weekly_question 27/12/2020

[Admin]

A 5 year old underwent a successful hepatoportoenterostomy (HPE - Kasai procedure) as a newborn. She is clinically well with her native liver.

The most common long term complication after successful HPE is

A Failure to thrive

B Abnormal liver synthetic function

C Bowel obstruction

D Malignant transformation

E Cholangitis

[Admin]

correct answer
E Cholangitis

Hepatoportoenterostomy (HPE) allows for restoration of biliary flow relieving the extrahepatic biliary obstruction of biliary atresia. Long term data for those children who do not progress to transplantation is limited, but is beneficial to clinicians caring for these children. Ng et al. looked at the outcomes of 219 patients with biliary atresia beyond five years after a Kasai procedure who did not require liver transplant. Consistent with previous data, they determined that mean weight and height were normal. Furthermore, 75% had normal total bilirubin levels. Other tests of liver synthetic function such as INR and albumin were normal in 73% and 85% of children at 5 years. They did find that almost 98% of patients had clinical or biochemical evidence of chronic liver disease. Cholangitis was reported in 60% and occurred more frequently in the first 1-2 years following HPE. Despite the chronic inflammation that can be found in these children, malignant transformation is rare. These data support the need for continued surveillance throughout childhood, particularly given concerns for the development of portal hypertension.

[Gunduz Aghayev]

e

[Manal Dhaiban]

E
cholangitis is the most common complication after successful HPE

[Emir]

E

[Arwa]

E

[Basma Waseem]

E

[Aras]

E

[Secundino López Ibarra]

E

[Bashar]

E

[Admin]

correct answer
E Cholangitis

Hepatoportoenterostomy (HPE) allows for restoration of biliary flow relieving the extrahepatic biliary obstruction of biliary atresia. Long term data for those children who do not progress to transplantation is limited, but is beneficial to clinicians caring for these children. Ng et al. looked at the outcomes of 219 patients with biliary atresia beyond five years after a Kasai procedure who did not require liver transplant. Consistent with previous data, they determined that mean weight and height were normal. Furthermore, 75% had normal total bilirubin levels. Other tests of liver synthetic function such as INR and albumin were normal in 73% and 85% of children at 5 years. They did find that almost 98% of patients had clinical or biochemical evidence of chronic liver disease. Cholangitis was reported in 60% and occurred more frequently in the first 1-2 years following HPE. Despite the chronic inflammation that can be found in these children, malignant transformation is rare. These data support the need for continued surveillance throughout childhood, particularly given concerns for the development of portal hypertension.