Biliary atresia (BA) is a progressive, obliterative cholangiopathy of infancy characterized by obstruction of bile flow, leading to persistent cholestasis, progressive hepatic fibrosis, and eventual cirrhosis if untreated. It remains the most common indication for pediatric liver transplantation worldwide. Early diagnosis and timely surgical intervention are crucial, as delayed restoration of bile flow is associated with irreversible liver damage and poorer long-term outcomes (1,2). Kasai portoenterostomy (KPE), first introduced in the late 1950s, continues to be the standard first-line surgical treatment for infants with biliary atresia. The procedure aims to re-establish bile drainage by excising the fibrotic extrahepatic biliary remnants and creating a portoenteric conduit at the porta hepatis. When successful, KPE can significantly prolong survival with the native liver and delay or prevent the need for liver transplantation (1,2). Reported two-year transplant-free survival rates following KPE range from approximately 60% to as high as 91.3%, with wide variability across geographic regions and healthcare systems (1,3,4). Several factors have been identified as key determinants of outcome following Kasai portoenterostomy. Age at surgery is among the most consistently reported prognostic factors, with earlier intervention—particularly before 60 days of life—associated with higher rates of jaundice clearance and improved native liver survival (5,6). Surgical expertise and institutional experience also play an important role, influencing both early postoperative outcomes and long-term survival (5,7). In addition, the anatomical pattern of biliary atresia identified at the time of surgery has prognostic significance; certain subtypes, such as those with more favorable intrahepatic bile duct anatomy, are associated with better short- and long-term outcomes (2,8). Despite initial surgical success, a substantial proportion of children experience progressive liver disease and complications such as recurrent cholangitis, portal hypertension, and hepatic dysfunction, necessitating liver transplantation during infancy or early childhood (9,10,11). Long-term studies indicate that even among survivors with native liver, ongoing morbidity remains common, underscoring the need for vigilant follow-up and timely referral for transplantation when indicated (9,10). Various adjuvant therapies following Kasai portoenterostomy, including corticosteroids and other immunomodulatory or antifibrotic agents, have been investigated to enhance bile flow and improve outcomes. However, evidence to date has not demonstrated a consistent or definitive benefit in improving long-term native liver survival (3,12). Emerging research has focused on biochemical and molecular predictors of outcome, including markers of fibrosis, redox imbalance, and hepatocellular injury, which may help refine prognostication and guide individualized management strategies (12,13). Given the substantial variability in outcomes following Kasai portoenterostomy and the multifactorial nature of disease progression, continued evaluation of baseline clinical profiles and perioperative factors remains essential. This study aims to assess two-year survival outcomes following Kasai portoenterostomy in infants with biliary atresia and to examine the association of demographic, clinical, and perioperative variables with prognosis. By integrating surgical timing, biochemical parameters, jaundice clearance, and postoperative complications into a unified analysis, this work seeks to generate clinically actionable evidence to optimize early referral, postoperative surveillance, and decision-making in pediatric hepatobiliary care.

Study Design and Setting A retrospective cohort study was conducted in the Department of Paediatric Surgery at a tertiary care centre in South India. The study included infants diagnosed with biliary atresia who underwent Kasai portoenterostomy between July 2015 and July 2023. Study Population All infants who underwent Kasai portoenterostomy for biliary atresia during the study period were eligible for inclusion. Infants whose parents declined participation and cases that were not traceable through hospital records were excluded from the analysis. A total of 60 infants met the inclusion criteria and were enrolled in the study. Data Collection Clinical data were collected from inpatient case records, operation theatre registers, and the hospital’s electronic and paper-based medical records using a pre-designed and validated data collection proforma. Variables extracted included demographic details, age at surgery, perioperative findings, and postoperative course. Two-year outcome data—including survival status, jaundice clearance, and native liver survival versus post–liver transplantation status—were obtained through review of follow-up records and supplemented by telephonic or in-person interviews with parents when necessary. Outcome Measures The primary outcome measure was two-year survival following Kasai portoenterostomy. Secondary outcomes included jaundice clearance status and native liver survival at two years postoperatively. Statistical Analysis Data were entered into Microsoft Excel (version 14.0) and subsequently exported to SPSS version 27.0 for statistical analysis. Categorical variables were summarized as frequencies and percentages. The 95% confidence interval (CI) was calculated for the mortality rate following Kasai portoenterostomy. Associations between categorical variables and outcomes were assessed using the Chi-square test. A p-value of <0.05 was considered statistically significant.

A total of 60 infants with biliary atresia who underwent Kasai portoenterostomy during the study period were included in the analysis. The results summarize the demographic characteristics, clinical and perioperative variables, and two-year outcomes, including survival status, jaundice clearance, and native liver survival. Associations between these variables and survival outcomes were assessed to identify factors influencing prognosis following Kasai portoenterostomy. Table 1: baseline characteristics of the study cohort Variable Characteristics N % Age <60 26 43.3 60-90 18 30.0 >90 16 26.7 Gender Male 31 51.7 Female 29 48.3 Total Bilirubin (mg/dl) 5-10 44 73.3 >10 16 26.7 GGT value and survival <300 19 31.7 300-600 21 35.0 >600 20 33.3 This table presents the baseline characteristics of the study cohort (n = 60) and shows how these characteristics are distributed across age, gender, total bilirubin (TB), and gamma-glutamyl transferase (GGT) categories with reference to associated survival brackets. The age distribution includes 26 participants younger than 60 years (43.3%), 18 participants aged 60–90 years (30.0%), and 16 participants over 90 years (26.7%). The gender split is nearly balanced, with 31 males (51.7%) and 29 females (48.3%). In terms of liver function at baseline, 44 participants (73.3%) had TB in the 5–10 mg/dL range, while 16 participants (26.7%) had TB greater than 10 mg/dL. Baseline GGT values were categorized as follows: fewer than 300 U/L in 19 participants (31.7%), 300–600 U/L in 21 participants (35.0%), and greater than 600 U/L in 20 participants (33.3%). Table 2: The infection status of the cohort with respect to TORCH testing. Variable Characteristics N % TORCH infection Positive 34 56.7 Negative 26 43.3 Type of infection (n=34) CMV 33 97.1 HSV 10 29.4 Rubella 11 32.4 Toxoplasma 7 20.6 Table 2 summarizes the infection status of the cohort with respect to TORCH testing. Among the 60 participants, 34 (56.7%) tested positive for TORCH infection, while 26 (43.3%) tested negative. Among those with a confirmed TORCH infection (n = 34), the breakdown by infectious agent shows that cytomegalovirus (CMV) was detected in 33 individuals (97.1%), herpes simplex virus (HSV) in 10 individuals (29.4%), rubella in 11 individuals (32.4%), and toxoplasma in 7 individuals (20.6%). These figures reflect the distribution of TORCH-related pathogens within the subset of positive cases and highlight CMV as the predominant infection among the tested positives in this cohort Table 3: Mortality outcomes and vital status in the overall cohort and among TORCH-positive cases Variable Characteristics N % 95% CI for mortality Vital status Alive 22 36.7 49.9-75.4 Dead 38 63.3 Vital status of TORCH positive cases (n=34) Alive 22 64.7 Dead 12 35.3 Table 3 summarizes mortality outcomes and vital status in the overall cohort and among TORCH-positive cases. In the total sample (n = 60), 38 participants died (63.3%), and 22 remained alive (36.7%). The mortality estimate for the overall cohort is accompanied by a 95% confidence interval of 49.9% to 75.4%, reflecting the precision around the observed mortality proportion. Among the 34 TORCH-positive cases, 12 died (35.3%) and 22 remained alive (64.7%), indicating a substantially lower mortality rate within the TORCH-positive subset compared with the entire cohort. These results highlight a differential mortality pattern by TORCH status and underscore the need to explore whether TORCH infection status influences survival after Kasai portoenterostomy in this population. Table 4: Mortality outcomes within the study cohort (n = 60) Variable Characteristics Alive Dead Total X2 p N % N % Age at surgery <60 14 53.8 12 46.2 26 9.9 0.007 60-90 8 44.4 10 55.6 18 >90 1 6.3 15 93.8 16 GGT value and survival <300 6 31.6 13 68.4 19 0.89 0.642 300-600 9 42.9 12 57.1 21 >600 6 30.0 14 70.0 20 This table presents mortality outcomes within the study cohort (n = 60) across two clinical dimensions: age at Kasai surgery and baseline GGT level. The data reveal a clear age-related gradient in survival: surgery before 60 days yields the highest proportion alive (53.8%), with mortality rising to 46.2% in this group; at 60–90 days the alive proportion is 44.4% (55.6% dead); and at >90 days only 6.3% are alive (93.8% dead), indicating markedly worse outcomes with later intervention. By baseline GGT category, mortality is substantial across all groups but does not show a consistent pattern: alive proportions are 31.6% (<300 U/L), 42.9% (300–600 U/L), and 30.0% (>600 U/L), corresponding to dead proportions of 68.4%, 57.1%, and 70.0%, respectively. This study summarizes mortality outcomes in a cohort of 60 participants undergoing Kasai portoenterostomy, examining two clinical dimensions: age at surgery and baseline GGT level. The results show a pronounced age-related gradient in survival, with earlier surgical intervention (before 60 days) associated with the highest proportion alive and markedly better outcomes compared with later surgery (60–90 days and especially >90 days). In contrast, baseline GGT level did not demonstrate a consistent association with mortality; mortality was substantial across all GGT categories without a clear, monotonic pattern. Taken together, the data support prioritizing earlier surgical timing to improve survival, while baseline GGT alone appears insufficient as a standalone predictor of mortality

This study explores the complex interplay between patient demographics, timing of surgery, and survival outcomes following Kasai portoenterostomy (KPE) for biliary atresia (BA). The findings reinforce the critical importance of early surgical intervention, consistent with existing evidence that identifies a narrow therapeutic window for optimal outcomes. Infants undergoing KPE before 60 days of age demonstrated superior survival outcomes, supporting the widely accepted principle that earlier bile drainage improves native liver survival (NLS). Previous studies have shown that delaying surgery beyond 90 days is associated with a significant decline in long-term NLS, with markedly poorer five-year transplant-free survival rates in older infants (6,4). These findings collectively emphasize that timely diagnosis and referral remain paramount in improving prognosis for infants with BA. Beyond surgical timing, this analysis highlights the prognostic relevance of postoperative jaundice clearance and liver biochemical parameters. Jaundice clearance has consistently been identified as a strong predictor of long-term native liver survival, reflecting effective bile drainage following KPE (2). Elevated baseline gamma-glutamyl transferase (GGT) levels have been associated with more advanced biliary injury and worse outcomes in several studies (8,13). However, the present study observed that GGT did not uniformly correlate with two-year survival, suggesting that isolated biochemical markers may have limited prognostic utility when assessed independently. This finding supports prior observations that outcomes in BA are influenced by a multifactorial interplay between liver injury, bile flow restoration, and postoperative disease evolution rather than any single laboratory parameter alone (14,15). Postoperative cholangitis remains one of the most significant complications following KPE and was found to adversely influence survival outcomes in this cohort. Recurrent or severe cholangitis episodes contribute to progressive hepatic inflammation, fibrosis, and deterioration of liver function, ultimately reducing native liver survival (15,16). These findings are consistent with earlier reports documenting the high incidence of cholangitis and its strong association with early failure of KPE. Effective prevention, early detection, and aggressive management of postoperative infections may therefore play a critical role in improving medium- and long-term outcomes following surgery (16,17). The distinction between initial surgical success and eventual progression to liver transplantation underscores an important consideration in the long-term management of biliary atresia. While KPE remains an essential first-line intervention, a substantial proportion of children continue to experience progressive liver disease despite early bile drainage, necessitating transplantation during infancy or early childhood (6,7). These realities highlight the importance of structured follow-up protocols, early identification of surgical failure, and timely referral for transplantation to optimize survival and quality of life. Overall, the findings of this study underscore the multifactorial nature of outcomes following Kasai portoenterostomy. Optimal survival depends not only on early surgical timing but also on comprehensive perioperative and postoperative care, including close monitoring of liver function, effective management of infectious complications, and individualized decision-making regarding transplantation. In resource-limited or decentralized healthcare settings, real-world data such as these provide valuable insight into practical challenges and opportunities for improving outcomes. When interpreted alongside existing literature, the results reinforce the need for refined strategies focused on early detection, timely referral, standardized surgical care, and robust long-term follow-up for infants with biliary atresia.

In conclusion, this analysis brings to light the pressing need to prioritize surgical intervention at an early age, understand the importance of individualized patient management strategies, and develop protocols to mitigate postoperative complications. Future research should focus on identifying risk factors that influence survival rates further while addressing the discrepancies in varying clinical settings to provide optimal care for infants suffering from biliary atresia. Conflict of Interest: Nil

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