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Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome

W De Jesus-Rojas1, K McBeth1, A Yadav1

  • 1Department of Pediatrics, Division of Pulmonary Medicine, McGovern Medical School at University of Texas Health Science Center, Houston, TX, USA.

Case Reports in Pediatrics
|September 9, 2017
PubMed

Insights

Hepatopulmonary Syndrome (HPS) is a rare complication in children with liver disease, often presenting as unexplained hypoxemia. Early recognition of HPS in pediatric patients with Caroli Syndrome is crucial for timely management and potential liver transplant prioritization.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Pulmonology

Background:

  • Hepatopulmonary Syndrome (HPS) is a known complication of chronic liver disease, predominantly observed in adults.
  • Caroli Syndrome, a rare inherited disorder, involves intrahepatic ductal dilation and liver fibrosis, often leading to portal hypertension.

Observation:

  • Hepatopulmonary Syndrome (HPS) should be considered in the differential diagnosis for children experiencing prolonged, unexplained hypoxemia, particularly those with underlying liver disease.
  • A case report details a 6-year-old girl with Caroli Syndrome and End-Stage Renal Disease who presented with persistent hypoxemia.

Findings:

  • The case highlights the presentation of Hepatopulmonary Syndrome in a pediatric patient with Caroli Syndrome.
  • Persistent hypoxemia in children with liver disease may indicate the presence of HPS.

Implications:

  • Increased awareness of HPS in pediatric populations is essential for accurate diagnosis and management.
  • Identifying HPS can impact liver transplantation waitlist priority for pediatric patients, irrespective of their Pediatric End-Stage Liver Disease (PELD) score.

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