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Congenital hepatoportal arteriovenous fistula
N D Heaton1, M Davenport, J Karani
1Department of Surgery, King's College Hospital, London, U.K.
Insights
Congenital hepatoportal arteriovenous fistulas in children can cause severe symptoms. Surgical ligation of the hepatic artery is the preferred treatment, offering better long-term control than embolization.
Area of Science:
- Pediatric Surgery
- Vascular Anomalies
- Gastroenterology
Background:
- Congenital hepatoportal arteriovenous fistulas are rare vascular malformations.
- This study reviews two pediatric cases and five previously reported instances.
- These fistulas can lead to significant complications in infants and children.
Observation:
- Patients presented with failure to thrive, hepatosplenomegaly, ascites, and gastrointestinal bleeding.
- Doppler ultrasonography identified abnormal portal venous flow.
- Angiography confirmed the presence of fistulas and significant portal hypertension (>30 mm Hg).
Findings:
- Surgical resection was unsuccessful in one patient.
- Surgical ligation of the hepatic artery effectively controlled symptoms in both investigated children.
- Embolization was a short-term solution due to rapid collateralization of hepatic arterial supply.
Implications:
- Surgical ligation of the hepatic artery is the treatment of choice for congenital hepatoportal arteriovenous fistulas.
- Early diagnosis and surgical intervention are crucial for managing these complex pediatric vascular anomalies.
- Understanding the pathophysiology aids in developing effective therapeutic strategies for portal hypertension in children.
Background:
Two children with congenital hepatoportal arteriovenous fistulas have been investigated and treated surgically. These cases have been reviewed with five cases previously reported.
Methods:
Two children, 5 months and 14 months of age, presenting with failure to thrive, hepatosplenomegaly, ascites, and recurrent gastrointestinal bleeding with evidence of portal hypertension, were found to have congenital hepatoportal arteriovenous fistulas.
Results:
Doppler ultrasonographic examination was important in identifying abnormal portal venous flow. Angiogram identified the fistulas, confirming the diagnosis. Both patients had significant portal hypertension (pressure more than 30 mm Hg). Surgical resection in one child was unsuccessful, but surgical ligation of the hepatic artery controlled the symptoms in both patients.
Conclusions:
Rapid collateralization of the hepatic arterial blood supply made embolization a short-term therapeutic measure, and surgical ligation of the hepatic artery is the treatment of choice for congenital hepatoportal arteriovenous fistulas.