Biliary Atresia With Rare Associations, a Case Report

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 colored urine a few days after birth, which were progressively aggravated.

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 law grade fever of 37.8°C, a weight of 3870 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, 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 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. Preoperative percutaneous liver biopsy reported liver fibrosis, bile ductules 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 behind the liver. Extracorporealization of the liver is done . The gall bladder was rudimentary. On further exploration, the principle investigator also found intestinal malrotation (IM) and PDPV. All extrahepatic bile ducts were absent; type III portal atresia. The portal palate was dissected easily and the rudimentary gall bladder was removed.

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) was 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 revealed features of bile drainage in the intestine; the stool returned to its normal brawn 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 post-operative day.

Inclusion criteria

• Biliary atresia with another syndrome

Exclusion criteria

• Biliary atresia alone