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Updated: Sep 15, 2026
Endoscopic Ultrasound-Guided Biliary Drainage: Endoscopic Ultrasound-Guided Hepaticogastrostomy in Malignant Biliary Obstruction
Published on: March 25, 2022
Biliary atresia: the Brazilian experience
Elisa de Carvalho1, Jorge Luiz dos Santos, Themis Reverbel da Silveira
1Hospital de Base do Distrito Federal, Brasília, DF, Brazil. elisacarvalho@terra.com.br
Insights
Late referral for biliary atresia in Brazil significantly impacts survival rates in children. Earlier diagnosis and intervention are crucial for improving outcomes and reducing the need for liver transplantation.
Area of Science:
- Pediatric Surgery
- Hepatology
- Clinical Epidemiology
Background:
- Biliary atresia is a severe neonatal liver disease requiring timely intervention.
- Early diagnosis and surgical treatment, such as portoenterostomy, are critical for improving prognosis.
- Liver transplantation (LTx) is a life-saving option for advanced cases.
Purpose of the Study:
- To analyze the epidemiological, clinical, and prognostic factors of biliary atresia in Brazilian children.
- To evaluate the impact of surgical timing and treatment strategies on patient survival.
- To identify regional disparities in the management of biliary atresia within Brazil.
Main Methods:
- A multicenter retrospective study involving 513 children diagnosed with biliary atresia between 1982 and 2008.
- Data collected included age at portoenterostomy, liver transplantation (LTx) status, and survival outcomes.
- Patient data were analyzed across different decades of surgery and geographical regions in Brazil.
Main Results:
- Portoenterostomy was performed in 76.4% of patients, with a median age of 82.6 days; 46.6% underwent LTx.
- Survival rates significantly improved over the decades, from the 1980s to the 2000s (p < 0.001).
- Delayed portoenterostomy (>90 days) was associated with lower native liver survival (26.6%) and overall survival (60.5%).
Conclusions:
- Late referral for biliary atresia remains a significant challenge in Brazil, negatively affecting patient survival.
- Improved survival trends correlate with increased rates of liver transplantation over time.
- Developing strategies for earlier detection and referral is essential to decrease the burden of biliary atresia and the need for LTx.
Objective:
To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia.
Methods:
Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers (1982-2008) and compared regarding decades of surgery.
Results:
Of 513 patients, 76.4% underwent portoenterostomy [age: 60-94.7 (82.6±32.8) days] and 46.6% underwent LTx. In 69% of cases, LTx followed portoenterostomy, whereas in 31% of cases LTx was performed as the primary surgery. Patients from the Northeast region underwent portoenterostomy later than infants from Southern (p = 0.008) and Southeastern (p = 0.0012) Brazil, although even in the latter two regions age at portoenterostomy was higher than desirable. Over the decades, LTx was increasingly performed. Overall survival was 67.6%. Survival increased over the decades (1980s vs. 1990s, p = 0.002; 1980s vs. 2000s, p < 0.001; 1990s vs. 2000s, p < 0.001). The 4-year post-portoenterostomy survival, with or without LTx, was 73.4%, inversely correlated with age at portoenterostomy (80, 77.7, 60.5% for ≤ 60, 61-90, > 90 days, respectively). Higher survival rates were observed among transplanted patients (88.3%). The 4-year native liver survival was 36.8%, inversely correlated with age at portoenterostomy (54, 33.3, 26.6% for ≤ 60, 61-90, > 90 days, respectively).
Conclusions:
This multicenter study showed that late referral for biliary atresia is still a problem in Brazil, affecting patient survival. Strategies to enhance earlier referral are currently being developed aiming to decrease the need for liver transplantation in the first years of life.
