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Published on: August 23, 2022
A study of associated congenital anomalies with biliary atresia
Lucky Gupta1, Veereshwar Bhatnagar1
1Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
Insights
Infants with extrahepatic biliary atresia (EHBA) often have associated anomalies, particularly vascular and digestive system issues. Male infants showed a higher incidence of these anomalies, suggesting a generalized embryonic insult.
Area of Science:
- Pediatric Surgery
- Developmental Biology
- Gastroenterology
Background:
- Extrahepatic biliary atresia (EHBA) is a serious neonatal liver condition.
- Associated congenital anomalies are frequently observed in infants with EHBA.
- Understanding these anomalies is crucial for patient management and prognosis.
Purpose of the Study:
- To determine the incidence and types of anomalies in infants with EHBA.
- To compare these findings with existing literature.
- To evaluate the impact of associated anomalies on EHBA management.
Main Methods:
- Retrospective analysis of 137 infants who underwent the Kasai procedure for EHBA over 12 years.
- Review of medical records to identify and categorize associated anomalies.
- Data collection on patient demographics and management details.
Main Results:
- 40 out of 137 infants (29.2%) had 58 associated anomalies.
- Vascular anomalies (37.9%) and hernias (22.4%) were most common.
- Male infants exhibited a higher incidence of anomalies compared to females.
Conclusions:
- Vascular and digestive system anomalies are most prevalent in EHBA patients.
- The occurrence of anomalies suggests a potential "generalized" embryonic insult.
- Male infants with EHBA have a significantly higher rate of associated anomalies than previously reported.
Background/Purpose:
This study aims to analyze the incidence and type of various associated anomalies among infants with extrahepatic biliary atresia (EHBA), compare their frequency with those quoted in the existing literature and assess their role in the overall management.
Materials And Methods:
A retrospective study was performed on 137 infants who underwent the Kasai procedure for EHBA during the past 12 years. The medical records were reviewed for the incidence and type of associated anomalies in addition to the details of the management of the EHBA.
Results:
Of the137 infants, 40 (29.2%) were diagnosed as having 58 anomalies. The majority of patients had presented in the 3(rd) month of life; mean age was 81 ± 33 days (range = 20-150 days). There were 32 males and 8 females; boys with EHBA had a higher incidence of associated anomalies. Of these 40 patients, 22 (37.9%) had vascular anomalies, 13 patients (22.4%) had hernias (umbilical-10, inguinal-3), 7 patients (12.1%) had intestinal malrotation, 4 patients (6.8%) had choledochal cyst, 1 patient (1.7%) had Meckel's diverticulum, 3 patients (5%) had undergone prior treatment for jejunoileal atresias (jejunal-2, ileal-1), 2 patients (3.4%) had undergone prior treatment for esophageal atresia and tracheoesophageal fistula, 2 patients (3.4%) had spleniculi, and 2 patients (3.4%) were diagnosed as having situs inversus.
Conclusions:
The most common associated anomalies in our study were related to the vascular variation at the porta hepatis and the digestive system. The existence of anomalies in distantly developing anatomic regions in patients with EHBA supports the possibility of a "generalized" insult during embryogenesis rather than a "localized" defect. In addition, male infants were observed to have significantly more associated anomalies as compared with the female infants in contrast to earlier reports.
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