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Observational Study
. 2025 May;29(3):e70076.
doi: 10.1111/petr.70076.

Absence of Pupillary Reflexes in Pediatric Acute Liver Failure and Neurological Outcome After Liver Transplantation

Kirsten J Schouwstra  1   2 , René Scheenstra  2 , Ruben H de Kleine  3 , Vincent E de Meijer  3 , Sander T H Bontemps  4 , Henkjan J Verkade  2 , Deborah A Sival  1
Affiliations
Observational Study

Absence of Pupillary Reflexes in Pediatric Acute Liver Failure and Neurological Outcome After Liver Transplantation

Kirsten J Schouwstra et al. Pediatr Transplant. 2025 May.

Abstract

Background: Pediatric Acute Liver Failure (PALF) frequently requires liver transplantation (LTx). The neurological condition can deteriorate rapidly, but the difficulty in assessing the (ir)reversibility of neurological symptoms can hamper therapeutic decision-making, including transplantation. We aimed to determine the association between pupillary reflexes (PR), brain stem reflexes (BSR), radiological signs of brain herniation, and subsequent neurological outcome.

Methods: We analyzed a retrospective, observational cohort of PALF patients with severe hepatic encephalopathy (grade III-IV), admitted to our national pediatric liver transplantation center between 1993 and 2023. We subdivided the patients into groups with PR present or PR absent. We compared the two groups for pre-treatment neurological and neuro-radiological parameters and related the findings to neurological outcomes.

Results: Survival rate in patients with PR present was higher compared to patients with PR absent [70% (26/37) and 29% (4/14); resp., p = 0.008]. In the absence of PR, neurological outcome could still be favorable after LTx (n = 3/6). Presence or absence of BSR was not related to the outcome in terms of survival or death. Radiologically proven brain herniation was associated with mortality (6/7) or minimally conscious state (1/7), irrespective of undergoing a LTx or not.

Conclusions: Although absence of PR is associated with a poor prognosis, the neurological outcome can still be favorable after LTx. Radiological signs of brain herniation are strongly associated with mortality or severe neurological outcomes, irrespective of subsequent transplantation. We therefore advocate that absence of PR should be an indication for radiological imaging to assess brain herniation before making major treatment decisions.

Keywords: brain herniation; brain stem reflexes; neurological outcome; pediatric acute liver failure (PALF); pupillary reflexes; radiological imaging.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Flow diagram representing the outcome in children with PALF HE III‐IV. The study group was subdivided based on the presence and absence of pupillary reflexes as indicated in this figure. This flow diagram shows the stratified results for treatment with and without LTx in terms of survival and neurological complications of each group. HE, hepatic encephalopathy; LTx, liver transplantation; PALF, pediatric acute liver failure.
See this image and copyright information in PMC

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