A full-term female infant, G1P1, weighing 3450 g at birth, was referred to our department at the age of 2 months old with severe jaundice and poor feeding. The mother reported yellow sclera and skin, lightning-colored stool, and dark urine a few days after birth, which were progressively aggravated. The infant had not received any drugs or medical treatment before, and the parents were relatives.

On physical examination, the infant had an olive-green colored sclera and skin with an itching mark, a pale clay stool in the diaper, a low-grade fever of 37.8 °C, a weight of 3770 g, a pulse rate of 138 b/m, and a respiratory rate of 38 b/m. The heart sounds and breath sounds were normal. The abdomen was soft and lax on examination. The liver was two fingers below the costal margin. The intestinal sound was normal.

The laboratory investigation revealed that total bilirubin was elevated at 7 mg/dl, direct bilirubin was elevated at 4 mg/dl, gamma-glutamyl transferase (GGT) was elevated at 157.1 U/L, alkaline phosphatase was elevated at 279 U/L, and albumin was decreased at 3.4 g/dL.

Abdominal ultrasonography revealed a triangular cord sign in the liver hilum, an absent gall bladder, and a normal-sized spleen on the right side behind the liver. A chest X-ray revealed dextrocardia. The HIDA scan revealed a failure of radioisotope excretion in the duodenum. Non-contrast magnetic resonance cholangiopancreatography (MRCP) showed that the common hepatic duct and the common bile duct could not be seen. A preoperative percutaneous liver biopsy reported liver fibrosis, bile ductule proliferation, and cholestasis.

An experienced pediatric surgeon obtained the decision for exploratory laparotomy by right subcostal abdominal incision. The spleen is located in the right upper quadrant below the liver (Fig. 1). Extracorporealization of the liver is done (Fig. 2). The gall bladder was rudimentary. On further exploration, we also found intestinal malrotation (IM) and PDPV. All extrahepatic bile ducts were absent; type III portal atresia. The portal plate was dissected easily, and the rudimentary gall bladder was removed (Fig. 3).

A surprise is the presence of the spleen in the right upper quadrant below the liver

Our policy is to extra-corporealize the liver

The portal plate was dissected without extra effort than usual. It is suspended in white rubber in the porta-hepatis region, below it is the pre-duodenal portal vein

Widening of the narrow base of the intestinal mesentery, then reconstruction of the retro-colic Roux-en-Y limb of the jejunum, and finally hepato-portoentostomy (Kasai operation) were done. A wedge liver biopsy obtained reported ductular proliferation, hepatic fibrosis, and bile plugs; a feature suggestive of biliary obstruction.

The post-operative follow-up of the patient in the pediatric ICU revealed features of bile drainage in the intestine; the stool returned to its normal brown color; and the yellow skin color was slightly improved. Total bilirubin was 4 mg/dl, and direct bilirubin was 2 mg/dl. The patient was discharged on the tenth postoperative day.

Unfortunately, 3 months later, the patient developed the manifestations of liver cell failure and ascites. The patient died in the intensive care unit.