Long-term neurodevelopment in patients treated for biliary atresia

Biliary atresia is a peri-natal liver disease of unknown etiology in which the
extrahepatic bile ducts obliterate. The only option for cure is resection of
the fibrotic tissue and re-establishing bile-flow via a porto-enterostomy, in
which a bowel loop is anastomosed to the liver. Despite a successful operation,
liver fibrosis often progresses. Complications such as cholangitis or
esophageal varices due to progressive fibrosis/cirrhosis occur frequently, and
one third of the patients will need a liver transplantation before the second
year of life. Only some 20% of patients reach their twenties with their own
liver.
As early childhood is a time of critical brain growth severe (liver) disease
has the potential to interfere with the developing brain. As our population
consists of children with isolated biliary atresia which do not have other
defects, this group provides an excellent opportunity to assess the effects of
liver disease and possible transplantation in early childhood on
neurodevelopment, such as the development of memory, attention, planning
skills, inhibition control and visuomotory integration. Besides lengthy and
multiple hospitalizations, other factors associated with severe liver disease
such as malnutrition and encephalopathy may disrupt development.
Little is known about neurodevelopment in children surviving biliary atresia.
Early identification of subgroups at risk for neurodevelopment impairment may
offer the possibility to intervene in an earlier stage, thereby improving
outcome with regards to school functioning and every day life.

This study aims to investigate neurodevelopment in primary school children
surviving biliary atresia when compared to a healthy reference group. Second
aim is to identify prognostic factors associated with a worse outcome in these
children.

Observational cohort study.

Patient and his/her family are confronted with a diagnosis they might have
repressed all these years. Another possible burden could be that the patient
becomes tired (physically and/or mentally) by the end of the testing session,
since the examination will take 2 * 2.5 hours. However, previous studies in our
department have shown that this is a rare event.

Children experiencing significant impairment in various areas of their
cognitive and motor function are at risk of lagging behind in school and
developing psychosocial problems in adolescence and early adulthood. The
identification of possible risk factors in patients with biliary atresia
associated with a worse neurodevelopmental outcome might enable us to identify
these patients at an earlier stage, which might offer therapeutical benefits as
early intervention/counseling has proven to be beneficial to the outcome.