Neurocognitive and Motor Functions in Patients With Biliary Atresia

graphic depiction of brain activity
graphic depiction of brain activity
Investigators assessed neurocognitive and motor development of children with biliary atresia and identified risk factors for impaired development.

Intelligence quotient (IQ) and motor scores were moderately and markedly impaired, respectively, in patients with biliary atresia compared with normative data, according to a study published in Journal of Pediatric Gastroenterology and Nutrition.

To evaluate neurocognitive and motor development of patients with biliary atresia in childhood and adolescence, investigators enrolled patients aged 1 to 20 years from the Helsinki University Children’s Hospital who were followed up between January 1, 2019, and January 31, 2020. All patients underwent age appropriate and validated neurocognitive tests, including the Movement Assessment Battery for children, version 2, for patients aged 3.0 to 16.9 years. The guardians of participants aged 5 to 17 years completed the Five-to-Fifteen-Revised parental questionnaire.

Overall, 39 of 54 eligible patients participated, none of whom had diagnoses of developmental brain abnormalities. The median age of participants 9 (range, 1.2–17.7) years and 16 (41%) participants were living with liver transplantation at the time of assessment. However, there were no significant differences between those having received a transplant and those who did not.

Compared with the normative data (mean IQ, 100±15), mean total IQ of all study patients was significantly lower (mean IQ, 91±15; P <.01). Patient scores remained significantly lower when divided into normal, intermediate, and low scores. Scores among different age groups did not differ significantly. Earlier clearance of jaundice (COJ) had a positive effect on mean total IQ (COJ <3 months total IQ, 96±13 vs COJ ≥3 months postportoenterostomy total IQ, 84±13; P <.05).

Result of the Five-to-Fifteen-Revised parental questionnaire showed significant differences in elevated rates of functional difficulties affecting everyday life among study patients compared with normative data. The numbers of patients scoring in the ≥90th percentile for motor skills, executive functions, memory, language and communication, and learning skills were, 9 of 26 (35%; P <.01), 9 of 26 (35%; P <.01), 10 of 26 (38%; P <.001), 7 of 26 (27%; P <.05) and 8 of 26 (31%; P <.01), respectively.

For the 30 patients who completed motor tests, the total motor score percentile distributions differed significantly from test norms: 56.7% tested normal vs 85%, 16.7% vs 10% were at risk for motor difficulty, and 26.6% vs 5% fulfilled criteria for motor difficulty, respectively (P <.001).

The study had a relatively small sample size due to the rarity of biliary atresia. Additionally, investigators could not analyze potential socioeconomic differences between participants and nonparticipants. Finally, the study’s cross-sectional design prevented assessment of longitudinal neurodevelopmental evolvements.

Researchers concluded, “Considering our findings, cognitive and motor assessment before school-age for all [biliary atresia] patients in order to identify individuals in need of additional support seems highly advisable.”


Ruuska S, Lähteenmäki M, Häyrinen T, et al. Neurocognitive and motor functions in biliary atresia patients: a cross-sectional, prospective national cohort study. J Pediatr Gastroenterol Nutr. 2021;73(4):491-498. doi: 10.1097/MPG.0000000000003206 

This article originally appeared on Gastroenterology Advisor