Biliary Atresia Splenic Malformation: A Case Report from A Tertiary Centre in West Africa and the Lessons Learnt

Abstract

Biliary atresia (BA) is a rare disease characterised by biliary obstruction of unknown origin that presents in the neonatal period. It is classified into syndromic with various congenital anomalies and non-syndromic (isolated anomaly). We present a case of syndromic BA associated with polysplenia and intestinal malrotation, discovered incidentally during the Kasai procedure. The small intestine was found to be non-rotated with the duodenojejunal junction to the right of the vertebral column. The presence of accessory spleens was noted. Kasai portoenterostomy and Ladd's procedure were performed. The patient had an uneventful postoperative course with the passage of cholic stool from the third postoperative day. At the seventh-month follow-up, the stool remained cholic. A multidisciplinary approach in the care of babies with BA and long-term follow-up is crucial for a successful outcome.

Keywords: BASM; Cholestatic jaundice; biliary atresia; intestinal malrotation; polysplenia.

Figure 1

Intraoperative finding showing intestinal non-rotation, with the large bowel- the descending colon, appendix on the left, and small bowel on the right side

Figure 2

Intraoperative finding showing an atretic gallbladder (blue arrow)

Figure 3

Intraoperative photographs showing accessory spleens (blue arrow) and spleen (white arrow)

Figure 4

Intraoperative photograph after dissection of the porta hepatis, showing the right and left portal veins (blue arrow), and the fibrous cone transected at porta hepatis (white arrow)

Figure 5

Intraoperative photograph showing portoenterostomy: (A) placement of posterior wall sutures of the Roux-en-Y jejunal loop, (B) after completion of end-to-side portojejunostomy, and (C) after completion of end-to-side jejuno-jejunostomy