Preoperative clinical findings may predict the probability of experiencing neurological complications following a liver transplant in patients with Alagille syndrome (ALGS), among other etiologies, according to an article recently published in Pediatric Transplantation.

“A timely diagnosis with correction of the precipitating cause and symptom-oriented therapy is lifesaving and can avoid long-term residual disability,” the authors wrote.

This retrospective study included 71 patients within a pediatric population who underwent liver transplants. It analyzed clinical characteristics before the surgery and their relation to postoperative complications, with a special emphasis placed on neurological complications.

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Among all participants, almost one-fourth (21.1%) experienced neurological complications in the postoperative period following a liver transplant. The most common manifestation was seizures, present in 5 cases, followed by extrapyramidal movements in 3 patients, diaphragmatic palsy and encephalopathy beyond 96 hours in 2 patients each, and lastly, cerebrovascular accident, peripheral neuropathy, and central pontine myelinolysis in 1 patient each.

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Neurological complications presented between postoperative day 1 and day 58, with a median onset of 8.5 days after surgery. After a median follow-up of 15.5 months, 8 patients had died, including 4 that had exhibited neurological complications. The remaining 11 individuals in the neurological complication group recovered without further neurologic manifestations.

When revising all preoperative features, hepatic encephalopathy, pediatric end-stage liver disease/model for end-stage liver disease (PELD/MELD) score, ventilation patterns, the presence of infections, increased bilirubin levels the day before surgery, and high tacrolimus doses were associated with postoperative neurological complications on univariate analysis. However, after multivariate analysis, only neurological complications appeared to maintain the ability to predict neurological complications after transplantation.

The most common etiology in patients who experienced neurological complications was biliary atresia, accounting for 6 cases, followed by Wilson disease in 5 patients, and finally ALGS and acute chronic liver failure in 2 children each.

One of the patients with ALGS was 14 years old. The indications for transplant were pruritus and portal hypertension, and the patient presented with seizures on postsurgical day 8. Imaging studies contributed to a diagnosis of posterior reversible encephalopathy syndrome. The patient stayed in the intensive care unit for 5 days, with an overall hospital stay of 10 days. At the 13-month check-up, there were no abnormal findings.

The other child with ALGS was 12 years old. The patient underwent a liver transplant due to intractable pruritus and presented on the 20th postoperative day with bilateral weakness of the lower limbs, with left predominance. The magnetic resonance imaging scan was normal, so the patient was diagnosed with peripheral neuropathy. The patient stayed in the intensive care unit for 5 days, with a total hospital stay of 19 days. No pathologic findings were reported at the 21-month follow-up.


Menon J, Shanmugam N, Rammohan A, Hakeem A, Reddy MS, Rela M. Neurological complications in pediatric liver transplant recipients. Pediatr Transplant. Published online August 12, 2022. doi:10.1111/petr.14376