Biliary atresia: outcome and management.

Untreated, biliary atresia remains a fatal condition of the newborn. Most present within four to six weeks of conjugated jaundice and acholic stools and, although still a challenging diagnosis to make, therein lies the opportunity of changing the course of this otherwise inexorable disease. The aim of surgery is to restore bile flow, alleviate jaundice and abbreviate the cholangiodestructive process within the liver. The Kasai portoenterostomy, introduced almost 50 years ago in Japan, aims to expose microscopic biliary ductules within the fibroinflammatory mass at the porta hepatis and restore bile drainage into a mobilised Roux loop. About 50% of infants with BA will be able to clear their jaundice following Kasai alone, given appropriately experienced surgeons and if performed prior to the onset of overt cirrhosis. They have a reasonable expectation of long-term survival to adulthood with a good quality-of-life. The remainder may be candidates for liver transplantation (where available) although donor organ shortage and immunosuppresion-related complications remain significant problems.

Acute and chronic pancreatitis.

Pancreatitis is under appreciated during childhood although its diagnosis is simple and management straightforward in most cases. There is a range of possible causes, which is quite different to the situation in adults. The commonest underlying problems are probably structural abnormalities of the pancreatic and biliary ducts such as choledochal malformation, common pancreatobiliary channel and pancreas divisum. Other causes, which can be important in certain groups and geographical areas, are those due to drug reactions, viral infection and parasitic infestation, and blunt abdominal trauma. The diagnosis is established by showing a significantly raised plasma amylase level. Other diagnostic tools such as ultrasound, computed tomography (CT) scanning and endoscopic retrograde cholangiopancreatography (ERCP) have a major role in determining possible underlying causes, and hence selecting out those who require definitive corrective surgery. The pathophysiology of pancreatitis remains to be fully elucidated and, in the acute phase can affect other organs such as the renal and respiratory systems. Later complications include sepsis, pancreatic abscess and typically pseudocyst formation. Most of these can be treated using minimally invasive techniques such as percutaneous aspiration although open surgical techniques such as cystgastrostomy may be required in a few.