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.

Key findings:

• 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)

Potential explanations:

• 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.

Related blog posts:

• Outcomes of successful Biliary Atresia Patients
• Outcomes of Biliary Atresia | gutsandgrowth