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Intellectual Disability in Pediatric Liver Transplantation

August 2, 2016 7:00 am

A recent study (A Wightman et al. JPGN 2016; 62: 808-12) describes the prevalence of intellectual disability in a retrospective cohort from 2008-2013. The importance and the discomfort of the topic is referenced in the introduction:

“A 2005 survey of pediatric liver transplant programs (n=12) found that 33% of centers reported that cognitive disability was “always or usually” considered in their decision. No pediatric centers, however, considered mild or moderate cognitive disability alone (IQ 35-70) to be a relative or absolute contraindication to transplantation.”
The 2005 AASLD guideline states that “children with mental retardation pose significant logistical and ethical challenges” but does not comment on whether this is a contraindication.

This retrospective study of children who underwent an isolated liver transplant from 2008-2013 (n=254).

Key findings:

15% of all first pediatric liver transplantation recipients have intellectual disability
Graft function and patient survival were similar among those with and without intellectual disabilities. Metabolic disease, as the indication for liver transplantation, was the etiology more commonly among children with intellectual disability.

This study had numerous limitations. Due to these limitations (eg. selection bias, lack of a standardized mechanism of measuring intellectual disability), it is likely that intellectual disability occurs more commonly than in 15% of pediatric liver transplant recipients. In fact, a previous study showed 42% of recipients required special education and 29% had IQ <85 (Cognitive Outcomes after Liver Transplantation | gutsandgrowth).

Related neurocognitive recommendations from the 2014 AASLD Pediatric Liver Transplantation Guidelines:

28. “Neurocognitive testing should be performed in children awaiting LT to identify areas warranting early intervention to minimize later cognitive diffi- culties (2-B).”
75, 76, and 92. LT is contraindicated with Alper’s disease, multiorgan mitochondrial disease, and Niemann-Pick type C.
In nearly 40 pages of recommendations, this guideline offers very little guidance on this topic.