OUR CARE
A surprising pediatric complication of Long COVID: Acute liver failure
John Fung, MD, part of the team who performed the liver transplant
As clinicians were wrestling with how to treat the many manifestations of the novel SARS-CoV-2 infection in early summer 2021, acute liver failure wasn’t high on the list of complications, especially in pediatric patients. Although there were reports of the virus causing mild and self-limiting liver injury in adults, there were few reports of COVID-19-related liver injury in children. And there was nothing in the medical literature about COVID-19 causing immune dysregulation leading to acute liver failure.
But when 2-year-old Esmeralda Perez was transferred to the University of Chicago Medicine Comer Children’s Hospital with fever, abdominal pain and jaundice, Ruba K. Azzam, MD, MPH, Director of Pediatric Hepatology and Medical Director of the Pediatric Liver Transplant team, suspected that Long COVID was targeting Esmeralda’s liver. Four months earlier, Esmeralda and her parents had tested positive for SARS-CoV-2 with mild symptoms.
Esmeralda’s liver biopsy showed acute hepatitis with patchy hepatic necrosis. A team of pediatric gastroenterologists and infectious disease physicians performed exhaustive tests to rule out other potential infections — Esmeralda had recently played with goats, sheep and chickens on her grandparents’ farm — that might be causing her liver injury.
Despite receiving maximum medical support for her liver, Esmeralda rapidly developed worsening coagulation problems, changes in her mental status and encephalopathy, which are severe manifestations of a failed liver.
On her tenth day in the pediatric intensive care unit, Esmeralda had a second liver biopsy that showed massive necrosis, and she was emergently listed for a liver transplant. “She had less than seven days to live without a transplant,” said John Fung, MD, PhD, Chief of Transplant Surgery. “Young children with acute liver failure often progress quickly to brain death.”
Two days later, Esmeralda was the recipient of a split-liver transplant, one of only 200 such transplants done in the US each year.
Three teams of transplant surgeons performed the procedure. Fung and co-surgeon John LaMattina, MD, split the liver from a deceased adult; Diego di Sabato, MD, and Talia Baker, MD, transplanted the left lateral segment in Esmeralda; and LaMattina and Rolf Barth, MD, transplanted the other three-quarters of the liver in an adult with cancer that had metastasized to the liver and who had been on the transplant waiting list for three years. Plastic surgeon David Chang, MD, performed the microvascular arterial reconstruction in Esmeralda, a challenging procedure in children under age 5.
“Split-liver transplants, which are typically done electively in stable patients, require that an institution have a combined adult and pediatric transplant program and enough experienced transplant surgeons to do all three procedures simultaneously,” said Fung. “Few institutions can do that.” Esmeralda’s high priority status on the transplant list also made the adult’s transplant possible. “If Esmeralda hadn’t received the offer of a liver, she wouldn’t have received that organ either,” said Fung.
Ruba K. Azzam, MD, MPH
Azzam immediately enlisted a pediatric hematologist, who did a bone-marrow biopsy and confirmed the diagnosis. “We initiated treatment quite rapidly — a heavy regimen of immunosuppression — which prevented Esmeralda from having a bone-marrow transplant,” said Azzam. “More than one year after her transplant, Esmeralda has normal liver function and is doing very well.