Related Experiment Videos
Hepatic haemangiomata: diagnosis and management
Insights
This study details hepatic hemangiomas in children, highlighting their varied presentation and complications. Early intervention, such as hepatic artery ligation for cardiac failure, is crucial for improving outcomes in these rare liver tumors.
Area of Science:
- Pediatric Hepatology
- Vascular Anomalies
- Medical Imaging
Background:
- Hepatic hemangiomas are benign vascular tumors of the liver, presenting a diagnostic and therapeutic challenge in pediatric patients.
- The clinical spectrum of hepatic hemangiomas is broad, ranging from asymptomatic incidental findings to life-threatening conditions.
Observation:
- Five pediatric cases of hepatic hemangioma are presented, with a review of a sixth case, illustrating diverse clinical features and prognoses.
- Infants often present with hepatomegaly and may develop congestive cardiac failure or cutaneous hemangiomata.
- Older children can present with anemia and disseminated intravascular coagulation; imaging reveals characteristic patterns of reduced technetium-99m colloid uptake.
Findings:
- Congestive cardiac failure occurred in 4 out of 5 infants, necessitating intervention.
- Hepatic artery ligation was effective in controlling cardiac failure in 3 children, with long-term follow-up.
- Spontaneous regression was observed in one case, while another responded to irradiation after initial non-response to hepatic artery ligation.
Implications:
- Prompt recognition and management of complications like congestive cardiac failure are vital.
- Hepatic artery ligation is a potentially life-saving intervention for severe cardiac compromise.
- Multidisciplinary approaches considering surgical, radiological, and medical management are essential for optimizing outcomes in pediatric hepatic hemangiomas.
Abstract:
Five cases of hepatic haemangioma are described, and a sixth (previously reported) is reviewed. Clinical features, investigation, and management are described to show the great variability of the complications and prognosis. Five children presented in the first 10 weeks of life with hepatomegaly; 4 developed congestive cardiac failure; 3 had cutaneous haemangiomata. One child presented at age 4 years with hepatomegaly and anaemia, and on investigation had features of chronic disseminated intravascular coagulation. Focal decrease or patchiness in hepatic uptake of technetium-99m colloid, and abnormal intrahepatic circulation was shown in all cases. In 3 children liver biopsy was performed to exclude malignant disease. In one patient there was spontaneous regression of the tumour by age 3 years. In 3 cases hepatic artery ligation was necessary to control congestive cardiac failure which had persisted despite treatment with digoxin, diuretics, and oral corticosteroids, a procedure which was without complications after up to 8 years. One infant with intractable portal hypertension, hepatic vein obstruction, and severe cholestasis died with persisting alimentary haemorrhage and intra-abdominal sepsis. One child aged 4 years showed no immediate response to hepatic artery ligation but the size of her tumour got smaller and the clinical features diminished after irradiation. These tumours cause considerable morbidity and have a high reported mortality. If congestive cardiac failure is not rapidly controlled, hepatic artery ligation should be performed.