective: To determine whether revision laparoscopic Kasai portoenterostomy (RLKPE) is a viable treatment option for patients with biliary atresia (BA) who underwent initially successful laparoscopic Kasai portoenterostomy (ILKPE). Methods: The medical records of 312 patients with nonsyndromic BA who underwent ILKPE between May 2009 and May 2017 were retrospectively reviewed. The patients were divided into three groups according to their outcomes after ILKPE: group A: 25 patients who underwent RLKPE; group B: 203 patients who underwent ILKPE and required no further surgical intervention; and group C: 84 patients with failed ILKPE who either died or required liver transplantation for survival. The 3-year and 5-year survival with native liver (SNL) rates were compared between groups A and B C. Among the 25 patients in group A, the perioperative data of RLKPE were compared with those of ILKPE. Results: Of the 312 patients who underwent ILKPE, 228 reached the normal bilirubin concentration range within 6 months postoperatively. Among them, 25 patients with a sudden cessation of bile flow underwent RLKPE. Adequate biliary drainage evidenced by normalized conjugated bilirubin levels was achieved in 80% of the patients who underwent RLKPE. The perioperative variables, including operative time, blood loss, rate of conversion to open surgery and complications of RLKPE, were not significantly different between RLKPE and ILKPE. The 3-year and 5-year SNL rates in patients after RLKPE were 64.0% and 52.0%, respectively, which were not significantly different from the corresponding 86.2% and 73.9% in patients after unrevised ILKPE ( P >0.05). Conclusion: Our data demonstrated that RPLKE can be a viable and effective treatment opinion in patients who experience sudden cessation of bile drainage after ILKPE. RPLKE can delay the need for liver transplantation, yielding encouraging medium-term patient outcomes. Internal Medicine Biliary atresia Laparoscopic Kasai portoenterostomy Revision Outcomes Figures Figure 1 Introduction Biliary atresia (BA) is a progressive fibrous occlusive disease that, if not treated promptly and effectively, may lead to cirrhosis or end-stage liver disease [1]. Although liver transplantation (LT) significantly improves the prognosis of patients with BA, Kasai portoenterostomy is still regarded as the standard initial option due to the technical complexity associated with liver transplantation in young children and the shortage of qualified donors [2, 3]. In 2002, Esteves [4] developed the laparoscopic Kasai portoenterostomy (LKPE) procedure, which created a new era of minimally invasive surgery for BA. Although a learning curve of LKPE is present, some satisfactory results have been achieved, in which the 3-year and 5-year survival with native liver (SNL) rates after LKPE were not inferior, or even superior, to those after open Kasai portoenterostomy (OKPE) [5-10]. Unfortunately, LKPE is not effective in every patient with BA, as is the case for OKPE. In patients who experienced sudden cessation of bile drainage after initially successful LKPE (ILKPE), the necessity and indications for revision LKPE (RLKPE) have not been previously reported. Herein, we review and analyze our experiences with RLKPE in patients with BA. 1. Materials And Methods 2.1 Design and study population This study was approved by the Ethical Committee of the West China Hospital of Sichuan University. The patients’ parents or guardians gave written informed consent. The diagnosis of BA was based on a combination of ultrasonic findings, intraoperative cholangiography findings, and histological examinations of the hepatic parenchyma and extrahepatic bile ducts. Patients with BA splenic malformation, severe cardiac anomalies, or other significant comorbidities that would influence the postoperative course were excluded from the study. The 312 patients with nonsyndromic BA who underwent LKPE from May 2009 to May 2017 were enrolled in this study. They were divided into 3 cohorts based on their outcome after ILKPE: (1) the 25 patients in group A underwent RLKPE; (2) the 203 patients in group B underwent ILKPE and required no further surgical intervention; and (3) the 84 patients in group C experienced failed ILKPE and either died or required liver transplantation for survival. The indications for revision were the sudden cessation of bile drainage after ILKPE and no improvement after 2 weeks of antibiotic treatment. The 3-year and 5-year SNL rates were compared between groups A and B and between groups A and C. Among the patients in group A, the perioperative data, rates of clearance of jaundice (CJ) and cholangitis of RLKPE were compared with those of ILKPE. 2.2 Operative technique 2.2.1 The LKPE procedure The LKPE procedure was similar to that described in a previously published paper [5-7]. Succinctly, a percutaneous suture was used to snare the round and falciform ligament and to retract the liver. The atretic gallbladder and cystic duct are dissected free from the gallbladder fossa. The portal plate over the bifurcation of the main portal vein could be found along the common hepatic duct. Once the base of the fibrous cone was reached, the fibrous cord was transected with laparoscopic scissors. The resection level of the fibrous cone was dependent on the presence of abundant bile-like juice over the fibrous stump. Bleeding from the fibrous remains of the portal plate was controlled by direct compression with gauze pads. The ligament of Treitz was identified, and the jejunum 15 cm distal to the ligament was exteriorized through the umbilical port site. A 25-40 cm Roux-en-Y limb was fashioned. The Roux loop was delivered to the hilum through a retrocolic path. One layer of end-to-side portoenterostomy (diameter of the anastomosis 1.0-1.5 cm) was performed with interrupted 5-0 absorbable sutures. A drain was inserted into the foramen of Winslow. 2.2.2 The RLKPE procedure The trocar placement and pneumoperitoneum pressure settings were the same as those in LKPE, as mentioned above. The other steps of RLPKE were as follows: (1) removing the greater omentum adhered to the porta hepatis; (2) partly dismantling the anastomosed Roux limb adjacent to the porta hepatis; (3) resecting the granulation tissue; (4) dissecting the hilar fibrous stump layer by layer until abundant bile-like juice was observed; and (5) carrying out end-to-end portoenterostomy after trimming the tail of the Roux limb. 2.3 The management algorithm after surgery Postoperatively, the BA patients in the 3 groups received the same medical treatment protocol. Antibiotic therapy was continued intravenously for 25-30 days in the hospital. Methylprednisolone was administered intravenously 5 days postoperatively at a dose of 5 mg/kg/day initially and was reduced by 1 mg/kg/day every 3 days for 2 weeks or longer until a normal total bilirubin value was reached. Sulfamethoxazole and cephalosporin antibiotics were orally administered alternately weekly until 1 year of age. Ursodeoxycholic acid and hepatoprotective tablets were used until 3 years of age. 2.4 Definitions The operative time (ORT) was calculated as the length of time between the skin incision and closure. Any perioperative complications (APOC) was defined as a complication that occurred during the perioperative period, including wound infection, umbilical hernia, intestinal anastomotic fistula, and adhesive intestinal obstruction. CJ was defined as a total bilirubin level 2.5 mg/dL), leukocytosis with left shift, and normal to acholic stools in a febrile patient (>38.0 °C). Postoperative outcomes were followed through outpatient clinic evaluations and were excluded if the patients underwent liver transplantation or died. 2.5 Statistics The software applied for statistical calculations was SPSS 22.0 for Windows 10.0 (SPSS Inc., Chicago, IL, USA). The demographic data of BA patients were compared using one-way analysis of variance and the chi-squared test. The difference in SNL rate among the patients was analyzed by the Kaplan–Meier method with endpoints of death or liver transplantation and compared using the log-rank test. A P value <0.05 was considered statistically significant. 2. Results 2.1 Demographic and clinical characteristics of all patients in the three groups From May 2009 to May 2019, 312 patients with nonsyndromic BA underwent ILKPE. The demographic and clinical characteristics of all patients in the three groups are presented in Table 1. The median age at operation was 82.8±19.4 days, with a male proportion of 32.1%. The proportions of types I, II, and III and cystic BA were 1.6%, 4.8%, 82.4% and 11.2%, respectively. The median follow-up time was 67.2±25.5 months. The rates of CJ and cholangitis after ILKPE were 73.1% and 58.9%, respectively. The 3-year and 5-year SNL rates were 62.6% and 54.1%, respectively. 2.2 Comparison between group A and group B In group A, 25 patients underwent RLKPE. The male proportion, age and follow-up time were not significantly different between groups A and B. The 3-year and 5-year SNL rates were 64.0% and 52.0% in group A and were not significantly different from the 86.2% and 73.9% in group B ( P >0.05) (Fig 1). 2.3 Comparison of perioperative variables and rates of CJ and cholangitis between ILKPE and RLKPE in 25 patients The demographic and clinical characteristics of 25 patients who underwent both ILKPE and RLKPE are presented in Table 2. The ages at ILKPE and RLKPR were 76.7±15.9 days and 159.8±50.4 days, respectively. The time interval from suspension of bile drainage to revision was 21.9±8.0 days. The ORT, blood loss, conversion rate and incidence of AOPC during ILKPE were not significantly different from those during RLKPE. The rates of CJ and cholangitis after ILKPE were 100% and 64.0% and were significantly different from the 80.0% and 28.0% after RLKPE ( P <0.01). 3. Discussion Experiences with revision for BA patients with recurrent jaundice after initial OKPE or ILKPE have rarely been reported. In two studies, CJ rates of 60% [11] and 83.3% [12] were obtained after revision of OKPE for patients with initially successful OKPE. Naruhiko et al. showed good results of RLKPE for patients with recurrent jaundice after initial OKPE. The authors found that 10/12 patients had normal bilirubin concentrations after RLKPE [13]. In our study, 20/25 (80%) patients with sudden cessation of bile drainage achieved normal bilirubin concentrations after RLKPE. In addition, the 3-year and 5-year SNL rates of patients who underwent RLKPE were comparable to those of patients after unrevised ILKPE. These satisfactory results were attributed to a rigorous selection of candidates with poor jaundice reduction after the initial Kasai operation. If the indications for revision were relaxed, the postoperative results would be quite different. Nonetheless, we cannot exclude the possibility that the absence of statistical significance between RLKPE and ILKPE regarding SNL rates is due to the small sample of RLKPE. In a survey from the Japanese Biliary Atresia Registry, the revision rate was 21% and the CJ rate was 35% among 2630 BA patients after the failed initial OKPE during 1989 - 2011 [10]. The indications for RLKPE in our center were bile drainage that stopped abruptly after ILKPE and no improvement after 2 weeks of antibiotic treatment. However, a consensus has never been reached for the optimal timing of revision worldwide. Some reports have shown that revision could be effective for patients in whom jaundice suddenly recurred after a favorable initial reduction, irrespective of the time since the initial surgery [14, 15]. However, Shirota suggested that the revision should be performed as soon as irreversible jaundice is recognized [12]. In our study, the time interval from the cessation of bile drainage to revision was 21.9±8.0 days, which should be shortened to avoid further liver damage. RLKPE is not recommended for patients with bile drainage failure for more than 60 days or patients with severe ascites or aggravated liver function. For patients over 1 year old with sudden bile drainage failure, antibiotics should be administered first, and liver transplantation is recommended if conservative treatment is ineffective. Technically, RLKPE is relatively easy to perform, partly due to the omission of the Roux limb anastomosis, which is a time-consuming step in ILKPE. Under the magnified and clear view of the laparoscope, the Roux limb is easily identified by only removing the greater omentum adhered to the porta hepatis. After partly dismantling the tail of the Roux limb, the fibrous occlusive hilar plate could be spotted. At this point, the main portal vein and its branches should be clearly and accurately marked, and the resection of the fibrous plate should be confined to the area between the portal branches. The resection level of the fibrous plate depends on the bile drainage status, which is consistent with that in ILKPE. The last step of portoenterostomy in RLKPE is the same as that in ILKPE [15-18]. However, some authors were reluctant to repeat the Kasai operation because of the possibility of a high incidence of perioperative complications (e.g., uncontrolled intraoperative bleeding) and longer ORT [13]. Perineal adhesion was more common in patients who underwent RLKPE. Specifically, in the vicinity of the porta hepatis, dense fibrous granulation tissue sometimes may result in unanticipated bleeding and damage to the Roux limb [11]. In some cases, oozing bleeding from the remains of the hilar fibrous plate is difficult to stop under laparoscopy. In addition, accidental portal hemorrhage from iatrogenic injuries may place patients at great risk. In our study, however, fewer peritoneal adhesions around the porta hepatis were observed during RLKPE, at least partially due to the laparoscopic technique used for ILKPE. Under laparoscopy, the image could be zoomed in, and the portal vein could be easily recognized, which may help to protect it from accidental injuries when dissecting the hilar fibrous plate [19]. Active bleeding at the fibrous stump can be stopped by compression with a gauze pad for a period. In the event of uncontrolled hemorrhage, conversion to OKPE should be adopted without taking risks to ensure the safety of the patients. The above measures adopted in our hospital may make RLKPE safer. In our study, the blood loss volume, conversion rate, ORT and incidence of APOC of RLKPE were not significantly different from those of ILKPE. Perhaps the technique of RLKPE may repeatedly be used for revision in patients with recurrent jaundice postoperatively. Two limitations of our study deserve comment. First, the evaluation of RLKPE was based on retrospective data. Second, there were only a few cases of RLKPE compared with the number of unrevised ILKPE cases. Nonetheless, we recommend RLKPE as the preferred surgical treatment for patients with recurrent jaundice after the initial Kasai operation. If the time interval between the cessation of bile drainage and revision is shortened, the postoperative outcomes could be improved. Conclusions In conclusion, RLKPE is a feasible, safe and effective procedure for the treatment of patients with recurrent jaundice after ILKPE. However, studies with a larger number of patients and a longer follow-up time are needed to confirm the advantages of this technique. Abbreviations BA: biliary atresia; RLKPE: revision of laparoscopic Kasai portoenterostomy; ILKPE: initially successful laparoscopic Kasai portoenterostomy; SNL: survival with native liver; LT: liver transplantation; OKPE: open Kasai portoenterostomy; CJ: clearance of jaundice; ORT: operative time; APOC: any perioperative complications Declarations Ethical approval and consent to participate The study was approved by the Ethics Committee of the West China Hospital of Sichuan University. Written informed consent was obtained from the patients’ parents, according to the provisions of the Declaration of Helsinki. Consent to publish Written informed consent for publication this study was obtained from the patients’ parents. Copies of the signed informed consent forms are available for review by the Series Editor. Availability of data and materials The datasets used and/or analyzed during the current study available from the corresponding author on reasonable request. Competing interests The authors declare that they have no competing interests, either financial or non-financial, that could be perceived as prejudicing the impartiality of the research reported. Funding This work was supported by the National Natural Science Foundation of China (grant numbers 81400862 and 81401606), the Key Project in the Science & Technology Program of Sichuan Province (grant number 2019YFS0322), the Science Foundation for The Excellent Youth Scholars of Sichuan University (grant number 2015SU04A15), and the 1·3·5 Project for Disciplines of Excellence-Clinical Research Incubation Project, West China Hospital of Sichuan University (grant numbers 2019HXFH056 and 2020HXFH048). Authors’ contributions YJ, XPZ, SYC, YNL, KYY, JYZ, and ZCX were involved in the clinical management of these patients and collected clinical details of this study. ZCX reviewed the literature, and drafted the manuscript. YJ reviewed the manuscript. All authors read and approved the final manuscript. Acknowledgement We thank the patients and their parents for their co-operation and support, and for providing consent for publication. We also thank the study and hospital nurses for their assistance with the management of the patients. References Hartley JL, Davenport M, Kelly DA (2009) Biliary atresia. Lancet 374 : 1704-1713 Lucianetti A, Guizzetti M, Bertani A, Corno V, Maldini G, Pinelli D, Aluffi A, Codazzi D, Spotti A, Spada M, Gridelli B, Torre G, Colledan M (2005) Liver transplantation in children weighting less than 6 kg: the Bergamo experience. Transplant Proc 37 : 1143-1145 Hsu EK, Shaffer ML, Gao L, Sonnenday C, Volk ML, Bucuvalas J, Lai JC (2017) Analysis of Liver Offers to Pediatric Candidates on the Transplant Wait List. Gastroenterology 153 : 988-995 Esteves E, Clemente Neto E, Ottaiano Neto M, Devanir J, Jr., Esteves Pereira R (2002) Laparoscopic Kasai portoenterostomy for biliary atresia. Pediatr Surg Int 18 : 737-740 Ji Y, Yang K, Zhang X, Jin S, Jiang X, Chen S, Xu Z (2020) The short-term outcome of modified laparoscopic Kasai portoenterostomy for biliary atresia. Surg Endosc Ji Y, Yang K, Zhang X, Chen S, Xu Z (2018) Learning curve of laparoscopic Kasai portoenterostomy for biliary atresia: report of 100 cases. BMC Surg 18 : 107 Li Y, Xiang B, Wu Y, Wang C, Wang Q, Zhao Y, Chen S, Ji Y, Xu Z (2018) Medium-term Outcome of Laparoscopic Kasai Portoenterostomy for Biliary Atresia With 49 Cases. J Pediatr Gastroenterol Nutr 66 : 857-860 Wada M, Nakamura H, Koga H, Miyano G, Lane GJ, Okazaki T, Urao M, Murakami H, Kasahara M, Sakamoto S, Ishizaki Y, Kawasaki S, Yamataka A (2014) Experience of treating biliary atresia with three types of portoenterostomy at a single institution: extended, modified Kasai, and laparoscopic modified Kasai. Pediatr Surg Int 30 : 863-870 Yamataka A, Lane GJ, Koga H, Cazares J, Nakamura H (2014) Role of laparoscopy during surgery at the porta hepatis. S Afr Med J 104 : 820-824 Nio M, Sasaki H, Tanaka H, Okamura A (2013) Redo surgery for biliary atresia. Pediatr Surg Int 29 : 989-993 Mendoza MM, Chiang JH, Lee SY, Kao CY, Chuang JH, Tiao MM, Hsieh CS (2012) Reappraise the effect of redo-Kasai for recurrent jaundice following Kasai operation for biliary atresia in the era of liver transplantation. Pediatr Surg Int 28 : 861-864 Shirota C, Uchida H, Ono Y, Murase N, Tainaka T, Yokota K, Oshima K, Shirotsuki R, Hinoki A, Ando H (2016) Long-term outcomes after revision of Kasai portoenterostomy for biliary atresia. J Hepatobiliary Pancreat Sci 23 : 715-720 Murase N, Uchida H, Ono Y, Tainaka T, Yokota K, Tanano A, Shirota C, Shirotsuki R (2015) A New Era of Laparoscopic Revision of Kasai Portoenterostomy for the Treatment of Biliary Atresia. Biomed Res Int 2015 : 173014 Shneider BL, Brown MB, Haber B, Whitington PF, Schwarz K, Squires R, Bezerra J, Shepherd R, Rosenthal P, Hoofnagle JH, Sokol RJ (2006) A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr 148 : 467-474 Haber BA, Erlichman J, Thayu M, Flake AW, Rand EB (2006) Successful revision of portoenterostomy in an infant with biliary atresia. J Pediatr Surg 41 : e1-3 Bondoc AJ, Taylor JA, Alonso MH, Nathan JD, Wang Y, Balistreri WF, Bezerra JA, Ryckman FC, Tiao GM (2012) The beneficial impact of revision of Kasai portoenterostomy for biliary atresia: an institutional study. Ann Surg 255 : 570-576 Urahashi T, Ihara Y, Sanada Y, Wakiya T, Yamada N, Okada N, Mizuta K (2013) Effect of repeat Kasai hepatic portoenterostomy on pediatric live-donor liver graft for biliary atresia. Exp Clin Transplant 11 : 259-263 Sugawara Y, Makuuchi M, Kaneko J, Ohkubo T, Mizuta K, Kawarasaki H (2004) Impact of previous multiple portoenterostomies on living donor liver transplantation for biliary atresia. Hepatogastroenterology 51 : 192-194 Li Y, Gan J, Wang C, Xu Z, Zhao Y, Ji Y (2019) Comparison of laparoscopic portoenterostomy and open portoenterostomy for the treatment of biliary atresia. Surg Endosc 33 : 3143-3152 Table Table 1. Demographic and clinical characteristics of all patients who underwent ILKPE Characteristics Group A N=25 Group B N=203 Group C N=84 Total N=312 P Sex, n (%) Male 9 (36%) 65 (32%) 26 (31.0%) 100 (32.1%) 0.69 Age at LKPE (days) 76.7±15.9 81.8±18.7 87.1±21.5 82.8±19.4 0.53 Classification of BA, n (%) Ⅰ Ⅱ Ⅲ Cystic 1 (4%) 1 (4%) 22 (88%) 1 (4%) 3 (1.5%) 12 (5.9%) 156 (76.8%) 32 (15.8%) 1 (1.2%) 2 (2.4%) 79 (94.0%) 2 (2.4%) 5 (1.6%) 15 (4.8%) 257 (82.4%) 35 (11.2%) 0.37 0.70 0.20 0.12 Follow-up duration (months) 63.9±26.0 67.9±25.6 66.4±25.2 67.2±25.5 0.81 Values are presented as n (%) or median. P : comparison between group A and group B. LKPE: laparoscopic Kasai portoenterostomy. ILKPE: initially successful laparoscopic Kasai portoenterostomy. CJ: clearance of jaundice. Cite Share Download PDF Status: Published Journal Publication published 30 Apr, 2021 Read the published version in Orphanet Journal of Rare Diseases → Version 2 posted Review # 2 received at journal 05 Mar, 2021 Editorial decision: Minor revision 05 Mar, 2021 Reviewer # 2 agreed at journal 20 Feb, 2021 Review # 1 received at journal 16 Dec, 2020 Reviewer # 1 agreed at journal 13 Dec, 2020 Reviewers invited by journal 10 Dec, 2020 Editor assigned by journal 08 Dec, 2020 Submission checks completed at journal 08 Dec, 2020 Editor invited by journal 08 Dec, 2020 You are reading this latest preprint version Show more versions Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-70828","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research","associatedPublications":[],"authors":[{"id":6343402,"identity":"9398cc20-bbdc-47a5-8888-3d37b94225d6","order_by":0,"name":"Yi Ji","email":"","orcid":"https://orcid.org/0000-0002-9289-9660","institution":"Sichuan University West China Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Yi","middleName":"","lastName":"Ji","suffix":""},{"id":6343403,"identity":"a96bd106-abfb-4ffb-bc3c-c2c533dae5e2","order_by":1,"name":"Xuepeng Zhang","email":"","orcid":"","institution":"Sichuan University West China Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Xuepeng","middleName":"","lastName":"Zhang","suffix":""},{"id":6343404,"identity":"44145dd1-e537-458a-bd1c-9fc369d697b6","order_by":2,"name":"Siyuan Chen","email":"","orcid":"","institution":"Sichuan University West China Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Siyuan","middleName":"","lastName":"Chen","suffix":""},{"id":6343405,"identity":"85fa7a90-abee-4790-a629-31ba01cc164b","order_by":3,"name":"Yanan Li","email":"","orcid":"","institution":"Sichuan University West China Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Yanan","middleName":"","lastName":"Li","suffix":""},{"id":6343406,"identity":"6f286be5-efe9-4189-bc4f-58d19500c4aa","order_by":4,"name":"Kaiying Yang","email":"","orcid":"","institution":"Sichuan University West China Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Kaiying","middleName":"","lastName":"Yang","suffix":""},{"id":6343407,"identity":"a1d45568-916b-4602-9772-6d870b5ccb49","order_by":5,"name":"Jiangyuan Zhou","email":"","orcid":"","institution":"Sichuan University West China Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Jiangyuan","middleName":"","lastName":"Zhou","suffix":""},{"id":6343408,"identity":"5f03cda3-a036-4c35-b26e-6bc5af00e758","order_by":6,"name":"Zhicheng Xu","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAArElEQVRIiWNgGAWjYPCCBDk29vYDpGkx5uM5k0CalsR5Eg4GxKk1OH/4mcTHtrT0NgmGBIYfFdsIa5FsOGZsOLMtJ7dNuvEAY8+Z24S18DP2MD7m3VaR2yZzIIGZsY0ILWzMPAyH/26rSGeTSDAgTgs/Gw/jY8ZtOQnEa5HsYTM27P2XZtgGDOSDRPkFHGI/ziTLy7e3H3zwo4IILSjgAInqR8EoGAWjYBTgAgD2YTi1HHZW/gAAAABJRU5ErkJggg==","orcid":"","institution":"West China Hospital of Sichuan University","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Zhicheng","middleName":"","lastName":"Xu","suffix":""}],"badges":[],"createdAt":"2020-09-02 11:34:13","currentVersionCode":2,"declarations":"","doi":"10.21203/rs.3.rs-70828/v2","doiUrl":"https://doi.org/10.21203/rs.3.rs-70828/v2","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s13023-021-01835-z","type":"published","date":"2021-04-30T21:03:12+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":4573020,"identity":"8a025d79-50f9-4c8c-9ba7-40189aec9f94","added_by":"auto","created_at":"2020-12-29 14:00:59","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":122157,"visible":true,"origin":"","legend":"Kaplan-Meier analysis showed that the survival of patients with native livers was not significantly different between groups A and B (P\u003e0.01).","description":"","filename":"Fig1.png","url":"https://assets-eu.researchsquare.com/files/rs-70828/v2/d02e1969f988b7e691a73b1c.png"},{"id":13641518,"identity":"efe6a004-1af5-46af-bb46-457b680d7428","added_by":"auto","created_at":"2021-09-17 09:03:56","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":457666,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-70828/v2/18336265-145d-4db4-8c07-a5c75e275b31.pdf"}],"financialInterests":"","formattedTitle":"Medium-term outcomes after laparoscopic revision of laparoscopic Kasai portoenterostomy in patients with biliary atresia","fulltext":[{"header":"Introduction","content":"\u003cp\u003eBiliary atresia (BA) is a progressive fibrous occlusive disease that, if not treated promptly and effectively, may lead to cirrhosis or end-stage liver disease [1]. Although liver transplantation (LT) significantly improves the prognosis of patients with BA, Kasai portoenterostomy is still regarded as the standard initial option due to the technical complexity associated with liver transplantation in young children and the shortage of qualified donors [2, 3]. In 2002, Esteves [4] developed the laparoscopic Kasai portoenterostomy (LKPE) procedure, which created a new era of minimally invasive surgery for BA. Although a learning curve of LKPE is present, some satisfactory results have been achieved, in which the 3-year and 5-year survival with native liver (SNL) rates after LKPE were not inferior, or even superior, to those after open Kasai portoenterostomy (OKPE) [5-10]. Unfortunately, LKPE is not effective in every patient with BA, as is the case for OKPE. In patients who experienced sudden cessation of bile drainage after initially successful LKPE (ILKPE), the necessity and indications for revision LKPE (RLKPE) have not been previously reported. Herein, we review and analyze our experiences with RLKPE in patients with BA.\u003c/p\u003e\n"},{"header":"1. Materials And Methods","content":"\u003cp\u003e\u003cstrong\u003e2.1 Design and\u0026nbsp;study population\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was approved by the Ethical Committee of the West China Hospital of Sichuan University. The patients\u0026rsquo; parents or guardians\u0026nbsp;gave written informed consent.\u0026nbsp;The diagnosis of BA was based on a combination of ultrasonic findings,\u0026nbsp;intraoperative\u0026nbsp;cholangiography findings, and histological examinations of the hepatic parenchyma and extrahepatic bile ducts. Patients with BA splenic malformation, severe cardiac anomalies, or other significant\u0026nbsp;comorbidities\u0026nbsp;that would influence the postoperative course were excluded from the study. The 312 patients with\u0026nbsp;nonsyndromic\u0026nbsp;BA\u0026nbsp;who\u0026nbsp;underwent LKPE\u0026nbsp;from\u0026nbsp;May 2009 to May 2017 were enrolled in this study. They were divided into 3 cohorts based on their outcome after ILKPE: (1) the 25 patients in group A underwent RLKPE; (2) the 203 patients in group B underwent ILKPE and required no further surgical intervention; and (3) the 84 patients in group C experienced failed ILKPE and either died or required liver transplantation for survival. The indications for revision were the sudden cessation of bile drainage after ILKPE and no improvement\u0026nbsp;after 2 weeks of antibiotic treatment. The 3-year and 5-year SNL rates were compared between groups A and B and between groups A and C. Among the patients in group A, the perioperative data, rates of clearance of jaundice (CJ) and cholangitis of RLKPE were compared with\u0026nbsp;those\u0026nbsp;of ILKPE.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.2 Operative technique\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.2.1 The LKPE procedure\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe\u0026nbsp;LKPE\u0026nbsp;procedure was similar to that described in\u0026nbsp;a previously\u0026nbsp;published paper\u0026nbsp;[5-7]. Succinctly, a percutaneous suture was used to snare the round and falciform ligament and to retract the liver. The atretic gallbladder and cystic duct are dissected free from the gallbladder fossa. The portal plate over the bifurcation of the main portal vein could be found along the common hepatic duct. Once the base of the fibrous cone was reached, the fibrous cord was transected with laparoscopic scissors. The resection level of the fibrous cone was\u0026nbsp;dependent\u0026nbsp;on the presence of\u0026nbsp;abundant bile-like juice over the fibrous stump. Bleeding from the fibrous remains of\u0026nbsp;the\u0026nbsp;portal plate was controlled by direct compression with gauze pads. The ligament of Treitz was identified,\u0026nbsp;and the jejunum 15 cm distal to\u0026nbsp;the\u0026nbsp;ligament\u0026nbsp;was\u0026nbsp;exteriorized through the umbilical port site. A 25-40 cm Roux-en-Y limb was fashioned. The Roux\u0026nbsp;loop was delivered to the hilum through a\u0026nbsp;retrocolic\u0026nbsp;path. One layer of end-to-side portoenterostomy (diameter of the anastomosis 1.0-1.5\u0026nbsp;cm) was performed with interrupted 5-0 absorbable sutures. A drain was inserted into the foramen of Winslow.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.2.2 The RLKPE procedure\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe trocar placement and pneumoperitoneum pressure settings were\u0026nbsp;the\u0026nbsp;same as those in LKPE, as mentioned above. The other steps of RLPKE were as\u0026nbsp;follows:\u0026nbsp;(1) removing the greater omentum adhered to the porta hepatis; (2) partly dismantling the anastomosed Roux limb adjacent to the porta hepatis; (3) resecting the granulation tissue; (4) dissecting the hilar fibrous stump layer by layer until abundant bile-like juice was observed;\u0026nbsp;and\u0026nbsp;(5) carrying out end-to-end portoenterostomy after trimming the tail of the Roux limb.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.3 The management algorithm after\u0026nbsp;surgery\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003ePostoperatively, the\u0026nbsp;BA\u0026nbsp;patients in the 3 groups received the same medical treatment\u0026nbsp;protocol. Antibiotic\u0026nbsp;therapy was continued intravenously for 25-30 days in the hospital. Methylprednisolone was administered intravenously 5 days postoperatively at a dose of 5 mg/kg/day initially and was reduced by 1 mg/kg/day every 3 days for 2 weeks or longer until a normal total bilirubin value was reached. Sulfamethoxazole and cephalosporin antibiotics were orally administered alternately weekly until 1 year of age.\u0026nbsp;Ursodeoxycholic\u0026nbsp;acid and hepatoprotective tablets were used until 3 years of age.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.4 Definitions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe operative time (ORT) was calculated as the length of time between the skin incision and closure.\u0026nbsp;Any\u0026nbsp;perioperative complications (APOC) was defined as a\u0026nbsp;complication that occurred\u0026nbsp;during the perioperative period, including wound infection, umbilical hernia, intestinal anastomotic fistula, and adhesive intestinal obstruction. CJ was defined as a total bilirubin level \u0026lt;1.2 mg/dL within 6 months postoperatively. Cholangitis was defined as an elevated serum bilirubin (\u0026gt;2.5 mg/dL), leukocytosis with left shift, and normal to acholic stools in a febrile patient (\u0026gt;38.0 \u0026deg;C). Postoperative outcomes were followed through outpatient clinic evaluations and were excluded if the patients underwent liver transplantation or died.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.5 Statistics\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe software applied for statistical calculations was SPSS 22.0 for Windows 10.0 (SPSS Inc., Chicago, IL, USA). The demographic data of BA patients were compared using one-way analysis of variance and\u0026nbsp;the\u0026nbsp;chi-squared test. The difference\u0026nbsp;in\u0026nbsp;SNL rate among the patients was analyzed by\u0026nbsp;the\u0026nbsp;Kaplan\u0026ndash;Meier method with endpoints of death or liver transplantation and compared using the log-rank test. A \u003cem\u003eP\u003c/em\u003e value \u0026lt;0.05 was considered statistically significant.\u003c/p\u003e"},{"header":"2. Results","content":"\u003cp\u003e\u003cstrong\u003e2.1 Demographic and clinical characteristics of all patients in\u003c/strong\u003e\u003cstrong\u003e the\u003c/strong\u003e\u003cstrong\u003e three groups\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eFrom May 2009 to May 2019, 312 patients with nonsyndromic BA underwent ILKPE. The demographic and clinical characteristics of all patients in the three groups are presented in Table 1. The median age at operation was 82.8\u0026plusmn;19.4 days, with a male proportion of 32.1%. The proportions of types I, II, and III and cystic BA were 1.6%, 4.8%, 82.4% and 11.2%, respectively. The median follow-up time was 67.2\u0026plusmn;25.5 months. The rates of CJ and cholangitis after ILKPE were 73.1% and 58.9%, respectively. The 3-year and 5-year SNL rates were 62.6% and 54.1%, respectively.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.2 Comparison between group A and group B\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eIn group A, 25 patients underwent RLKPE. The male proportion, age and follow-up time were not significantly different between groups A and B. The 3-year and 5-year SNL rates were 64.0% and 52.0% in group A and were not significantly different from the 86.2% and 73.9% in group B (\u003cem\u003eP \u003c/em\u003e\u0026gt;0.05) (Fig 1).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.3 Comparison of \u003c/strong\u003e\u003cstrong\u003eperioperative\u003c/strong\u003e\u003cstrong\u003e variables and rates of CJ and cholangitis between ILKPE and RLKPE in 25 patients\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe demographic and clinical characteristics of 25 patients who underwent both ILKPE and RLKPE are presented in Table 2. The ages at ILKPE and RLKPR were 76.7\u0026plusmn;15.9 days and 159.8\u0026plusmn;50.4 days, respectively. The time interval from suspension of bile drainage to revision was 21.9\u0026plusmn;8.0 days. The ORT, blood loss, conversion rate and incidence of AOPC during ILKPE were not significantly different from those during RLKPE. The rates of CJ and cholangitis after ILKPE were 100% and 64.0% and were significantly different from the 80.0% and 28.0% after RLKPE (\u003cem\u003eP\u003c/em\u003e\u0026lt;0.01).\u003c/p\u003e"},{"header":"3. Discussion","content":"\u003cp\u003eExperiences with revision for BA patients with recurrent jaundice after initial OKPE or ILKPE have\u0026nbsp;rarely\u0026nbsp;been reported. In two studies, CJ rates of 60%\u0026nbsp;[11]\u0026nbsp;and 83.3%\u0026nbsp;[12]\u0026nbsp;were obtained after revision of OKPE for patients with initially successful OKPE. Naruhiko et al. showed good results of RLKPE for patients with recurrent jaundice after initial OKPE. The authors found that 10/12 patients had normal bilirubin concentrations after RLKPE\u0026nbsp;[13]. In our study, 20/25 (80%) patients with sudden cessation of bile drainage achieved normal bilirubin concentrations after RLKPE. In addition, the 3-year\u0026nbsp;and\u0026nbsp;5-year SNL rates of patients who underwent RLKPE were comparable to those of patients after unrevised ILKPE. These satisfactory results were attributed to a rigorous selection of candidates with poor jaundice reduction after the initial Kasai operation. If the indications for revision were relaxed, the postoperative results would be quite different. Nonetheless, we cannot exclude the possibility that the absence of statistical significance between RLKPE and ILKPE regarding SNL rates is due to the small sample of RLKPE. In a survey from the Japanese Biliary Atresia Registry, the revision rate was 21% and the CJ rate was 35% among 2630 BA patients after the failed initial OKPE during 1989 - 2011\u0026nbsp;[10].\u003c/p\u003e\n\u003cp\u003eThe indications for RLKPE in our center were bile drainage that stopped abruptly after ILKPE and no improvement after 2 weeks of antibiotic treatment. However,\u0026nbsp;a\u0026nbsp;consensus has never been reached for the optimal timing of revision worldwide. Some reports have shown that revision could be effective for patients in whom jaundice suddenly recurred after a favorable initial reduction, irrespective of the time since the initial surgery\u0026nbsp;[14, 15].\u0026nbsp;However,\u0026nbsp;Shirota suggested that the revision should be performed as soon as irreversible jaundice\u0026nbsp;is\u0026nbsp;recognized\u0026nbsp;[12]. In our study, the time interval from the cessation of bile drainage to revision was 21.9\u0026plusmn;8.0 days, which should be shortened to avoid further liver damage. RLKPE is not recommended for patients with bile drainage failure for more than 60 days or patients with severe ascites or aggravated liver function. For patients over 1 year old with sudden bile drainage failure, antibiotics should be administered first, and liver transplantation is recommended if conservative treatment is ineffective.\u003c/p\u003e\n\u003cp\u003eTechnically, RLKPE is relatively easy to perform, partly due to the omission of the Roux limb anastomosis, which is a time-consuming step in ILKPE. Under the magnified and clear view of the laparoscope, the Roux limb is easily identified by\u0026nbsp;only\u0026nbsp;removing the greater omentum adhered to the porta hepatis. After partly dismantling the tail of\u0026nbsp;the\u0026nbsp;Roux limb, the fibrous occlusive hilar plate could be spotted. At this point,\u0026nbsp;the main portal vein and its branches should be clearly and accurately marked, and the resection of\u0026nbsp;the\u0026nbsp;fibrous plate should be confined to the area between the portal branches. The resection level of\u0026nbsp;the\u0026nbsp;fibrous plate depends on the bile drainage status,\u0026nbsp;which is consistent with that in ILKPE. The last step of portoenterostomy in RLKPE is the same as that in ILKPE\u0026nbsp;[15-18].\u003c/p\u003e\n\u003cp\u003eHowever, some authors were reluctant to repeat the Kasai operation because\u0026nbsp;of\u0026nbsp;the possibility of\u0026nbsp;a\u0026nbsp;high incidence of perioperative complications (e.g., uncontrolled intraoperative bleeding) and longer ORT\u0026nbsp;[13]. Perineal adhesion was more common in patients who underwent RLKPE.\u0026nbsp;Specifically, in the vicinity of the porta hepatis,\u0026nbsp;dense fibrous granulation tissue sometimes may result in unanticipated bleeding and damage\u0026nbsp;to the\u0026nbsp;Roux limb\u0026nbsp;[11]. In some cases, oozing bleeding from the remains of\u0026nbsp;the\u0026nbsp;hilar fibrous plate is difficult to stop under laparoscopy. In addition, accidental portal hemorrhage from iatrogenic injuries may place patients at great risk. In our study, however, fewer peritoneal adhesions around the porta hepatis were observed during RLKPE, at least partially due to the laparoscopic technique used for ILKPE. Under laparoscopy, the image could be zoomed in, and the portal vein could be easily recognized, which may help to protect it from accidental injuries when dissecting the hilar fibrous plate\u0026nbsp;[19]. Active bleeding at the fibrous stump can be stopped by compression with a gauze pad for a period. In the event of uncontrolled hemorrhage, conversion to OKPE should be adopted without taking risks to ensure the safety of the patients. The above measures adopted in our hospital may make RLKPE safer. In our study, the blood loss volume, conversion rate, ORT and incidence of APOC of RLKPE were not significantly different\u0026nbsp;from\u0026nbsp;those of ILKPE. Perhaps the technique of RLKPE may repeatedly be used for revision in patients with recurrent jaundice postoperatively.\u003c/p\u003e\n\u003cp\u003eTwo limitations of our study deserve comment. First, the evaluation of RLKPE was based on retrospective data. Second, there were only a few cases of RLKPE compared with the number of unrevised ILKPE cases. Nonetheless, we recommend RLKPE as the preferred surgical treatment for patients with recurrent jaundice after the initial Kasai operation. If the time interval between the cessation of bile drainage and revision is shortened, the postoperative outcomes could be improved.\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003eIn conclusion, RLKPE is a feasible, safe and effective procedure for the treatment of patients with recurrent jaundice after ILKPE. However, studies with a larger number of patients and a longer follow-up time are needed to confirm the advantages of this technique.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eBA: biliary atresia; RLKPE: revision of laparoscopic Kasai portoenterostomy; ILKPE: initially successful laparoscopic Kasai portoenterostomy; SNL: survival with native liver; LT: liver transplantation; OKPE: open Kasai portoenterostomy; CJ: clearance of jaundice; ORT: operative time; APOC: any perioperative complications\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthical approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe study was approved by the Ethics Committee of the West China Hospital of Sichuan University. Written informed consent was obtained from the patients\u0026rsquo; parents, according to the provisions of the Declaration of Helsinki.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to publish\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent for publication this study was obtained from the patients\u0026rsquo; parents. Copies of the signed informed consent forms are available for review by the Series Editor.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets used and/or analyzed during the current study available from the corresponding author on reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests, either financial or non-financial, that could be perceived as prejudicing the impartiality of the research reported.\u003c/p\u003e\n\u003cp\u003e\u003cstrong style=\"text-align: inherit;\"\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis work was supported by the National Natural Science Foundation of China (grant numbers 81400862 and 81401606), the Key Project in the Science \u0026amp; Technology Program of Sichuan Province (grant number 2019YFS0322), the Science Foundation for The Excellent Youth Scholars of Sichuan University (grant number 2015SU04A15), and the 1\u0026middot;3\u0026middot;5 Project for Disciplines of Excellence-Clinical Research Incubation Project, West China Hospital of Sichuan University (grant numbers 2019HXFH056 and 2020HXFH048).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eYJ, XPZ, SYC, YNL, KYY, JYZ, and ZCX were involved in the clinical management of these patients and collected clinical details of this study. ZCX reviewed the literature, and drafted the manuscript. YJ reviewed the manuscript. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgement\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWe thank the patients and their parents for their co-operation and support, and for providing consent for publication. We also thank the study and hospital nurses for their assistance with the management of the patients.\u003c/p\u003e\n"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eHartley JL, Davenport M, Kelly DA (2009) Biliary atresia. Lancet 374\u003cstrong\u003e:\u003c/strong\u003e1704-1713\u003c/li\u003e\n\u003cli\u003eLucianetti A, Guizzetti M, Bertani A, Corno V, Maldini G, Pinelli D, Aluffi A, Codazzi D, Spotti A, Spada M, Gridelli B, Torre G, Colledan M (2005) Liver transplantation in children weighting less than 6 kg: the Bergamo experience. Transplant Proc 37\u003cstrong\u003e:\u003c/strong\u003e1143-1145\u003c/li\u003e\n\u003cli\u003eHsu EK, Shaffer ML, Gao L, Sonnenday C, Volk ML, Bucuvalas J, Lai JC (2017) Analysis of Liver Offers to Pediatric Candidates on the Transplant Wait List. Gastroenterology 153\u003cstrong\u003e:\u003c/strong\u003e988-995\u003c/li\u003e\n\u003cli\u003eEsteves E, Clemente Neto E, Ottaiano Neto M, Devanir J, Jr., Esteves Pereira R (2002) Laparoscopic Kasai portoenterostomy for biliary atresia. Pediatr Surg Int 18\u003cstrong\u003e:\u003c/strong\u003e737-740\u003c/li\u003e\n\u003cli\u003eJi Y, Yang K, Zhang X, Jin S, Jiang X, Chen S, Xu Z (2020) The short-term outcome of modified laparoscopic Kasai portoenterostomy for biliary atresia. Surg Endosc\u003c/li\u003e\n\u003cli\u003eJi Y, Yang K, Zhang X, Chen S, Xu Z (2018) Learning curve of laparoscopic Kasai portoenterostomy for biliary atresia: report of 100 cases. BMC Surg 18\u003cstrong\u003e:\u003c/strong\u003e107\u003c/li\u003e\n\u003cli\u003eLi Y, Xiang B, Wu Y, Wang C, Wang Q, Zhao Y, Chen S, Ji Y, Xu Z (2018) Medium-term Outcome of Laparoscopic Kasai Portoenterostomy for Biliary Atresia With 49 Cases. J Pediatr Gastroenterol Nutr 66\u003cstrong\u003e:\u003c/strong\u003e857-860\u003c/li\u003e\n\u003cli\u003eWada M, Nakamura H, Koga H, Miyano G, Lane GJ, Okazaki T, Urao M, Murakami H, Kasahara M, Sakamoto S, Ishizaki Y, Kawasaki S, Yamataka A (2014) Experience of treating biliary atresia with three types of portoenterostomy at a single institution: extended, modified Kasai, and laparoscopic modified Kasai. Pediatr Surg Int 30\u003cstrong\u003e:\u003c/strong\u003e863-870\u003c/li\u003e\n\u003cli\u003eYamataka A, Lane GJ, Koga H, Cazares J, Nakamura H (2014) Role of laparoscopy during surgery at the porta hepatis. S Afr Med J 104\u003cstrong\u003e:\u003c/strong\u003e820-824\u003c/li\u003e\n\u003cli\u003eNio M, Sasaki H, Tanaka H, Okamura A (2013) Redo surgery for biliary atresia. Pediatr Surg Int 29\u003cstrong\u003e:\u003c/strong\u003e989-993\u003c/li\u003e\n\u003cli\u003eMendoza MM, Chiang JH, Lee SY, Kao CY, Chuang JH, Tiao MM, Hsieh CS (2012) Reappraise the effect of redo-Kasai for recurrent jaundice following Kasai operation for biliary atresia in the era of liver transplantation. Pediatr Surg Int 28\u003cstrong\u003e:\u003c/strong\u003e861-864\u003c/li\u003e\n\u003cli\u003eShirota C, Uchida H, Ono Y, Murase N, Tainaka T, Yokota K, Oshima K, Shirotsuki R, Hinoki A, Ando H (2016) Long-term outcomes after revision of Kasai portoenterostomy for biliary atresia. J Hepatobiliary Pancreat Sci 23\u003cstrong\u003e:\u003c/strong\u003e715-720\u003c/li\u003e\n\u003cli\u003eMurase N, Uchida H, Ono Y, Tainaka T, Yokota K, Tanano A, Shirota C, Shirotsuki R (2015) A New Era of Laparoscopic Revision of Kasai Portoenterostomy for the Treatment of Biliary Atresia. Biomed Res Int 2015\u003cstrong\u003e:\u003c/strong\u003e173014\u003c/li\u003e\n\u003cli\u003eShneider BL, Brown MB, Haber B, Whitington PF, Schwarz K, Squires R, Bezerra J, Shepherd R, Rosenthal P, Hoofnagle JH, Sokol RJ (2006) A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr 148\u003cstrong\u003e:\u003c/strong\u003e467-474\u003c/li\u003e\n\u003cli\u003eHaber BA, Erlichman J, Thayu M, Flake AW, Rand EB (2006) Successful revision of portoenterostomy in an infant with biliary atresia. J Pediatr Surg 41\u003cstrong\u003e:\u003c/strong\u003ee1-3\u003c/li\u003e\n\u003cli\u003eBondoc AJ, Taylor JA, Alonso MH, Nathan JD, Wang Y, Balistreri WF, Bezerra JA, Ryckman FC, Tiao GM (2012) The beneficial impact of revision of Kasai portoenterostomy for biliary atresia: an institutional study. Ann Surg 255\u003cstrong\u003e:\u003c/strong\u003e570-576\u003c/li\u003e\n\u003cli\u003eUrahashi T, Ihara Y, Sanada Y, Wakiya T, Yamada N, Okada N, Mizuta K (2013) Effect of repeat Kasai hepatic portoenterostomy on pediatric live-donor liver graft for biliary atresia. Exp Clin Transplant 11\u003cstrong\u003e:\u003c/strong\u003e259-263\u003c/li\u003e\n\u003cli\u003eSugawara Y, Makuuchi M, Kaneko J, Ohkubo T, Mizuta K, Kawarasaki H (2004) Impact of previous multiple portoenterostomies on living donor liver transplantation for biliary atresia. Hepatogastroenterology 51\u003cstrong\u003e:\u003c/strong\u003e192-194\u003c/li\u003e\n\u003cli\u003eLi Y, Gan J, Wang C, Xu Z, Zhao Y, Ji Y (2019) Comparison of laparoscopic portoenterostomy and open portoenterostomy for the treatment of biliary atresia. Surg Endosc 33\u003cstrong\u003e:\u003c/strong\u003e3143-3152\u003c/li\u003e\n\u003c/ol\u003e\n"},{"header":"Table","content":"\u003cdiv align=\"center\" style=\"margin:0in;text-align:justify;font-size:14px;font-family:DengXian;\"\u003e\n \u003ctable style=\"width:100.0%;border-collapse:collapse;border:none;\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd colspan=\"6\" style=\"width:100.0%;border:none;border-bottom: solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:left;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eTable 1. Demographic and clinical characteristics of all patients who underwent ILKPE\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width:21.82%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eCharacteristics\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:15.2%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eGroup A\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eN=25\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:18.72%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eGroup B\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eN=203\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eGroup C\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eN=84\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eTotal\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eN=312\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:8.92%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cem\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eP\u003c/span\u003e\u003c/em\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width:21.82%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eSex, n (%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eMale\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:15.2%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e9 (36%)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:18.72%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e65 (32%)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e26 (31.0%)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e100 (32.1%)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:8.92%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e0.69\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width:21.82%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eAge at LKPE (days)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:15.2%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e76.7\u0026plusmn;15.9\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:18.72%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e81.8\u0026plusmn;18.7\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e87.1\u0026plusmn;21.5\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e82.8\u0026plusmn;19.4\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:8.92%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e0.53\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width:21.82%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eClassification of BA, n (%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eⅠ\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eⅡ\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eⅢ\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eCystic\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:15.2%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e1 (4%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e1 (4%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e22 (88%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e1 (4%)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:18.72%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e3 (1.5%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e12 (5.9%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e156 (76.8%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e32 (15.8%)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e1 (1.2%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e2 (2.4%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e79 (94.0%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e2 (2.4%)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e5 (1.6%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e15 (4.8%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e257 (82.4%)\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e35 (11.2%)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:8.92%;border:none;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e0.37\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e0.70\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e0.20\u003c/span\u003e\u003c/p\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e0.12\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width:21.82%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eFollow-up duration (months)\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:15.2%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e63.9\u0026plusmn;26.0\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:18.72%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e67.9\u0026plusmn;25.6\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e66.4\u0026plusmn;25.2\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:17.66%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e67.2\u0026plusmn;25.5\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width:8.92%;border:none;border-bottom:solid windowtext 1.0pt;padding:0in 5.4pt 0in 5.4pt;\"\u003e\n \u003cp style=\"margin:0in;text-align:center;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e0.81\u003c/span\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n \u003c/table\u003e\n\u003c/div\u003e\n\u003cp style=\"margin:0in;text-align:justify;font-size:14px;font-family:DengXian;\"\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;color:black;'\u003eValues\u0026nbsp;\u003c/span\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003eare\u003c/span\u003e\u003cspan style='font-size:16px;font-family:\"Times New Roman\",serif;'\u003e\u0026nbsp;presented as n (%) or median. \u003cem\u003eP\u003c/em\u003e: comparison between group A and group B. LKPE: laparoscopic Kasai portoenterostomy. ILKPE: initially successful laparoscopic Kasai portoenterostomy. CJ: clearance of jaundice.\u003c/span\u003e\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":true,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"orphanet-journal-of-rare-diseases","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"ojrd","sideBox":"Learn more about [Orphanet Journal of Rare Diseases](http://ojrd.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/ojrd/default.aspx","title":"Orphanet Journal of Rare Diseases","twitterHandle":"@bmc","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Biliary atresia, Laparoscopic Kasai portoenterostomy, Revision, Outcomes","lastPublishedDoi":"10.21203/rs.3.rs-70828/v2","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-70828/v2","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eObjective:\u003c/strong\u003e To determine whether revision laparoscopic Kasai portoenterostomy (RLKPE) is a viable treatment option for patients with biliary atresia (BA) who underwent initially successful laparoscopic Kasai portoenterostomy (ILKPE).\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eMethods: \u003c/strong\u003eThe\u003cstrong\u003e \u003c/strong\u003emedical records of 312 patients with nonsyndromic BA who underwent ILKPE between May 2009 and May 2017 were retrospectively reviewed. The patients were divided into three groups according to their outcomes after ILKPE: group A: 25 patients who underwent RLKPE; group B: 203 patients who underwent ILKPE and required no further surgical intervention; and group C: 84 patients with failed ILKPE who either died or required liver transplantation for survival. The 3-year and 5-year survival with native liver (SNL) rates were compared between groups A and B C. Among the 25 patients in group A, the perioperative data of RLKPE were compared with those of ILKPE.\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eResults:\u003c/strong\u003e Of the 312 patients who underwent ILKPE, 228 reached the normal bilirubin concentration range within 6 months postoperatively. Among them, 25 patients with a sudden cessation of bile flow underwent RLKPE. Adequate biliary drainage evidenced by normalized conjugated bilirubin levels was achieved in 80% of the patients who underwent RLKPE. The perioperative variables, including operative time, blood loss, rate of conversion to open surgery and complications of RLKPE, were not significantly different between RLKPE and ILKPE. The 3-year and 5-year SNL rates in patients after RLKPE were 64.0% and 52.0%, respectively, which were not significantly different from the corresponding 86.2% and 73.9% in patients after unrevised ILKPE (\u003cem\u003eP\u003c/em\u003e\u0026gt;0.05).\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eConclusion: \u003c/strong\u003eOur data demonstrated that RPLKE can be a viable and effective treatment opinion in patients who experience sudden cessation of bile drainage after ILKPE. RPLKE can delay the need for liver transplantation, yielding encouraging medium-term patient outcomes.\u003c/p\u003e","manuscriptTitle":"Medium-term outcomes after laparoscopic revision of laparoscopic Kasai portoenterostomy in patients with biliary atresia","msid":"","msnumber":"","nonDraftVersions":[{"code":2,"date":"2020-12-29 13:58:56","doi":"10.21203/rs.3.rs-70828/v2","editorialEvents":[{"type":"communityComments","content":0},{"type":"editorInvitedReview","content":"","date":"2021-03-06T00:00:00+00:00","index":2,"fulltext":"Recommendation: Reviewer's comments unavailable due to the journal's policy.\n"},{"type":"decision","content":"Minor revision","date":"2021-03-06T00:00:00+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"","date":"2021-02-21T00:00:00+00:00","index":2,"fulltext":""},{"type":"editorInvitedReview","content":"","date":"2020-12-17T00:00:00+00:00","index":1,"fulltext":"Recommendation: Reviewer's comments unavailable due to the journal's policy.\n"},{"type":"reviewerAgreed","content":"","date":"2020-12-14T00:00:00+00:00","index":1,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2020-12-11T00:00:00+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2020-12-09T00:00:00+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2020-12-08T23:00:00+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2020-12-08T23:00:00+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"orphanet-journal-of-rare-diseases","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"ojrd","sideBox":"Learn more about [Orphanet Journal of Rare Diseases](http://ojrd.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/ojrd/default.aspx","title":"Orphanet Journal of Rare Diseases","twitterHandle":"@bmc","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true}},{"code":1,"date":"2020-09-03 16:54:18","doi":"10.21203/rs.3.rs-70828/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Minor revision","date":"2020-11-29T00:00:00+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"","date":"2020-10-18T12:00:00+00:00","index":2,"fulltext":""},{"type":"editorInvitedReview","content":"","date":"2020-09-15T12:00:00+00:00","index":1,"fulltext":"Recommendation: Reviewer's comments unavailable due to the journal's policy.\n"},{"type":"reviewerAgreed","content":"","date":"2020-09-04T12:00:00+00:00","index":1,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2020-09-03T12:00:00+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2020-09-02T12:00:00+00:00","index":"","fulltext":""},{"type":"submitted","content":"","date":"2020-09-01T12:00:00+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2020-09-01T12:00:00+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2020-09-01T12:00:00+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"orphanet-journal-of-rare-diseases","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"ojrd","sideBox":"Learn more about [Orphanet Journal of Rare Diseases](http://ojrd.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/ojrd/default.aspx","title":"Orphanet Journal of Rare Diseases","twitterHandle":"@bmc","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"7bdf5023-b0b5-410b-bed7-45dedc5bbf57","owner":[],"postedDate":"December 29th, 2020","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[{"id":429504,"name":"Internal Medicine"}],"tags":[],"updatedAt":"2021-07-27T21:03:12+00:00","versionOfRecord":{"articleIdentity":"rs-70828","link":"https://doi.org/10.1186/s13023-021-01835-z","journal":{"identity":"orphanet-journal-of-rare-diseases","isVorOnly":false,"title":"Orphanet Journal of Rare Diseases"},"publishedOn":"2021-04-30 21:03:12","publishedOnDateReadable":"April 30th, 2021"},"versionCreatedAt":"2020-12-29 13:58:56","video":"","vorDoi":"10.1186/s13023-021-01835-z","vorDoiUrl":"https://doi.org/10.1186/s13023-021-01835-z","workflowStages":[]},"version":"v2","identity":"rs-70828","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-70828","identity":"rs-70828","version":["v2"]},"buildId":"ehx78VzkSd0WSzXnipQa-","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}