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Hepatic adenomatosis in a 7-year-old child treated earlier with a Fontan procedure
Kadir Babaoglu1, Fatih Köksal Binnetoglu, Ayşen Aydoğan
1Department of Pediatric Cardiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey. babaogluk@yahoo.com
Insights
Patients with Fontan-associated liver disease may develop rare hepatic adenomas. This case highlights the youngest patient diagnosed with hepatic adenomatosis following a Fontan procedure, a unique occurrence.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Surgery
Background:
- Fontan procedure is linked to liver disease due to hemodynamic changes and hypoxia.
- Cardiac hepatopathy commonly presents as congestion, necrosis, or cirrhosis.
- Hepatic adenoma and adenomatosis are uncommon in this patient population.
Observation:
- A 7-year-old girl with Fontan-associated liver disease presented with chronic hepatic changes.
- Magnetic resonance imaging and liver biopsy confirmed hepatic adenomatosis.
- This represents the youngest documented case of hepatocellular adenomatosis post-Fontan.
Findings:
- The patient exhibited hepatic adenomatosis, a rare finding in Fontan-associated liver disease.
- The diagnosis was confirmed through advanced imaging and histopathological analysis.
- This case underscores the potential for unusual hepatic pathologies in Fontan survivors.
Implications:
- This case expands the known spectrum of liver pathologies following the Fontan procedure.
- It suggests the need for vigilant monitoring for rare hepatic conditions in Fontan patients.
- Further research may elucidate the mechanisms linking Fontan circulation to hepatic adenomatosis.
Abstract:
Patients who undergo a Fontan procedure experience some degree of liver disease. Hemodynamic changes such as central venous hypertension, depressed dynamic cardiac output, and late ventricular dysfunction combined with long-standing hypoxia preceding the Fontan procedure all are recognized risk factors for hepatic injury. The histopathologic changes associated with cardiac hepatopathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis. However, hepatic adenoma and hepatic adenomatosis (HA) are not well-known pathologies during the course of cardiac hepatopathy. This study focused on a 7-year-old girl with chronic hepatic changes and HA who had undergone a Fontan procedure. Hepatic adenomatosis was diagnosed on the basis of magnetic resonance imaging (MRI) and MRI-guided liver biopsy. To the best of the authors' knowledge, this case involved the youngest patient with hepatocellular adenomatosis documented in the literature. It was a unique case because the patient experienced HA after a Fontan procedure.
