A recent study from the Netherlands (LH Rodijk et al. J Pediatr 2020; 217: 118-24) which included 46 children provides data on the suboptimal neurodevelopmental outcomes of children with biliary atresia (BA). This cohort did not exclude children born prematurely or those with a history of intracranial hemorrhage; the children had undergone Kasai portoenterostomy (KPE) between 2002-2012 and had a median age of 11 years.
- 36 of 46 (78%) had undergone liver transplantation
- Median age at time of KPE was 60 days
- 12 (25%) received special education (vs. 2.4% in ‘normal’ population)
- Motor outcomes were affected with up to half scoring low on motor skills
- Total IQ was 91 (compared with 100 in norms)
- There were no significant differences in the cognitive outcomes of the patients with their native livers compared to those who had undergone liver transplantation (*small sample size)
- Detrimental affects of cholestasis
- Major surgery/anesthesia may result in impaired neurodevelopment
My take: This study documents a fairly high rate of neurodevelopmental problems in children with BA. The information we need now –how to mitigate this.
VL NG et al. J Pediatr 2018; 196: 139-47. This study with 148 children examined the neurodevelopmental outomes of young children with biliary atresia (ChiLDRen Study). Key finding: Children with their native livers were at increased risk for neurodevelopmental delays at 12 and 24 months. This risk was more than 4-fold increased among those with unsuccessful Kasai procedure.
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