Early Kasai portoenterostomy in a 9-day-old newborn with extrahepatic biliary atresia: a case report highlighting improved prognosis with prompt intervention

Abstract

Extrahepatic biliary atresia (EHBA) is a leading cause of neonatal cholestasis, often resulting in end-stage cirrhosis and portal hypertension without early diagnosis and treatment. This report highlights the importance of timely intervention, describing a 6-day-old male newborn diagnosed with EHBA who underwent successful Kasai portoenterostomy at 9 days of age. While the procedure is typically performed within the first 60 days of life, this exceptionally early intervention led to significantly improved outcomes. Postoperative recovery was marked by bilirubin normalization within 2 months and steady improvement in liver function tests, demonstrating the advantages of early surgery. The procedure involved creating a Roux-en-Y hepatic portojejunostomy to restore bile flow, preventing progression to biliary cirrhosis. Early intervention achieved effective bile drainage and substantial clinical improvement. At the 1-year follow-up, the infant displayed normal growth and liver function. This case supports the hypothesis that performing Kasai portoenterostomy earlier than current guidelines recommend may lead to better outcomes. It underscores the need for vigilant neonatal care to recognize early signs of cholestasis and enable prompt surgical intervention. Early diagnosis and intervention can preserve liver function, potentially delaying or preventing the need for liver transplantation.

Keywords: Extrahepatic biliary atresia; Kasai portoenterostomy; case report; early intervention; hepatic portoenterostomy; neonatal cholestasis.

Figure 1.

Four-day-old neonate with jaundiced sclera.

Figure 2.

Clay-colored stool at 4 days of age.

Figure 3.

Surgical steps of Kasai portoenterostomy. (a) Right upper quadrant muscle-cutting incision with mobilization of the liver by division of the falciform ligament. (b, c) The liver was elevated outside the abdominal cavity, showing mobilization of the gallbladder and exposure of the porta hepatis. The liver appeared healthy due to early recognition and intervention and (d) Exposure and dissection of the porta hepatis.

Figure 4.

Stool color changes post-Kasai portoenterostomy indicating successful bile drainage. (a) Colored stool 48 hours postoperatively and (b) More deeply colored stool 7 days postoperatively.