Liver Transplantation in 3 Cholestatic Infants With History of COVID Exposure

TL;DR

Three infants exposed to SARS-CoV-2 perinatally developed severe liver disease and required transplants, possibly linked to biliary atresia and early viral exposure.

Contribution

This case series highlights a potential link between perinatal SARS-CoV-2 exposure and severe liver disease in infants with atypical biliary atresia.

Findings

Three infants with perinatal SARS-CoV-2 exposure developed liver failure requiring transplantation.

Histologic evidence suggested biliary atresia with atypical features possibly exacerbated by SARS-CoV-2.

Infants showed low GGT cholestasis and extrahepatic biliary obstruction despite mild or no COVID symptoms.

Abstract

The COVID-19 pandemic presents several challenges during pregnancy including thromboembolic complications, direct placental infection, transplacental transmission, and systemic hyperinflammatory state. The liver is the second most commonly affected organ in SARS-CoV-2 infection after the lungs. Mechanisms of liver injury in COVID-19 patients can include: direct viral cytopathic effect, worsening of underlying liver disease, cytokine storm, hypoxic ischemic injury, and cholangiopathy leading to persistent marked cholestasis. Here we describe 3 infants at Texas Children’s Hospital with perinatal SARS-CoV-2 exposure with persistent cholestasis and histologic evidence of extrahepatic biliary obstruction suggesting underlying biliary atresia (BA) with some atypical features possibly exacerbated by SARS-CoV-2 infection. All 3 patients described in this case series developed liver failure in the setting of low GGT cholestasis, and all 3 required liver transplantation within the first year of life. Though post-COVID cholangiopathy is described in adults in the literature, none of the infants in our series had moderate or severe COVID infection but still progressed to advanced liver disease. Instead it is very likely that the patients in our series had underlying BA with some atypical features, with the commonality of having been exposed perinatally to SARS-CoV-2 Though further studies are needed to determine causality, our case series raises the question of if the timing of exposure/infection plays a role in prognosis.

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