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[Hepatic hemangiomatosis with congestive cardiac failure and development into a cholostatic hepatopathy]
Insights
This case study details a rare infantile hepatic hemangiomatosis presenting with cardiac failure and liver disease. Early medical intervention successfully resolved cardiac issues, followed by a favorable outcome for the liver condition.
Area of Science:
- Pediatric Cardiology
- Hepatology
- Vascular Anomalies
Background:
- Infantile hepatic hemangiomatosis is a rare vascular liver tumor presenting in infancy.
- Severe cases can lead to high-output cardiac failure due to arteriovenous shunting.
- Associated cutaneous hemangiomas are common.
Observation:
- A 3-month-old infant presented with multiple cutaneous hemangiomas, hepatomegaly, and severe cardiac failure.
- Cardiac catheterization revealed extensive arteriovenous (AV) fistulas within the liver.
- The liver demonstrated large vascular spaces and marked enlargement.
Findings:
- Medical management with digitalis, diuretics, and corticosteroids rapidly resolved cardiac failure and AV fistula signs.
- A subsequent diagnosis of severe cholestatic liver disease was confirmed histologically.
- The patient experienced a favorable clinical course with complete resolution of liver disease.
Implications:
- This case highlights the importance of early diagnosis and multidisciplinary management of infantile hepatic hemangiomatosis.
- Aggressive medical therapy can effectively manage the cardiac complications associated with this condition.
- Complete clinical resolution of liver disease is achievable, emphasizing the potential for favorable long-term outcomes.
Abstract:
The authors describe a case of hepatic hemangiomathosis in a three months old infant with a clinical picture of multiple cutaneous hemangioma, epatomegaly and severe cardiac failure. The cardiac catheterization with selective angiography showed extensive communication between the mammary artery and the hepatic circulation with the presence of large bloody areas into the markedly enlarged liver. The medical treatment with digitalis, diuretics, and corticosteroids obtained rapid resolution of the cardiac failure and of the A-V fistula signs. A clinical picture of severe liver cholostatic disease developed few months later which was confirmed by histological examination. The subsequent course has been favorable with complete clinical resolution of the liver disease.