Clinical Efficacy of Liver Transplantation in Caroli Syndrome: A Retrospective Analysis

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Methods: A retrospective analysis was conducted on the clinical data of patients diagnosed with Caroli syndrome and undergoing liver transplantation at Beijing Friendship Hospital, Capital Medical University, between April 2014 and May 2024. The analysis focused on baseline characteristics, surgical indicators, postoperative complications, and long-term survival rates. All patients were followed up postoperatively, with the follow-up period ending in February 2025. Results: A total of 20 patients diagnosed with Caroli syndrome underwent liver transplantation at Beijing Friendship Hospital, accounting for 1.23% (20/1623) of all liver transplant patients during the same period. Of these, 13 patients received living donor liver transplants (LDLT), and 7 received deceased donor liver transplants (DDLT). The cohort included 15 male and 5 female patients, with ages ranging from 3 to 56 years (median age 23.3). Preoperative major comorbidities included cirrhosis(18 patients), portal hypertension(17), splenomegaly(17), polycystic kidney disease(16), hypoalbuminemia(14), ascites(13), gastrointestinal bleeding(8), hepatomegaly(9), renal injury(2), pulmonary hypertension(3), hepatopulmonary syndrome(1), congenital liver fibrosis(2), and cholangitis(1). Preoperative APACHE II score ranged from 4 to 27 (mean 14.4 ±5.9), with an expected mortality rate between 5.1% and 72.5% (mean 29.4±17.3). The preoperative SOFA score ranged from 0 to 13 (mean 5.0), CLIF-SOFA score from 0 to 7 (mean 5.0), PELD score (for children under 12) from 0 to 28 (mean 15), and MELD score from 9 to 24 (mean 16.7±7.3). The graft weight ranged from 224 to 1327g (mean 492.1±331.1g), with a graft-to-recipient weight ratio (GRWR) of 0.76% to 4.90% (mean 1.7± 1.2%). The warm ischemia time ranged from 2 to 8 minutes (mean 3.4±1.7 minutes), and cold ischemia time ranged from 30 to 657 minutes (mean 233.2±209.3 minutes). None of the patients received a cross-match transplant or auxiliary liver transplantation. Three patients underwent simultaneous partial splenectomy. Postoperative complications included abdominal hemorrhage in three patients, and seizures in one patient. Comparing adult and pediatric patients, females were more frequent in the pediatric group (p=0.001), and preoperatively, chronic renal failure was more common in adults (p=0.010). There were no significant differences in other preoperative comorbidities, early postoperative complications, or late complications. Long-term postoperative complications included chronic renal failure in six patients (onset between 12.1 and 46.7 months post-surgery) and acute rejection in four patients, three of whom experienced recurrent acute rejection. Four patients (20%) died: one died from postoperative heart failure on day 8, one from unexplained sudden death at 6 months, one from pulmonary infection and respiratory failure at 1 year, and one from an unknown cause at 5.2 years. The 1-year, 3-year, and 5-year survival rates were 90%, 85%, and 78.5%, respectively. Conclusion: Liver transplantation significantly improves the survival prognosis for patients with Caroli syndrome, but attention to kidney function preservation and individualized immunosuppressive management is critical for optimal outcomes. Caroli syndrome liver transplantation clinical efficacy Long-Term Prognosis Survival Rates Living Donor Liver Transplantation Postoperative Complications Introduction Caroli syndrome is a congenital disorder inherited in an autosomal recessive pattern, characterized by multifocal segmental dilation of the intrahepatic bile ducts, often accompanied by congenital hepatic fibrosis (CHF) and varying degrees of renal cystic diseases [1]. The treatment for Caroli syndrome is primarily supportive; however, patients who experience recurrent biliary infections, particularly those with complications related to portal hypertension, may require liver transplantation. There are limited reports on the clinical efficacy and long-term prognosis of liver transplantation for Caroli syndrome. This study uses a retrospective case analysis to summarize the clinical features and outcomes of 20 patients with Caroli syndrome who underwent liver transplantation, and to conduct follow-up evaluations to assess the efficacy of liver transplantation in managing this condition. 1. Subjects A search was conducted to identify patients who underwent liver transplantation at Beijing Friendship Hospital, Capital Medical University, between April 2014 and May 2024, and were discharged with a diagnosis of Caroli syndrome. The diagnostic criteria were as follows: (1) The diagnosis of Caroli disease was based on imaging findings (such as CT, MRI, or ultrasound), which revealed multiple cystic or cylindrical low-density lesions in the liver that communicate with the intrahepatic bile ducts, displaying the “tadpole sign” or “hanging sign,” while the common bile duct remains normal. (2) The diagnosis of Caroli syndrome was made when Caroli disease was complicated by portal hypertension, as evidenced by esophageal and/or gastric varices, portal hypertensive bleeding, and/or imaging studies showing portosystemic shunting . 2. Methods (1) Data Collection: The following data were collected from the patients: demographic information, laboratory test results (including liver and renal function, complete blood count, coagulation tests, and urinalysis), preoperative comorbidities (such as cirrhosis, portal hypertension, gastrointestinal bleeding, renal injury, ascites, hepatopulmonary syndrome, pulmonary hypertension, cholangitis, hypoalbuminemia, splenomegaly, etc.), surgical details (including organ source, whether a cross-match transplant was performed, whether splenectomy was done, whether auxiliary liver transplantation was used, graft weight, graft-to-recipient weight ratio, ischemia time, etc.), liver disease-related scores (such as Child-Pugh-Turcotte score, MELD score, PELD score, SOFA score, etc.), and postoperative complications (including respiratory failure, heart failure, chronic renal failure, rejection, and vascular complications). Additionally, the patients were divided into two groups: pediatric (≤18 years) and adult (>18 years). Differences between the two groups in terms of preoperative and postoperative comorbidities, as well as follow-up parameters, were compared. All patients were followed up postoperatively, with the follow-up period concluding in February 2025. (2) Statistical Analysis: All statistical analyses were conducted using SPSS version 25.0 software. For normally distributed quantitative variables, data are presented as mean ± standard deviation (M ± SD), and group differences were assessed using the t-test. For non-normally distributed variables, data are presented as median (first quartile, third quartile) or Q2 (Q1, Q3), with group differences evaluated using the Mann-Whitney U test. Categorical variables are expressed as frequencies or percentages, with differences in proportions between groups compared using the chi-square test or Fisher’s exact test. A significance level of α = 0.05 was set, and p < 0.05 was considered statistically significant. 3. Results (1) General Information: Between April 2014 and May 2024, a total of 20 patients diagnosed with Caroli syndrome underwent liver transplantation at Beijing Friendship Hospital, Capital Medical University, representing 1.23% (20/1623) of all liver transplant cases during this period. The ages of the patients at the time of surgery ranged from 3 to 56 years, with a median age of 8 years (range: 6 to 20.5 years). Of these, 13 patients (65%) received living-donor liver transplants (LDLT), and 7 patients (35%) received deceased-donor liver transplants (DDLT). The cohort consisted of 15 male patients and 5 female patients, with 15 patients (75%) being children and 5 patients (25%) being adults. (2) Preoperative Major Comorbidities: Preoperative comorbidities included cirrhosis(18 patients), portal hypertension(17), splenomegaly(17), polycystic kidney disease(16), hypoalbuminemia(14), ascites(13), gastrointestinal bleeding(8), hepatomegaly(9), renal injury(2), pulmonary hypertension(3), hepatopulmonary syndrome(1), congenital liver fibrosis(2), and cholangitis(1). When comparing adult and pediatric patients, females were more prevalent in the pediatric group, while chronic renal failure was more commonly observed in adults prior to surgery. No significant differences were found in other preoperative comorbidities. A detailed comparison of the general characteristics and preoperative major comorbidities of pediatric and adult patients is provided in Table 1. 2.3 Intraoperative Conditions: The APACHE II score during surgery ranged from 4 to 27, with a mean of 14.4±5.9, and the predicted mortality ranged from 5.1% to 72.5%, with a mean of 29.4±17.3%. The SOFA score ranged from 0 to 13, with a median of 4 (interquartile range 2.5 to 5.0), and the CLIF-SOFA score ranged from 0 to 7, with a median of 2 (interquartile range 0.5 to 4.5). The PELD score (for children under 12) ranged from 0 to 28, with a median of 11 (interquartile range 6 to 17.5), and the MELD score ranged from 9 to 24, with a mean of 16.7±7.3. The graft weight ranged from 224 to 1327 g, with a mean of 492.1±331.1 g, and the graft-to-recipient weight ratio (GRWR) ranged from 0.76% to 4.90%, with a mean of 1.7±1.2%. The warm ischemia time ranged from 2 to 8 minutes, with a mean of 3.4±1.7 minutes, and the cold ischemia time ranged from 30 to 657 minutes, with a mean of 233.2±209.3 minutes. None of the patients received a cross-match transplant or auxiliary liver transplantation. Three patients underwent simultaneous partial splenectomy. There were no significant differences between adult and pediatric patients regarding donor source, graft type, surgical approach, and other intraoperative factors, as detailed in Table 2. 2.4 Early Postoperative Complications: Early postoperative complications included hypertension in 1 patient (pediatric group), acute kidney injury in 8 patients (3 adults, 5 children), acute rejection in 1 patient (pediatric group), respiratory failure in 1 patient (pediatric group), abdominal hemorrhage in 3 patients (2 adults, 1 child), and seizures and cerebral infarction in 1 patient each (both in the adult group). One patient died during the initial hospitalization, resulting in a 5% mortality rate. The patient passed away on postoperative day 8 due to heart failure. No significant differences were observed in early postoperative complications between adult and pediatric patients, as detailed in Table 2. 2.5 Long-Term Postoperative Complications and Follow-Up: During the follow-up period of over one year post-surgery, the following long-term complications were observed: one case of portal vein stenosis (1 year post-surgery, pediatric group), one case of hepatic artery stenosis (1 year post-surgery, pediatric group), and one case of bowel obstruction (5 years post-surgery, pediatric group). Three patients experienced episodes of rejection, with the first acute rejection occurring at 1 year, 3.2 years, and 5.2 years post-surgery, all of which improved with treatment. However, the first two patients experienced a second acute rejection episode at 5 and 6 years post-surgery, respectively, both of which also responded to treatment. Chronic renal failure developed in 6 patients, with the onset ranging from 12.1 to 46.7 months post-surgery (detailed in Table 2). 2.6 Follow-Up: In follow-up evaluations of patients monitored for more than 6 months, there were no significant differences in laboratory test results between adult and pediatric patients (as detailed in Table 3). Relevant data for patients with chronic renal failure are provided in Table 4. Of the total cohort, 4 patients (20%) died during the follow-up period. In the LDLT group, 3 patients died, while 1 patient in the DDLT group died, with no statistically significant difference in mortality rates between the two groups. The causes of death included heart failure on postoperative day 8, unexplained sudden death at 6 months post-surgery, respiratory failure due to pulmonary infection at 1 year post-surgery, and death from an unknown cause at 5.2 years post-surgery. Among the 16 surviving patients, the median follow-up time was 74.6 months (ranging from 7 to 125 months). The 1-year, 3-year, and 5-year survival rates for the entire cohort were 90%, 85%, and 78.5%, respectively. Discussion Caroli disease (CD) is a rare congenital disorder characterized by multifocal segmental dilation of the intrahepatic bile ducts, often associated with varying degrees of cystic kidney disease. It is distinguished by bile duct dilation without other significant liver abnormalities [3]. In contrast, Caroli syndrome (CS) involves bile duct dilation along with congenital hepatic fibrosis [4]. Caroli disease is extremely rare, with an estimated prevalence of approximately 1 in 1,000,000, while Caroli syndrome is more common, with a prevalence of around 1 in 100,000 [5]. CS is frequently associated with autosomal recessive polycystic kidney disease (ARPKD), which is caused by pathogenic mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene [1]. The PKHD1 gene is primarily expressed in the kidneys, with lower levels of expression in the liver, pancreas, and lungs. This expression pattern aligns with the disease phenotype, which primarily affects the liver and kidneys. Other studies have suggested that mutations in the WDR19 gene can also lead to either Caroli disease or Caroli syndrome [6]. Clinically, Caroli syndrome (CS) presents with a wide range of symptoms, but commonly includes right upper quadrant pain, fever, jaundice, pruritus, and, in some cases, signs of portal hypertension. As the disease progresses, recurrent cholangitis, abscesses, sepsis, pancreatitis, and even cholangiocarcinoma may develop, complicating the clinical course [7]. Therefore, early diagnosis of Caroli disease (CD) and CS in patients with recurrent cholangitis is essential, as it enables the identification of the best treatment options, including liver resection and liver transplantation [8]. Research has shown that identifying the “central dot sign” within dilated bile ducts in the early stages of the disease can aid in the early diagnosis of CD [9-11]. This study, through a retrospective analysis of clinical data from 20 patients with Caroli syndrome who underwent liver transplantation, confirmed the effectiveness of liver transplantation in treating this disease and its long-term survival benefits. The 1-year, 3-year, and 5-year survival rates for the cohort were 90%, 85%, and 78.5%, respectively, which align with the reported survival rates for Caroli syndrome patients in the UNOS/OPTN database (approximately 90% 1-year survival) [12] and the findings of Habib et al. [13], further supporting liver transplantation as a curative treatment for end-stage Caroli syndrome. In this study, 13 patients (65%) underwent living-donor liver transplantation (LDLT), and 7 patients (35%) received deceased-donor liver transplantation (DDLT). No significant difference in survival rates was observed between the two groups. This finding suggests that LDLT can be an effective alternative to DDLT in situations where donor availability is limited, particularly for pediatric patients, who comprised 75% of this cohort. The technical feasibility and safety of LDLT were confirmed through appropriate graft weight matching (224–1327g) and graft-to-recipient weight ratio (GRWR) (0.76%–4.90%). Additionally, 3 patients underwent simultaneous partial splenectomy, likely to address portal hypertension-related complications, although the long-term impact on prognosis requires further investigation. In terms of long-term postoperative complications, chronic renal failure (6 cases, 30%) and acute rejection (4 cases, 20%) were the most prominent. The onset of chronic renal failure (with a median onset time ranging from 12.1 to 46.7 months) was closely associated with preoperative comorbid polycystic kidney disease (16 cases, 80%) and postoperative nephrotoxicity from calcineurin inhibitors (CNI). Literature suggests that Caroli syndrome patients often have associated autosomal recessive polycystic kidney disease (ARPKD), and renal function may continue to deteriorate after liver transplantation [52]. In this study, one patient eventually required a kidney transplant. Therefore, for patients with pre-existing renal impairment (2 cases) or polycystic kidney disease, it is recommended to reduce or substitute CNI drugs (e.g., by using sirolimus in combination with mycophenolate mofetil) to protect renal function. Alternatively, combined liver-kidney transplantation (CLKT) should be prioritized, especially for those requiring renal replacement therapy (RRT) [1]. The incidence of chronic renal failure before surgery was significantly higher in the adult group compared to the pediatric group (p=0.010), likely due to the longer disease course and more severe renal involvement in adults. Additionally, postoperative AST levels were significantly higher in the pediatric group than in the adult group (p=0.008), suggesting differences in liver metabolism and repair mechanisms between children and adults. However, the clinical significance of this finding requires further evaluation, particularly in conjunction with long-term follow-up. It is worth noting that, despite the pediatric group having more preoperative comorbidities (e.g., biliary tract infections, congenital hepatic fibrosis), their survival rate was not significantly different from that of the adult group. This underscores the feasibility of liver transplantation in pediatric patients. Although no cases of cholangiocarcinoma were observed in this study, literature reports indicate that patients with Caroli disease/syndrome are at significantly higher risk for cholangiocarcinoma, with the risk increasing by up to 100 times compared to the general population [14, 15]. Therefore, long-term follow-up should include enhanced imaging surveillance (such as MRI/MRCP) and screening for tumor markers (e.g., CA19-9). Additionally, the occurrence of acute rejection (4 cases) and secondary rejection (3 cases) after surgery highlights the need for individualized adjustments to immunosuppressive regimens. For patients with recurrent rejection episodes, exploring precision medication strategies based on immune cell function testing could help strike a balance between managing rejection and minimizing the risks of infection or tumor development due to excessive immunosuppression. This study has several limitations. First, it is a single-center retrospective analysis with a small sample size (n=20), which may introduce selection bias. Additionally, the longest follow-up period was only 10 years, so the evaluation of long-term complications (such as cholangiocarcinoma) is not fully comprehensive. Future studies should involve multi-center, large-scale prospective research and incorporate molecular biomarkers (e.g., PKHD1 gene mutation analysis) to further optimize the timing of surgery and postoperative management strategies. Declarations Ethics approval and consent to participate This retrospective study utilized anonymized patient data and was approved by the Beijing Friendship Hospital Ethics Committee with a waiver of informed consent (Approval Number: 20241201). All procedures complied with the ethical standards of China and the principles of the Declaration of Helsinki. Consent for publication Not applicable. This study did not include any identifiable personal data, images, or videos. Availability of data and materials The de-identified datasets generated and/or analyzed during the current study are available from the corresponding author upon reasonable request. Raw data are not publicly accessible to protect patient privacy. Competing interests The authors declare that they have no competing interests, financial or non-financial. Funding This research received no specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Authors' contributions Fei Hou ,Hao-Feng Xiong: Conceptualization, data analysis, and manuscript drafting ; Ying Liu, Shuang Zhao, Yizhi Zhang, Qian Kang, Wei Qu,Zhigui Zeng,Lin Wei: Data curation and validation; Zhijun Zhu, Liying Sun: Methodology and critical revision. All authors reviewed and approved the final manuscript. Acknowledgements Not applicable. References Uchida H, Inui A, Okamoto T, et al. Optimal transplant strategy of pediatric liver transplantation for fibropolycystic liver disease: Multicenter retrospective study in Japan. Hepatol Res. 2024.Oct 15. doi: 10.1111/hepr.14122. Online ahead of print. EASL Clinical Practice Guidelines on the management of cystic liver diseases. J Hepatol. 2022;77(4):1083-1108. Orozco G, Shah MB, Gupta M, et al. Liver transplantation for biliary cysts: perioperative and long-term outcomes. HPB (Oxford). 2023;25(8):954-961. Caleça Emidio F, Costa Pereira R, Blanco R, Santos P, Abegão T, Fitas C. AN 84-YEAR-OLD PATIENT WITH CAROLI SYNDROME: WHAT IS THE PROGNOSIS OF THIS CONDITION? Eur J Case Rep Intern Med. 2023;10(3):003794. Ghassa A, Khouri L. Caroli syndrome associated with atrial septal defect and polydactyly: a case report. J Med Case Rep. 2023;17(1):213. Liu L, Huang Y, Fang F, Zhou H, Liu X. A case report of intrahepatic bile duct dilatation caused by WDR19 gene mutation and presented as Caroli syndrome. Transl Pediatr. 2024;13(4):682-689. Shi W, Huang XM, Feng YL, Wang FD, Gao XX, Jiao Y. Factors contributing to diagnostic delay of Caroli syndrome: a single-center, retrospective study. BMC Gastroenterol. 2020;20(1):317. Fahrner R, Dennler SGC, Dondorf F, Ardelt M, Rauchfuss F, Settmacher U. Liver resection and transplantation in Caroli disease and syndrome. J Visc Surg. 2019;156(2):91-95. Rivas A, Epelman M, Danzer E, Adzick NS, Victoria T. Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease. Radiol Case Rep. 2019;14(2):265-268. Perricone G. Image of the month: Caroli syndrome: central dot sign on CT. Am J Gastroenterol. 2015;110(4):497. Perricone G, Vanzulli A. Education and imaging. Hepatology: "central dot sign" of Caroli syndrome. J Gastroenterol Hepatol. 2015;30(2):234. Wen JW, Furth SL, Ruebner RL. Kidney and liver transplantation in children with fibrocystic liver-kidney disease: data from the US Scientific Registry of Transplant Recipients: 1990-2010. Pediatr Transplant. 2014;18(7):726-732. Yaghi M, Jaafar R, Kanso M, Khalife M, Faraj W. Choledochal anomalies in adults: A 20-year single-center retrospective cohort experience in the Middle East. Arab J Gastroenterol. 2022;23(4):235-240. Lasagni A, Cadamuro M, Morana G, Fabris L, Strazzabosco M. Fibrocystic liver disease: novel concepts and translational perspectives. Transl Gastroenterol Hepatol. 2021;6:26. Fahrner R, Dennler SG, Inderbitzin D. Risk of malignancy in Caroli disease and syndrome: A systematic review. World J Gastroenterol. 2020;26(31):4718-4728. Tables Table 1. Comparison of General Characteristics and Preoperative Major Comorbidities Between Adult and Pediatric Patients with Caroli Syndrome Adults (5 cases) Children (15 cases) Chi-square p Gender (F/M) 4/1 1/14 10.756 0.001 # Age (years) 37.6±11.7 7.0±3.3 Prognosis 0.741 0.389 Cured 5 13 Death 0 2 Preoperative biliary infection 0 2 0.741 0.389 Hypersplenism 5 12 1.176 0.278 Hypoproteinemia 5 9 2.857 0.091 Liver cirrhosis 5 13 0.741 0.389 Portal hypertension 5 12 1.176 0.278 Gastrointestinal bleeding 1 7 1.111 0.292 Preoperative chronic kidney injury 2 1 6.667 0.010 # Ascites 3 10 0.073 0.787 Hepatopulmonary syndrome 2 1 4.157 0.125 Polycystic kidney 5 10 2.222 0.136 Pulmonary hypertension 2 1 3.268 0.071 Note: Compared to pediatric patients, adults had a higher proportion of females and were more prone to preoperative chronic kidney injury. No other preoperative comorbidities showed statistically significant differences. Table 2. Comparison of Surgical-Related and Postoperative Major Complications Between Adult and Pediatric Patients with Caroli Syndrome Category Adults (5 cases) Children (15 cases) T p Organ Source 1.832 0.176 - LDLT 2 11 - DDLT 3 4 Splenectomy 0.131 0.718 - No splenectomy 4 13 - Partial splenectomy 1 2 Type of Living Graft 3.746 0.587 - Left lobe with MHV 1 5 - Left lobe without MHV 0 1 - Right lobe without MHV 1 1 - Left lateral segment 0 3 - Reduced-size left lateral 0 1 Postoperative hypertension 0 1 0.741 0.389 Postoperative AKI 3 5 1.111 0.292 Postoperative acute rejection 1 3 0 1 Postoperative chronic renal failure 2 3 0.8 0.371 Respiratory failure 0 1 0.351 0.554 Heart failure 1 1 0.741 0.389 Portal vein stenosis 0 1 0.351 0.554 Hepatic artery stenosis 0 1 0.351 0.554 Intra-abdominal hemorrhage 2 1 3.268 0.071 Seizure 1 0 3.158 0.076 Cerebral infarction 1 0 3.158 0.076 Note: LDLT = Living donor liver transplantation; DDLT = Deceased donor liver transplantation; MHV = Middle hepatic vein; AKI = Acute kidney injury. There were no statistically significant differences between adult and pediatric patients in terms of donor source, graft type, surgical approach, or early postoperative complications. Table 3. Comparison of Major Laboratory Tests During Post-Transplant Follow-Up Between Pediatric and Adult Patients with Caroli Syndrome Parameter Postoperative Last Follow-Up T p Cohen's d Children Adults WBC (×10^9/L) 6.7 ± 4.0 6.9 ± 3.1 -0.127 0.900 0.067 Hgb (g/L) 130.7 ± 20.1 112.0 ± 20.5 1.76 0.097 0.926 PLT (×10^9/L) 199.8 ± 51.6 173.0 ± 77.6 0.862 0.402 0.453 PT (s) 13.8 ± 4.2 11.9 ± 2.2 0.922 0.370 0.485 PTA (%) 76.7 ± 19.2 90.0 ± 21.2 -1.258 0.226 0.662 INR 1.26 ± 0.38 1.11 ± 0.21 0.8 0.435 0.421 ALT (IU/L) 24.2 ± 15.3 12.4 ± 5.8 1.659 0.117 0.873 AST (IU/L) 30.2 ± 8.5 17.6 ± 6.0 3.029 0.008 1.594 TBIL (μmol/L) 12.7 ± 6.4 10.3 ± 2.7 0.798 0.436 0.420 DBIL (μmol/L) 4.1 ± 1.3 3.8 ± 0.5 0.521 0.609 0.274 Alb (g/L) 42.6 ± 2.4 40.0 ± 2.5 2.007 0.062 1.056 ALP (IU/L) 309.6 ± 288.7 177.0 ± 122.6 0.979 0.342 0.515 GGT (IU/L) 41.5 ± 56.3 40.6 ± 31.3 0.0335 0.973 0.018 BUN (μmol/L) 8.1 ± 5.7 13.2 ± 9.5 -1.403 0.180 0.738 Cr (μmol/L) 98.4 ± 87.1 221.7 ± 150.2 -2.199 0.043 1.157 Table 4. Summary of Clinical Data for Patients with Combined Renal Impairment No. Gender Age (Surgery) Surgery Date Polycystic Kidney Liver Donor Type Splenectomy Preoperative Renal Injury Preoperative Peak Cr (μmol/L) Postoperative Renal Injury Postoperative Peak Cr (μmol/L) Follow-Up Outcome 1 Male 11 2015-10-03 Yes Deceased donor No splenectomy Yes 105.9 Yes 154 Liver and kidney function normalized 2 Male 8 2017-08-11 Yes Left lobe Partial splenectomy No 58.2 Yes 126 Death (5 years post-op, November 2022, cause unknown) 3 Female 56 2018-07-01 Yes Deceased donor No splenectomy Yes 130 Yes 130 Liver and kidney function normalized 4 Male 8 2018-12-25 Yes Left lateral segment No splenectomy No 99.2 Yes 99 Death (1 year post-op due to pulmonary infection) 5 Male 5 2019-10-31 Yes Left lobe without MHV No splenectomy No 75.8 Yes 130 Liver and kidney function normalized 6 Male 6 2019-10-22 Yes Deceased donor No splenectomy No 34.4 Yes 131 Death (3 days post-op due to uncontrolled intra-abdominal hemorrhage, hemorrhagic shock) 7 Female 38 2019-11-06 Yes Deceased donor No splenectomy Yes 281 Yes 700 Normal liver function. Underwent kidney transplant in August 2024; currently alive. 8 Male 29 2024-05-14 Yes Right lobe without MHV Partial splenectomy Yes 324 Yes 518 Death (6 months post-op, December 2024, sudden death at home, cause unknown) Cite Share Download PDF Status: Published Journal Publication published 02 Oct, 2025 Read the published version in Orphanet Journal of Rare Diseases → Version 1 posted Editorial decision: Major revision 12 Apr, 2025 Reviewers agreed at journal 23 Mar, 2025 Reviewers invited by journal 23 Mar, 2025 Editor assigned by journal 17 Mar, 2025 First submitted to journal 11 Mar, 2025 You are reading this latest preprint version 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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Hospital","correspondingAuthor":false,"prefix":"","firstName":"Lin","middleName":"","lastName":"Wei","suffix":""},{"id":432815022,"identity":"0f82647f-b09b-46dd-8f77-3790758f20c9","order_by":8,"name":"Wei Qu","email":"","orcid":"","institution":"Capital Medical University Affiliated Beijing Friendship Hospital","correspondingAuthor":false,"prefix":"","firstName":"Wei","middleName":"","lastName":"Qu","suffix":""},{"id":432815023,"identity":"fbe725ea-1242-4a77-ae46-8847d8b077b9","order_by":9,"name":"Zhigui Zeng","email":"","orcid":"","institution":"Capital Medical University Affiliated Beijing Friendship Hospital","correspondingAuthor":false,"prefix":"","firstName":"Zhigui","middleName":"","lastName":"Zeng","suffix":""},{"id":432815024,"identity":"6e66d935-c030-4ac2-9761-b50c72143319","order_by":10,"name":"Zhijun Zhu","email":"","orcid":"","institution":"Capital Medical University Affiliated Beijing Friendship Hospital","correspondingAuthor":false,"prefix":"","firstName":"Zhijun","middleName":"","lastName":"Zhu","suffix":""},{"id":432815025,"identity":"0b3e0d80-c34b-4375-8566-7c633befd0bb","order_by":11,"name":"Li-Ying Sun","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAvElEQVRIiWNgGAWjYLCCBww2QJK5gYHhALFaEhjSGBjYGEnTcpgELfIzco9JJLadlzO439j44MMZBnl+MQL6DG7kJRsknLltbHCMsdlwxg0Gw5mzEwhokcgxfJBQcTtxwzHGNmmeDwwJBrcJaJGfkWNwIMHgHAlaGG6AbTkA1XKDCC0GZ94YA/2SbCx5LBHolzMShP0i355jJvGxzU6O7/Dhgw8+HLOR55cm5DA0IEGa8lEwCkbBKBgF2AEARNVGXP87Xd8AAAAASUVORK5CYII=","orcid":"https://orcid.org/0000-0003-1101-7994","institution":"Capital Medical University Affiliated Beijing Friendship Hospital","correspondingAuthor":true,"prefix":"","firstName":"Li-Ying","middleName":"","lastName":"Sun","suffix":""}],"badges":[],"createdAt":"2025-03-11 17:59:03","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6205735/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6205735/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s13023-025-03943-6","type":"published","date":"2025-10-02T15:58:04+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":92884015,"identity":"a49298fb-71ba-4d0a-938d-af814831ee82","added_by":"auto","created_at":"2025-10-06 16:12:06","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":638114,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6205735/v1/25f15f7b-eef8-419a-bb8e-59812a7f8598.pdf"}],"financialInterests":"","formattedTitle":"Clinical Efficacy of Liver Transplantation in Caroli Syndrome: A Retrospective Analysis","fulltext":[{"header":"Introduction","content":"\u003cp\u003eCaroli syndrome is a congenital disorder inherited in an autosomal recessive pattern, characterized by multifocal segmental dilation of the intrahepatic bile ducts, often accompanied by congenital hepatic fibrosis (CHF) and varying degrees of renal cystic diseases [1]. The treatment for Caroli syndrome is primarily supportive; however, patients who experience recurrent biliary infections, particularly those with complications related to portal hypertension, may require liver transplantation. There are limited reports on the clinical efficacy and long-term prognosis of liver transplantation for Caroli syndrome. This study uses a retrospective case analysis to summarize the clinical features and outcomes of 20 patients with Caroli syndrome who underwent liver transplantation, and to conduct follow-up evaluations to assess the efficacy of liver transplantation in managing this condition.\u003c/p\u003e"},{"header":"1.\tSubjects","content":"\u003cp\u003eA search was conducted to identify patients who underwent liver transplantation at Beijing Friendship Hospital, Capital Medical University, between April 2014 and May 2024, and were discharged with a diagnosis of Caroli syndrome. The diagnostic criteria were as follows:\u003c/p\u003e\n\u003cp\u003e(1) The diagnosis of Caroli disease was based on imaging findings (such as CT, MRI, or ultrasound), which revealed multiple cystic or cylindrical low-density lesions in the liver that communicate with the intrahepatic bile ducts, displaying the \u0026ldquo;tadpole sign\u0026rdquo; or \u0026ldquo;hanging sign,\u0026rdquo; while the common bile duct remains normal.\u003c/p\u003e\n\u003cp\u003e(2) The diagnosis of Caroli syndrome was made when Caroli disease was complicated by portal hypertension, as evidenced by esophageal and/or gastric varices, portal hypertensive bleeding, and/or imaging studies showing portosystemic shunting .\u003c/p\u003e"},{"header":"2. Methods","content":"\u003cp\u003e(1) Data Collection: The following data were collected from the patients: demographic information, laboratory test results (including liver and renal function, complete blood count, coagulation tests, and urinalysis), preoperative comorbidities (such as cirrhosis, portal hypertension, gastrointestinal bleeding, renal injury, ascites, hepatopulmonary syndrome, pulmonary hypertension, cholangitis, hypoalbuminemia, splenomegaly, etc.), surgical details (including organ source, whether a cross-match transplant was performed, whether splenectomy was done, whether auxiliary liver transplantation was used, graft weight, graft-to-recipient weight ratio, ischemia time, etc.), liver disease-related scores (such as Child-Pugh-Turcotte score, MELD score, PELD score, SOFA score, etc.), and postoperative complications (including respiratory failure, heart failure, chronic renal failure, rejection, and vascular complications).\u003c/p\u003e\n\u003cp\u003eAdditionally, the patients were divided into two groups: pediatric (\u0026le;18 years) and adult (\u0026gt;18 years). Differences between the two groups in terms of preoperative and postoperative comorbidities, as well as follow-up parameters, were compared. All patients were followed up postoperatively, with the follow-up period concluding in February 2025.\u003c/p\u003e\n\u003cp\u003e(2) Statistical Analysis: All statistical analyses were conducted using SPSS version 25.0 software. For normally distributed quantitative variables, data are presented as mean \u0026plusmn; standard deviation (M \u0026plusmn; SD), and group differences were assessed using the t-test. For non-normally distributed variables, data are presented as median (first quartile, third quartile) or Q2 (Q1, Q3), with group differences evaluated using the Mann-Whitney U test. Categorical variables are expressed as frequencies or percentages, with differences in proportions between groups compared using the chi-square test or Fisher\u0026rsquo;s exact test. A significance level of \u0026alpha; = 0.05 was set, and p \u0026lt; 0.05 was considered statistically significant.\u003c/p\u003e"},{"header":"3. Results","content":"\u003cp\u003e(1) General Information:\u003c/p\u003e\n\u003cp\u003eBetween April 2014 and May 2024, a total of 20 patients diagnosed with Caroli syndrome underwent liver transplantation at Beijing Friendship Hospital, Capital Medical University, representing 1.23% (20/1623) of all liver transplant cases during this period. The ages of the patients at the time of surgery ranged from 3 to 56 years, with a median age of 8 years (range: 6 to 20.5 years). Of these, 13 patients (65%) received living-donor liver transplants (LDLT), and 7 patients (35%) received deceased-donor liver transplants (DDLT). The cohort consisted of 15 male patients and 5 female patients, with 15 patients (75%) being children and 5 patients (25%) being adults.\u003c/p\u003e\n\u003cp\u003e(2) Preoperative Major Comorbidities: \u0026nbsp;\u003c/p\u003e\n\u003cp\u003ePreoperative comorbidities included cirrhosis(18 patients), portal hypertension(17), splenomegaly(17), polycystic kidney disease(16), hypoalbuminemia(14), ascites(13), gastrointestinal bleeding(8), hepatomegaly(9), renal injury(2), pulmonary hypertension(3), hepatopulmonary syndrome(1), congenital liver fibrosis(2), and cholangitis(1).\u003c/p\u003e\n\u003cp\u003eWhen comparing adult and pediatric patients, females were more prevalent in the pediatric group, while chronic renal failure was more commonly observed in adults prior to surgery. No significant differences were found in other preoperative comorbidities. A detailed comparison of the general characteristics and preoperative major comorbidities of pediatric and adult patients is provided in Table 1.\u003c/p\u003e\n\u003cp\u003e2.3 Intraoperative Conditions:\u003c/p\u003e\n\u003cp\u003eThe APACHE II score during surgery ranged from 4 to 27, with a mean of 14.4±5.9, and the predicted mortality ranged from 5.1% to 72.5%, with a mean of 29.4±17.3%. The SOFA score ranged from 0 to 13, with a median of 4 (interquartile range 2.5 to 5.0), and the CLIF-SOFA score ranged from 0 to 7, with a median of 2 (interquartile range 0.5 to 4.5). The PELD score (for children under 12) ranged from 0 to 28, with a median of 11 (interquartile range 6 to 17.5), and the MELD score ranged from 9 to 24, with a mean of 16.7±7.3. The graft weight ranged from 224 to 1327 g, with a mean of 492.1±331.1 g, and the graft-to-recipient weight ratio (GRWR) ranged from 0.76% to 4.90%, with a mean of 1.7±1.2%. The warm ischemia time ranged from 2 to 8 minutes, with a mean of 3.4±1.7 minutes, and the cold ischemia time ranged from 30 to 657 minutes, with a mean of 233.2±209.3 minutes. None of the patients received a cross-match transplant or auxiliary liver transplantation. Three patients underwent simultaneous partial splenectomy. There were no significant differences between adult and pediatric patients regarding donor source, graft type, surgical approach, and other intraoperative factors, as detailed in Table 2.\u003c/p\u003e\n\u003cp\u003e2.4 Early Postoperative Complications:\u003c/p\u003e\n\u003cp\u003eEarly postoperative complications included hypertension in 1 patient (pediatric group), acute kidney injury in 8 patients (3 adults, 5 children), acute rejection in 1 patient (pediatric group), respiratory failure in 1 patient (pediatric group), abdominal hemorrhage in 3 patients (2 adults, 1 child), and seizures and cerebral infarction in 1 patient each (both in the adult group). One patient died during the initial hospitalization, resulting in a 5% mortality rate. The patient passed away on postoperative day 8 due to heart failure. No significant differences were observed in early postoperative complications between adult and pediatric patients, as detailed in Table 2.\u003c/p\u003e\n\u003cp\u003e2.5 Long-Term Postoperative Complications and Follow-Up:\u003c/p\u003e\n\u003cp\u003eDuring the follow-up period of over one year post-surgery, the following long-term complications were observed: one case of portal vein stenosis (1 year post-surgery, pediatric group), one case of hepatic artery stenosis (1 year post-surgery, pediatric group), and one case of bowel obstruction (5 years post-surgery, pediatric group). Three patients experienced episodes of rejection, with the first acute rejection occurring at 1 year, 3.2 years, and 5.2 years post-surgery, all of which improved with treatment. However, the first two patients experienced a second acute rejection episode at 5 and 6 years post-surgery, respectively, both of which also responded to treatment. Chronic renal failure developed in 6 patients, with the onset ranging from 12.1 to 46.7 months post-surgery (detailed in Table 2).\u003c/p\u003e\n\u003cp\u003e2.6 Follow-Up:\u003c/p\u003e\n\u003cp\u003eIn follow-up evaluations of patients monitored for more than 6 months, there were no significant differences in laboratory test results between adult and pediatric patients (as detailed in Table 3). Relevant data for patients with chronic renal failure are provided in Table 4. Of the total cohort, 4 patients (20%) died during the follow-up period. In the LDLT group, 3 patients died, while 1 patient in the DDLT group died, with no statistically significant difference in mortality rates between the two groups. The causes of death included heart failure on postoperative day 8, unexplained sudden death at 6 months post-surgery, respiratory failure due to pulmonary infection at 1 year post-surgery, and death from an unknown cause at 5.2 years post-surgery. Among the 16 surviving patients, the median follow-up time was 74.6 months (ranging from 7 to 125 months). The 1-year, 3-year, and 5-year survival rates for the entire cohort were \u0026nbsp;90%, 85%, and 78.5%, respectively.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eCaroli disease (CD) is a rare congenital disorder characterized by multifocal segmental dilation of the intrahepatic bile ducts, often associated with varying degrees of cystic kidney disease. It is distinguished by bile duct dilation without other significant liver abnormalities [3]. In contrast, Caroli syndrome (CS) involves bile duct dilation along with congenital hepatic fibrosis [4]. Caroli disease is extremely rare, with an estimated prevalence of approximately 1 in 1,000,000, while Caroli syndrome is more common, with a prevalence of around 1 in 100,000 [5]. CS is frequently associated with autosomal recessive polycystic kidney disease (ARPKD), which is caused by pathogenic mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene [1]. The PKHD1 gene is primarily expressed in the kidneys, with lower levels of expression in the liver, pancreas, and lungs. This expression pattern aligns with the disease phenotype, which primarily affects the liver and kidneys. Other studies have suggested that mutations in the WDR19 gene can also lead to either Caroli disease or Caroli syndrome [6].\u003c/p\u003e\n\u003cp\u003eClinically, Caroli syndrome (CS) presents with a wide range of symptoms, but commonly includes right upper quadrant pain, fever, jaundice, pruritus, and, in some cases, signs of portal hypertension. As the disease progresses, recurrent cholangitis, abscesses, sepsis, pancreatitis, and even cholangiocarcinoma may develop, complicating the clinical course [7]. Therefore, early diagnosis of Caroli disease (CD) and CS in patients with recurrent cholangitis is essential, as it enables the identification of the best treatment options, including liver resection and liver transplantation [8]. Research has shown that identifying the\u0026nbsp;\u0026ldquo;central dot sign\u0026rdquo;\u0026nbsp;within dilated bile ducts in the early stages of the disease can aid in the early diagnosis of CD [9-11]. This study, through a retrospective analysis of clinical data from 20 patients with Caroli syndrome who underwent liver transplantation, confirmed the effectiveness of liver transplantation in treating this disease and its long-term survival benefits. The 1-year, 3-year, and 5-year survival rates for the cohort were 90%, 85%, and 78.5%, respectively, which align with the reported survival rates for Caroli syndrome patients in the UNOS/OPTN database (approximately 90% 1-year survival) [12] and the findings of Habib et al. [13], further supporting liver transplantation as a curative treatment for end-stage Caroli syndrome.\u003c/p\u003e\n\u003cp\u003eIn this study, 13 patients (65%) underwent living-donor liver transplantation (LDLT), and 7 patients (35%) received deceased-donor liver transplantation (DDLT). No significant difference in survival rates was observed between the two groups. This finding suggests that LDLT can be an effective alternative to DDLT in situations where donor availability is limited, particularly for pediatric patients, who comprised 75% of this cohort. The technical feasibility and safety of LDLT were confirmed through appropriate graft weight matching (224\u0026ndash;1327g) and graft-to-recipient weight ratio (GRWR) (0.76%\u0026ndash;4.90%). Additionally, 3 patients underwent simultaneous partial splenectomy, likely to address portal hypertension-related complications, although the long-term impact on prognosis requires further investigation.\u003c/p\u003e\n\u003cp\u003eIn terms of long-term postoperative complications, chronic renal failure (6 cases, 30%) and acute rejection (4 cases, 20%) were the most prominent. The onset of chronic renal failure (with a median onset time ranging from 12.1 to 46.7 months) was closely associated with preoperative comorbid polycystic kidney disease (16 cases, 80%) and postoperative nephrotoxicity from calcineurin inhibitors (CNI). Literature suggests that Caroli syndrome patients often have associated autosomal recessive polycystic kidney disease (ARPKD), and renal function may continue to deteriorate after liver transplantation [52]. In this study, one patient eventually required a kidney transplant. Therefore, for patients with pre-existing renal impairment (2 cases) or polycystic kidney disease, it is recommended to reduce or substitute CNI drugs (e.g., by using sirolimus in combination with mycophenolate mofetil) to protect renal function. Alternatively, combined liver-kidney transplantation (CLKT) should be prioritized, especially for those requiring renal replacement therapy (RRT) [1].\u003c/p\u003e\n\u003cp\u003eThe incidence of chronic renal failure before surgery was significantly higher in the adult group compared to the pediatric group (p=0.010), likely due to the longer disease course and more severe renal involvement in adults. Additionally, postoperative AST levels were significantly higher in the pediatric group than in the adult group (p=0.008), suggesting differences in liver metabolism and repair mechanisms between children and adults. However, the clinical significance of this finding requires further evaluation, particularly in conjunction with long-term follow-up. It is worth noting that, despite the pediatric group having more preoperative comorbidities (e.g., biliary tract infections, congenital hepatic fibrosis), their survival rate was not significantly different from that of the adult group. This underscores the feasibility of liver transplantation in pediatric patients.\u003c/p\u003e\n\u003cp\u003eAlthough no cases of cholangiocarcinoma were observed in this study, literature reports indicate that patients with Caroli disease/syndrome are at significantly higher risk for cholangiocarcinoma, with the risk increasing by up to 100 times compared to the general population [14, 15]. Therefore, long-term follow-up should include enhanced imaging surveillance (such as MRI/MRCP) and screening for tumor markers (e.g., CA19-9). Additionally, the occurrence of acute rejection (4 cases) and secondary rejection (3 cases) after surgery highlights the need for individualized adjustments to immunosuppressive regimens. For patients with recurrent rejection episodes, exploring precision medication strategies based on immune cell function testing could help strike a balance between managing rejection and minimizing the risks of infection or tumor development due to excessive immunosuppression.\u003c/p\u003e\n\u003cp\u003eThis study has several limitations. First, it is a single-center retrospective analysis with a small sample size (n=20), which may introduce selection bias. Additionally, the longest follow-up period was only 10 years, so the evaluation of long-term complications (such as cholangiocarcinoma) is not fully comprehensive. Future studies should involve multi-center, large-scale prospective research and incorporate molecular biomarkers (e.g., PKHD1 gene mutation analysis) to further optimize the timing of surgery and postoperative management strategies.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis retrospective study utilized anonymized patient data and was approved by the Beijing Friendship Hospital Ethics Committee with a waiver of informed consent (Approval Number: 20241201). All procedures complied with the ethical standards of China and the principles of the Declaration of Helsinki.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable. This study did not include any identifiable personal data, images, or videos.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe de-identified datasets generated and/or analyzed during the current study are available from the corresponding author upon reasonable request. Raw data are not publicly accessible to protect patient privacy.\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, financial or non-financial.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis research received no specific grant from funding agencies in the public, commercial, or not-for-profit sectors.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026apos; contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eFei Hou ,Hao-Feng Xiong: Conceptualization, data analysis, and manuscript drafting ; Ying Liu, Shuang Zhao, Yizhi Zhang, Qian Kang, Wei Qu,Zhigui Zeng,Lin Wei: Data curation and validation; Zhijun Zhu, Liying Sun: Methodology and critical revision. All authors reviewed and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003eAcknowledgements\u003c/p\u003e\n\u003cp\u003eNot applicable.\u0026nbsp;\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eUchida H, Inui A, Okamoto T, et al. Optimal transplant strategy of pediatric liver transplantation for fibropolycystic liver disease: Multicenter retrospective study in Japan. Hepatol Res. 2024.Oct 15. doi: 10.1111/hepr.14122. Online ahead of print.\u003c/li\u003e\n\u003cli\u003eEASL Clinical Practice Guidelines on the management of cystic liver diseases. J Hepatol. 2022;77(4):1083-1108.\u003c/li\u003e\n\u003cli\u003eOrozco G, Shah MB, Gupta M, et al. Liver transplantation for biliary cysts: perioperative and long-term outcomes. HPB (Oxford). 2023;25(8):954-961.\u003c/li\u003e\n\u003cli\u003eCale\u0026ccedil;a Emidio F, Costa Pereira R, Blanco R, Santos P, Abeg\u0026atilde;o T, Fitas C. AN 84-YEAR-OLD PATIENT WITH CAROLI SYNDROME: WHAT IS THE PROGNOSIS OF THIS CONDITION? Eur J Case Rep Intern Med. 2023;10(3):003794.\u003c/li\u003e\n\u003cli\u003eGhassa A, Khouri L. Caroli syndrome associated with atrial septal defect and polydactyly: a case report. J Med Case Rep. 2023;17(1):213.\u003c/li\u003e\n\u003cli\u003eLiu L, Huang Y, Fang F, Zhou H, Liu X. A case report of intrahepatic bile duct dilatation caused by WDR19 gene mutation and presented as Caroli syndrome. Transl Pediatr. 2024;13(4):682-689.\u003c/li\u003e\n\u003cli\u003eShi W, Huang XM, Feng YL, Wang FD, Gao XX, Jiao Y. Factors contributing to diagnostic delay of Caroli syndrome: a single-center, retrospective study. BMC Gastroenterol. 2020;20(1):317.\u003c/li\u003e\n\u003cli\u003eFahrner R, Dennler SGC, Dondorf F, Ardelt M, Rauchfuss F, Settmacher U. Liver resection and transplantation in Caroli disease and syndrome. J Visc Surg. 2019;156(2):91-95.\u003c/li\u003e\n\u003cli\u003eRivas A, Epelman M, Danzer E, Adzick NS, Victoria T. Prenatal MR imaging features of Caroli syndrome in association with autosomal recessive polycystic kidney disease. Radiol Case Rep. 2019;14(2):265-268.\u003c/li\u003e\n\u003cli\u003ePerricone G. Image of the month: Caroli syndrome: central dot sign on CT. Am J Gastroenterol. 2015;110(4):497.\u003c/li\u003e\n\u003cli\u003ePerricone G, Vanzulli A. Education and imaging. Hepatology: \u0026quot;central dot sign\u0026quot; of Caroli syndrome. J Gastroenterol Hepatol. 2015;30(2):234.\u003c/li\u003e\n\u003cli\u003eWen JW, Furth SL, Ruebner RL. Kidney and liver transplantation in children with fibrocystic liver-kidney disease: data from the US Scientific Registry of Transplant Recipients: 1990-2010. Pediatr Transplant. 2014;18(7):726-732.\u003c/li\u003e\n\u003cli\u003eYaghi M, Jaafar R, Kanso M, Khalife M, Faraj W. Choledochal anomalies in adults: A 20-year single-center retrospective cohort experience in the Middle East. Arab J Gastroenterol. 2022;23(4):235-240.\u003c/li\u003e\n\u003cli\u003eLasagni A, Cadamuro M, Morana G, Fabris L, Strazzabosco M. Fibrocystic liver disease: novel concepts and translational perspectives. Transl Gastroenterol Hepatol. 2021;6:26.\u003c/li\u003e\n\u003cli\u003eFahrner R, Dennler SG, Inderbitzin D. Risk of malignancy in Caroli disease and syndrome: A systematic review. World J Gastroenterol. 2020;26(31):4718-4728.\u003c/li\u003e\n\u003c/ol\u003e"},{"header":"Tables","content":"\u003cp\u003e\u003cstrong\u003eTable 1. Comparison of General Characteristics and Preoperative Major Comorbidities Between Adult and Pediatric Patients with Caroli Syndrome\u003c/strong\u003e\u0026nbsp;\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"680\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003eAdults (5 cases)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003eChildren (15 cases)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003eChi-square\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003ep\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eGender (F/M)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e4/1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e1/14\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e10.756\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e\u0026nbsp; \u0026nbsp; \u0026nbsp; 0.001\u003csup\u003e#\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eAge (years)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e37.6\u0026plusmn;11.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e7.0\u0026plusmn;3.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003ePrognosis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e0.741\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.389\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eCured\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e13\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eDeath\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003ePreoperative biliary infection\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e0.741\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.389\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eHypersplenism\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e12\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e1.176\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.278\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eHypoproteinemia\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e2.857\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.091\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eLiver cirrhosis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e13\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e0.741\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.389\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003ePortal hypertension\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e12\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e1.176\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.278\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eGastrointestinal bleeding\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e1.111\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.292\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003ePreoperative chronic kidney injury\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e6.667\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.010\u003csup\u003e#\u003c/sup\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eAscites\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e10\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e0.073\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.787\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003eHepatopulmonary syndrome\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e4.157\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.125\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003ePolycystic kidney\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e10\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e2.222\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.136\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 19.5588%;\"\u003e\n \u003cp\u003ePulmonary hypertension\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 21.6176%;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 18.9706%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 19.1176%;\"\u003e\n \u003cp\u003e3.268\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20.7353%;\"\u003e\n \u003cp\u003e0.071\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eNote: Compared to pediatric patients, adults had a higher proportion of females and were more prone to preoperative chronic kidney injury. No other preoperative comorbidities showed statistically significant differences.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTable 2. Comparison of Surgical-Related and Postoperative Major Complications Between Adult and Pediatric Patients with Caroli Syndrome\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"671\"\u003e\n \u003cthead\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eCategory\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003eAdults (5 cases)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003eChildren (15 cases)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003eT\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003ep\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/thead\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eOrgan Source\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e1.832\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.176\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- LDLT\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e11\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- DDLT\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eSplenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e0.131\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.718\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- No splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e13\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- Partial splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eType of Living Graft\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e3.746\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.587\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- Left lobe with MHV\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- Left lobe without MHV\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- Right lobe without MHV\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- Left lateral segment\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003e- Reduced-size left lateral\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003ePostoperative hypertension\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e0.741\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.389\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003ePostoperative AKI\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e1.111\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.292\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003ePostoperative acute rejection\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003ePostoperative chronic renal failure\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e0.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.371\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eRespiratory failure\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e0.351\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.554\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eHeart failure\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e0.741\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.389\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003ePortal vein stenosis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e0.351\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.554\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eHepatic artery stenosis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e0.351\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.554\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eIntra-abdominal hemorrhage\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e3.268\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.071\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eSeizure\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e3.158\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.076\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd\u003e\n \u003cp\u003eCerebral infarction\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 11.3124%;\"\u003e\n \u003cp\u003e0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.2126%;\"\u003e\n \u003cp\u003e3.158\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 13.315%;\"\u003e\n \u003cp\u003e0.076\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eNote:\u0026nbsp;LDLT = Living donor liver transplantation; DDLT = Deceased donor liver transplantation; MHV = Middle hepatic vein; AKI = Acute kidney injury.\u003cbr\u003e\u0026nbsp;There were no statistically significant differences between adult and pediatric patients in terms of donor source, graft type, surgical approach, or early postoperative complications.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTable 3. Comparison of Major Laboratory Tests During Post-Transplant Follow-Up Between Pediatric and Adult Patients with Caroli Syndrome\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"629\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd rowspan=\"2\" style=\"width: 103px;\"\u003e\n \u003cp\u003eParameter\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd colspan=\"2\" style=\"width: 285px;\"\u003e\n \u003cp\u003ePostoperative Last Follow-Up\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd rowspan=\"2\" style=\"width: 66px;\"\u003e\n \u003cp\u003eT\u003c/p\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd rowspan=\"2\" style=\"width: 89px;\"\u003e\n \u003cp\u003ep\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd rowspan=\"2\" style=\"width: 87px;\"\u003e\n \u003cp\u003eCohen\u0026apos;s d\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003eChildren\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003eAdults\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eWBC (\u0026times;10^9/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e6.7\u0026nbsp;\u0026plusmn;\u0026nbsp;4.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e6.9\u0026nbsp;\u0026plusmn;\u0026nbsp;3.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e-0.127\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.900\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.067\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eHgb (g/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e130.7\u0026nbsp;\u0026plusmn;\u0026nbsp;20.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e112.0\u0026nbsp;\u0026plusmn;\u0026nbsp;20.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e1.76\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.097\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.926\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003ePLT (\u0026times;10^9/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e199.8\u0026nbsp;\u0026plusmn;\u0026nbsp;51.6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e173.0\u0026nbsp;\u0026plusmn;\u0026nbsp;77.6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e0.862\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.402\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.453\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003ePT (s)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e13.8\u0026nbsp;\u0026plusmn;\u0026nbsp;4.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e11.9\u0026nbsp;\u0026plusmn;\u0026nbsp;2.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e0.922\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.370\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.485\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003ePTA (%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e76.7\u0026nbsp;\u0026plusmn;\u0026nbsp;19.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e90.0\u0026nbsp;\u0026plusmn;\u0026nbsp;21.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e-1.258\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.226\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.662\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eINR\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e1.26\u0026nbsp;\u0026plusmn;\u0026nbsp;0.38\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e1.11\u0026nbsp;\u0026plusmn;\u0026nbsp;0.21\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e0.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.435\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.421\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eALT (IU/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e24.2\u0026nbsp;\u0026plusmn;\u0026nbsp;15.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e12.4\u0026nbsp;\u0026plusmn;\u0026nbsp;5.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e1.659\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.117\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.873\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eAST (IU/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e30.2\u0026nbsp;\u0026plusmn;\u0026nbsp;8.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e17.6\u0026nbsp;\u0026plusmn;\u0026nbsp;6.0\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e3.029\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.008\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e1.594\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eTBIL (\u0026mu;mol/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e12.7\u0026nbsp;\u0026plusmn;\u0026nbsp;6.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e10.3\u0026nbsp;\u0026plusmn;\u0026nbsp;2.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e0.798\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.436\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.420\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eDBIL (\u0026mu;mol/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e4.1\u0026nbsp;\u0026plusmn;\u0026nbsp;1.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e3.8\u0026nbsp;\u0026plusmn;\u0026nbsp;0.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e0.521\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.609\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.274\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eAlb (g/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e42.6\u0026nbsp;\u0026plusmn;\u0026nbsp;2.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e40.0\u0026nbsp;\u0026plusmn;\u0026nbsp;2.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e2.007\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.062\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e1.056\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eALP (IU/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e309.6\u0026nbsp;\u0026plusmn;\u0026nbsp;288.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e177.0\u0026nbsp;\u0026plusmn;\u0026nbsp;122.6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e0.979\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.342\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.515\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eGGT (IU/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e41.5\u0026nbsp;\u0026plusmn;\u0026nbsp;56.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e40.6\u0026nbsp;\u0026plusmn;\u0026nbsp;31.3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e0.0335\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.973\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.018\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eBUN (\u0026mu;mol/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e8.1\u0026nbsp;\u0026plusmn;\u0026nbsp;5.7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e13.2\u0026nbsp;\u0026plusmn;\u0026nbsp;9.5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e-1.403\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.180\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e0.738\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 103px;\"\u003e\n \u003cp\u003eCr (\u0026mu;mol/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 185px;\"\u003e\n \u003cp\u003e98.4\u0026nbsp;\u0026plusmn;\u0026nbsp;87.1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 100px;\"\u003e\n \u003cp\u003e221.7\u0026nbsp;\u0026plusmn;\u0026nbsp;150.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 66px;\"\u003e\n \u003cp\u003e-2.199\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 89px;\"\u003e\n \u003cp\u003e0.043\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e1.157\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTable 4. Summary of Clinical Data for Patients with Combined Renal Impairment\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"1055\"\u003e\n \u003cthead\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003eNo.\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eGender\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003eAge (Surgery)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003eSurgery Date\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003ePolycystic Kidney\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eLiver Donor Type\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003eSplenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003ePreoperative Renal Injury\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003ePreoperative Peak Cr (\u0026mu;mol/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003ePostoperative Renal Injury\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003ePostoperative Peak Cr (\u0026mu;mol/L)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eFollow-Up Outcome\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/thead\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003e1\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003e11\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e2015-10-03\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eDeceased donor\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003eNo splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e105.9\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003e154\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eLiver and kidney function normalized\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003e2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003e8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e2017-08-11\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eLeft lobe\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003ePartial splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e58.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003e126\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eDeath (5 years post-op, November 2022, cause unknown)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003e3\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003e56\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e2018-07-01\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eDeceased donor\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003eNo splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e130\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003e130\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eLiver and kidney function normalized\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003e8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e2018-12-25\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eLeft lateral segment\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003eNo splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e99.2\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003e99\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eDeath (1 year post-op due to pulmonary infection)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003e5\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e2019-10-31\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eLeft lobe without MHV\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003eNo splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e75.8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003e130\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eLiver and kidney function normalized\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003e6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003e6\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e2019-10-22\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eDeceased donor\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003eNo splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003eNo\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e34.4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003e131\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eDeath (3 days post-op due to uncontrolled intra-abdominal hemorrhage, hemorrhagic shock)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003e7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eFemale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003e38\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e2019-11-06\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eDeceased donor\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003eNo splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e281\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003e700\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eNormal liver function. Underwent kidney transplant in August 2024; currently alive.\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 33px;\"\u003e\n \u003cp\u003e8\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 52px;\"\u003e\n \u003cp\u003eMale\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 59px;\"\u003e\n \u003cp\u003e29\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e2024-05-14\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 68px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 72px;\"\u003e\n \u003cp\u003eRight lobe without MHV\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003ePartial splenectomy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 75px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 87px;\"\u003e\n \u003cp\u003e324\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 79px;\"\u003e\n \u003cp\u003eYes\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 86px;\"\u003e\n \u003cp\u003e518\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd style=\"width: 271px;\"\u003e\n \u003cp\u003eDeath (6 months post-op, December 2024, sudden death at home, cause unknown)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\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":"Caroli syndrome, liver transplantation, clinical efficacy, Long-Term Prognosis, Survival Rates, Living Donor Liver Transplantation, Postoperative Complications","lastPublishedDoi":"10.21203/rs.3.rs-6205735/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6205735/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eObjective:\u003c/p\u003e\n\u003cp\u003eTo investigate the clinical efficacy of liver transplantation in treating Caroli syndrome.\u003c/p\u003e\n\u003cp\u003eMethods:\u003c/p\u003e\n\u003cp\u003eA retrospective analysis was conducted on the clinical data of patients diagnosed with Caroli syndrome and undergoing liver transplantation at Beijing Friendship Hospital, Capital Medical University, between April 2014 and May 2024. The analysis focused on baseline characteristics, surgical indicators, postoperative complications, and long-term survival rates. All patients were followed up postoperatively, with the follow-up period ending in February 2025.\u003c/p\u003e\n\u003cp\u003eResults:\u003c/p\u003e\n\u003cp\u003eA total of 20 patients diagnosed with Caroli syndrome underwent liver transplantation at Beijing Friendship Hospital, accounting for 1.23% (20/1623) of all liver transplant patients during the same period. Of these, 13 patients received living donor liver transplants (LDLT), and 7 received deceased donor liver transplants (DDLT). The cohort included 15 male and 5 female patients, with ages ranging from 3 to 56 years (median age 23.3). Preoperative major comorbidities included cirrhosis(18 patients), portal hypertension(17), splenomegaly(17), polycystic kidney disease(16), hypoalbuminemia(14), ascites(13), gastrointestinal bleeding(8), hepatomegaly(9), renal injury(2), pulmonary hypertension(3), hepatopulmonary syndrome(1), congenital liver fibrosis(2), and cholangitis(1). Preoperative APACHE II score ranged from 4 to 27 (mean 14.4 ±5.9), with an expected mortality rate between 5.1% and 72.5% (mean 29.4±17.3). The preoperative SOFA score ranged from 0 to 13 (mean 5.0), CLIF-SOFA score from 0 to 7 (mean 5.0), PELD score (for children under 12) from 0 to 28 (mean 15), and MELD score from 9 to 24 (mean 16.7±7.3). The graft weight ranged from 224 to 1327g (mean 492.1±331.1g), with a graft-to-recipient weight ratio (GRWR) of 0.76% to 4.90% (mean 1.7± 1.2%). The warm ischemia time ranged from 2 to 8 minutes (mean 3.4±1.7 minutes), and cold ischemia time ranged from 30 to 657 minutes (mean 233.2±209.3 minutes). None of the patients received a cross-match transplant or auxiliary liver transplantation. Three patients underwent simultaneous partial splenectomy. Postoperative complications included abdominal hemorrhage in three patients, and seizures in one patient. Comparing adult and pediatric patients, females were more frequent in the pediatric group (p=0.001), and preoperatively, chronic renal failure was more common in adults (p=0.010). There were no significant differences in other preoperative comorbidities, early postoperative complications, or late complications. Long-term postoperative complications included chronic renal failure in six patients (onset between 12.1 and 46.7 months post-surgery) and acute rejection in four patients, three of whom experienced recurrent acute rejection. Four patients (20%) died: one died from postoperative heart failure on day 8, one from unexplained sudden death at 6 months, one from pulmonary infection and respiratory failure at 1 year, and one from an unknown cause at 5.2 years. The 1-year, 3-year, and 5-year survival rates were 90%, 85%, and 78.5%, respectively.\u003c/p\u003e\n\u003cp\u003eConclusion:\u003c/p\u003e\n\u003cp\u003eLiver transplantation significantly improves the survival prognosis for patients with Caroli syndrome, but attention to kidney function preservation and individualized immunosuppressive management is critical for optimal outcomes.\u003c/p\u003e","manuscriptTitle":"Clinical Efficacy of Liver Transplantation in Caroli Syndrome: A Retrospective Analysis","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-04-01 11:42:20","doi":"10.21203/rs.3.rs-6205735/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Major revision","date":"2025-04-12T06:22:04+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"","date":"2025-03-24T01:35:35+00:00","index":0,"fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-03-23T23:11:55+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-03-17T21:41:42+00:00","index":"","fulltext":""},{"type":"submitted","content":"Orphanet Journal of Rare Diseases","date":"2025-03-11T10:07:23+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":"c0beaf98-36be-4e1b-b5c1-d2aa4749e5f0","owner":[],"postedDate":"April 1st, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-10-06T16:07:23+00:00","versionOfRecord":{"articleIdentity":"rs-6205735","link":"https://doi.org/10.1186/s13023-025-03943-6","journal":{"identity":"orphanet-journal-of-rare-diseases","isVorOnly":false,"title":"Orphanet Journal of Rare Diseases"},"publishedOn":"2025-10-02 15:58:04","publishedOnDateReadable":"October 2nd, 2025"},"versionCreatedAt":"2025-04-01 11:42:20","video":"","vorDoi":"10.1186/s13023-025-03943-6","vorDoiUrl":"https://doi.org/10.1186/s13023-025-03943-6","workflowStages":[]},"version":"v1","identity":"rs-6205735","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6205735","identity":"rs-6205735","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}