[size=20px]You are asked to see a four-week old female with direct hyperbilirubinemia. Ultrasound suggests a small collapsed gallbladder and HIDA study shows hepatic uptake without secretion. Studies for metabolic and infectious diseases were sent off today and final results should be back in 7-10 days.

 

What is the best next step in managing this child with jaundice?

 

A wait for metabolic and infection studies.

 

B ERCP and stent placement

 

C cholangiogram and liver biopsy

 

D liver transplant

 

E MRCP[/size]

E

C

C

C cholangiogram and liver biopsy

C

C

Correct answer

c cholangiogram and liver biopsy

 

Portoenterostomy continues to be the appropriate first line therapy for biliary atresia. After the introduction of the laparoscopic Kasai, early retrospective and prospective studies reported a significantly worse outcome with the laparoscopic operation. The reason for this difference was unclear. However, more recent studies have shown equivalent results in experienced hands.

 

The operation should not be delayed while awaiting final results for additional testing because it is widely believed that younger infants have better results than older ones. Liver transplant should be reserved for patients who fail the Kasai procedure. Although ERCP has been described with success in a few centers, it is not generally indicated or possible in these patients as their small size makes this procedure very difficult. Even in cases in which ERCP can be performed, it would only be for diagnostic purposes and stent placement would not be indicated.

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