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Published on: August 25, 2014
Neurodevelopment in patients with biliary atresia up to toddler age: Outcomes and predictability
Marloes H J Dibbits1, Lyan H Rodijk1, Anne E den Heijer2
1Section of Pediatric Surgery, Department of Surgery, University Medical Center Groningen, the Netherlands.
Insights
General Movement Assessment (GMA) after Kasai porto-enterostomy (KPE) in infants with biliary atresia (BA) can predict neurodevelopmental impairments. This assessment helps identify at-risk infants for early intervention.
Area of Science:
- Pediatric Neurology
- Developmental Pediatrics
- Gastroenterology
Background:
- Biliary atresia (BA) is a serious neonatal liver disease requiring surgical intervention.
- Neurodevelopmental outcomes in BA patients can be affected by the condition and its treatment.
- Early identification of neurodevelopmental risks is crucial for timely intervention.
Purpose of the Study:
- To evaluate neurodevelopment in young biliary atresia (BA) patients.
- To determine if General Movement Assessment (GMA) in infancy predicts neurodevelopmental impairments in toddlerhood.
- To assess the predictive accuracy of GMA for motor and cognitive outcomes in BA survivors.
Main Methods:
- Prospective longitudinal study of infants diagnosed with BA.
- Neurodevelopmental assessment using Prechtl's General Movement Assessment (GMA) before and after Kasai porto-enterostomy (KPE).
- Toddler neurodevelopment evaluated using Bayley Scales of Infant Development at 2-3 years; predictive value of GMA analyzed.
Main Results:
- 39% of BA toddlers (n=38) exhibited below-average motor skills; 17% had below-average cognitive scores.
- Abnormal GMA post-KPE demonstrated high sensitivity and specificity in predicting motor and cognitive deficits.
- Positive predictive values for GMA were 77% for motor skills and 33% for cognition; negative predictive values were 94% for both.
Conclusions:
- Approximately one-third of toddlers with BA experience motor skill impairments.
- General Movement Assessment (GMA) performed after Kasai porto-enterostomy (KPE) is a valuable tool for identifying BA infants at risk for neurodevelopmental issues.
- GMA offers a high predictive value for identifying infants requiring closer neurodevelopmental monitoring and support.
Aim:
To assess neurodevelopment in young patients with biliary atresia (BA) and to determine the predictive value of General Movement Assessment (GMA) at infant age for neurodevelopmental impairments at toddler age.
Method:
Infants diagnosed with BA were prospectively included in a longitudinal study. Neurodevelopmental status was previously assessed before Kasai porto-enterostomy (KPE) and one month after KPE using Prechtl's GMA, including motor optimality scores. At 2-3 years, neurodevelopment was assessed using the Bayley Scales of Infant Development, and compared to the Dutch norm population. The predictive value of GMA at infant age for motor skills and cognition at toddler age was determined.
Results:
Neurodevelopment was assessed in 41 BA patients. At toddler age (n = 38, age 29 ± 5 months, 70 % liver transplantation), 13 (39 %) patients scored below-average on motor skills, and 6 (17 %) patients on cognition. Abnormal GMA after KPE predicted both below-average motor skills and cognitive score at toddler age (sensitivity, 91 % and 80 %; specificity 83 % and 67 %; negative predictive value, 94 % and 94 %; and, positive predictive value, 77 % and 33 %, resp.).
Interpretation:
One-third of toddlers with BA show impaired motor skills. GMA post-KPE has a high predictive value to identify infants with BA at risk of neurodevelopmental impairments.
