A Case Report on Congenital Biliary Atresia with Ventricular Septal Defect


Biliary Atresia
Kasi procedure


How to Cite

Rashid S, Zahier NA, Haidari F, Habibyar AF, Waheb AW, Naseri MW. A Case Report on Congenital Biliary Atresia with Ventricular Septal Defect. Integr J Med Sci [Internet]. 2023 Oct. 24 [cited 2024 Mar. 3];10. Available from: https://mbmj.org/index.php/ijms/article/view/717


Biliary atresia, an obstructive cholangiopathy with an idiopathic etiology, is a congenital disorder that eventually destroys liver parenchyma. In some circumstances, it is associated with other abnormalities such as congenital heart diseases, intestinal, and spleen anomalies. Diagnosis of biliary atresia is based on clinical manifestations and specific diagnostic tests. Prolonged pathological jaundice is a critically important sign in newborns that needs to be recognized early in order to reduce the chances of further complications. The mainstay of treatment is the Kasai procedure (portoenterostomy), and it is eighty percent successful if performed before or within the first two months of life.
Here, we report a case of a two-month-old baby who had biliary atresia with a ventricular septal defect (VSD). The baby had a prolonged history of jaundice, pruritus, yellow-colored urine, and pale stools. He was initially brought to a local clinic for his jaundice, where pharmacological treatment was commenced. However, the symptoms persisted, and he was referred to a hospital where he was placed under blue light phototherapy with no marked improvement in his symptoms. For a definite diagnosis and treatment, he was then referred to a multispecialty hospital where diagnostic tests were ordered. The HIDA scan showed no uptake of contrast in the bile ducts and gallbladder, which is suggestive of congenital biliary atresia. An intraoperative cholangiogram showed a definite diagnosis of biliary atresia, which was performed, and the Kasai procedure was done successfully. All his family members were healthy with no history of congenital disease.
The essential outcome in this case is to consider biliary atresia as a cause of prolonged jaundice in infants, and occasionally it is associated with other congenital anomalies. The diagnosis and treatment should be urgent in order to prevent complications.



Darisi S. A Case of Intrahepatic Biliary Atresia in Pediatrics. Journal of Pediatrics, Perinatology and Child Health. 2021;5(2):130-5. https://doi.org/10.26502/jppch.74050071

Ruíz N P, Aguirre R K, Mesa M C, Lara L L. Case report of syndromic biliary atresia in a pediatric patient. Revista colombiana de Gastroenterología. 2015;30(4):447-55.

Zani A, Quaglia A, Hadzić N, Zuckerman M, Davenport M. Cytomegalovirus-associated biliary atresia: An aetiological and prognostic subgroup. J Pediatr Surg. 2015;50(10):1739-45. https://doi.org/10.1016/j.jpedsurg.2015.03.001

Sharma MS, Poddar U. Biliary atresia and cytomegalovirus infection. Indian Pediatr. 2012 Feb 1;49(2):159.

Hartley JL, Davenport M, Kelly DA. Biliary atresia. The Lancet. 2009;374(9702):1704-13. https://doi.org/10.1016/S0140-6736(09)60946-6

Chardot C. Biliary atresia. Orphanet journal of rare diseases. 2006;1(1):1-9. https://doi.org/10.1186/1750-1172-1-28

Schreiber RA, Harpavat S, Hulscher JB, Wildhaber BE. Biliary atresia in 2021: epidemiology, screening and public policy. Journal of Clinical Medicine. 2022;11(4):999. https://doi.org/10.3390/jcm11040999

Abolurin, O. O., Senbanjo, I. O., Adekoya, A. O., & Ajibola, E. D. (2020). Congenital cytomegalovirus infection as an important cause of infantile cholestatic jaundice: a case report. The Pan African medical journal, 36, 106. https://doi.org/10.11604/pamj.2020.36.106.20577

Feldman, A., & Sokol, R. J. (2013). Alpha-1-Antitrypsin Deficiency: An Important Cause of Pediatric Liver Disease. Lung health professional magazine, 4(2), 8–11.

Kurtoğlu, S., Coban, D., Akın, M. A., Akın, L., & Yıkılmaz, A. (2009). Neonatal sludge: a finding of congenital hypothyroidism. Journal of clinical research in pediatric endocrinology, 1(4), 197–200. https://doi.org/10.4274/jcrpe.v1i4.54

Sahoo, T., Thukral, A., Agarwal, R., & Sankar, M. J. (2015). Galactosaemia: an unusual cause of chronic bilirubin encephalopathy. BMJ case reports, 2015, bcr2014206852. https://doi.org/10.1136/bcr-2014-206852

Singham J, Yoshida EM, Scudamore CH. Choledochal cysts: part 2 of 3: Diagnosis. Can J Surg. 2009;52(6):506-11.

Harpavat S, Finegold MJ, Karpen SJ. Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth. Pediatrics. 2011;128(6):e1428-33. https://doi.org/10.1542/peds.2011-1869

Wang KS, Section on Surgery, Committee on Fetus and Newborn, Childhood Liver Disease Research Network, Moss RL, Caty MG, Davidoff A, Fallat ME, Heiss KF, Holcomb III G, Meyers RL. Newborn screening for biliary atresia. Pediatrics. 2015;136(6):e1663-9. https://doi.org/10.1542/peds.2015-3570

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Copyright (c) 2023 Rashid S et al.


Download data is not yet available.


Metrics Loading ...