A Case Report on Congenital Biliary Atresia with Ventricular Septal Defect

Abstract

ABSTRACT

Introduction: Biliary atresia, an obstructive cholangiopathy with idiopathic etiology is a congenital disorder that eventually destroy liver parenchyma. In some circumstances, it is associated with other abnormalities such as congenital heart diseases, intestinal and spleen anomaly. Diagnosis of biliary atresia is made on clinical manifestations, and specific diagnostic tests. Prolonged pathological jaundice is a critically important sign in newborns that need to be recognized early in order to reduce the chances of further complications. The mainstay of treatment is Kasai procedure (portoenterostomy) and is eighty percent successful if it is performed before or in first two months of life.

Case report and clinical discussion: Here, we report a case of a two-month-old baby who had biliary atresia with ventricular septal defect (VSD). The baby had prolonged history of jaundice, pruritus, yellow colored urine, pale stools. He was initially brought to a local clinic for his jaundice where pharmacological treatment was commenced. However, the symptoms persisted and was referred to a hospital where he was placed under blue light phototherapy with no marked improvement in his symptoms. For definite diagnosis and treatment, he was than referred to a multispecialty hospital where diagnostic tests were ordered. HIDA scan showed no uptake of contrast to bile ducts and gallbladder, which is suggestive of congenital biliary atresia. Intraoperative cholangiogram showed definite diagnosis of biliary atresia, which was performed, and Kasai procedure was done successfully. All his family members were healthy with no history of congenital disease.

Conclusion: The essential outcome in this case is to consider biliary atresia as a cause of prolonged jaundice infants and occasionally it is associated with other congenital anomalies. The diagnosis and treatment should be urgent in order to prevent complications.