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Infantile hepatic hemangioendothelioma presenting as early heart failure: report of two cases
Chung-Ching Lu1, Sheung-Fat Ko, Chi-Di Liang
1Department of Pediatrics, Chang Gung Children's Hospital, Kaohsiung, Taiwan, ROC. Chung12025@yahoo.com.tw
Insights
Infantile hepatic hemangioendothelioma can cause severe heart failure in infants. Early treatment with prednisolone or hepatic artery ligation effectively manages this rare condition, preventing fatal outcomes.
Area of Science:
- Pediatric Oncology
- Cardiology
- Hepatology
Background:
- Infantile hepatic hemangioendothelioma (IHH) is a rare liver tumor in infants.
- It can present with life-threatening complications such as respiratory distress and congestive heart failure.
Observation:
- Two infants with IHH experienced significant cardiorespiratory symptoms.
- Serum alpha-fetoprotein (alphaFP) levels varied widely between the two cases.
- One infant had extremely high alphaFP levels (26,343 microg/l), while the other had near-normal levels (18 microg/l).
Findings:
- Prednisolone therapy in one infant and hepatic artery ligation in the other resulted in no residual sequelae after 12 months.
- Both treatment modalities were effective in managing heart failure associated with IHH.
- Early recognition and intervention are crucial for successful management.
Implications:
- Prompt diagnosis and treatment of IHH complicated by heart failure can lead to favorable outcomes.
- Prednisolone and hepatic artery ligation are viable therapeutic options for heart failure in IHH.
- Differentiating IHH from hepatic malignancies is essential for appropriate clinical management.
Abstract:
Hepatic hemangioendothelioma is rare. We report on hepatic hemangioendotheliomas in 2 young infants, with initial manifestations of respiratory distress and congestive heart failure. Serum alpha-fetoprotein (alphaFP) level was as high as 26,343 microg/l at 14 days old in 1 case, but was only 18 microg/l in the other case. The 2 patients were treated with prednisolone and hepatic artery ligation, respectively, with no residual sequelae after 12 months of follow-up. In this article, the clinical courses of these 2 young infants are reviewed, and the management of infantile hepatic hemangioendothelioma complicated with heart failure is discussed. In our experience, early heart failure caused by infantile hepatic hemangioendothelioma can be well controlled, especially with prednisolone therapy or by hepatic artery ligation. Spontaneous regression has been reported. However, without early recognition and therapeutic intervention, progression to decompensated heart failure may lead to death. Furthermore, it is necessary to differentiate infantile hepatic hemangioendothelioma (IHH) from hepatic malignancies.
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