Complications
Growth failure is the most common indication for liver transplantation. In the first year of life, many children with biliary atresia have slow growth that needs careful optimisation.
Early recognition and management of cholangitis is very important. It is the most serious and common complication, and without treatment can cause rapid progression of liver disease and death.
Common signs and symptoms are fever, increasing jaundice, acholic stools, and irritability. Cholangitis typically occurs in the first 2 years of life, but it can occur in school-age children. There is some evidence that 1 episode of cholangitis does not necessarily lead to a worse prognosis (i.e., liver transplantation).[70] It requires prompt identification and treatment with intravenous antibiotics for a minimum of 10 days, and up to 6 weeks.
Significant portal hypertension may occur rapidly and necessitate transplantation in the first 2 years of life.
Slowly progressive biliary cirrhosis does not result in significant portal hypertension until later childhood or teenage years. Sclerotherapy and banding are current modalities for therapy. In these patients, portal hypertension does not necessarily lead to liver transplantation and, if bleeding can be controlled, these patients can live into adulthood.
GI bleeding can result from oesophageal varices, gastric varices, gastric ulcers, duodenal varices, duodenal ulcers, or occasionally colonic vessels.
Vitamin A, D, E, and K levels should be checked prior to surgery in patients who present late, or within the first month following HPE.
Children with cholestasis and hepatobiliary inflammation are at risk for poor bone health for a variety of reasons, including vitamin D malabsorption and circulating cytokines that inhibit bone formation. These complications can result in bone fractures, rickets, osteomalacia, and osteopenia.
Children with cholestasis and hepatobiliary inflammation are at risk for poor bone health for a variety of reasons, including vitamin D malabsorption and circulating cytokines that inhibit bone formation. These complications can result in bone fractures, rickets, osteomalacia, and osteopenia.
Children with cholestasis and hepatobiliary inflammation are at risk for poor bone health for a variety of reasons, including vitamin D malabsorption and circulating cytokines that inhibit bone formation. These complications can result in bone fractures, rickets, osteomalacia, and osteopenia.
Children with cholestasis and hepatobiliary inflammation are at risk for poor bone health for a variety of reasons, including vitamin D malabsorption and circulating cytokines that inhibit bone formation. These complications can result in bone fractures, rickets, osteomalacia, and osteopenia.
Ascites can be a sequela of progressive biliary cirrhosis and is common among those patients ultimately listed for liver transplantation. It may also occur temporarily in the context of cholangitis or poor nutrition, and after surgery.
Presentation may resemble cholangitis and requires a high index of clinical suspicion. This would be an indication to consider liver transplantation.
Results from infected ascitic fluid and an inability to clear microorganisms due to cirrhosis and splenic dysfunction.
Results from dyslipidaemia secondary to cirrhosis.
A late complication. May present with shortness of breath and digital clubbing. Treatment is with liver transplantation.
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