From the Guidelines
The recommended management for congenital intrahepatic portosystemic shunt typically involves a staged approach, with initial medical management and definitive treatment using endovascular occlusion techniques or surgical ligation, as described in the study by 1. The initial medical management focuses on controlling clinical signs with lactulose and antibiotics like metronidazole to reduce ammonia-producing bacteria. Some key points to consider in the management of congenital intrahepatic portosystemic shunt include:
- Dietary protein restriction and supplementation with zinc acetate may also help manage hepatic encephalopathy, as hyperammonemia can affect cognition and lead to symptoms such as fatigue, mental retardation, and bouts of portosystemic encephalopathy, as noted in the study by 1.
- For definitive treatment, endovascular occlusion techniques are preferred, including coil embolization or vascular plugs to gradually close the shunt, with complete occlusion often achieved in stages over several months to allow the portal system to adapt to increased blood flow and prevent portal hypertension.
- In cases where endovascular techniques aren't feasible, surgical ligation may be considered, with preoperative evaluation of portal vein patency, portal pressure, and determination of the type of portosystemic shunt by angiography being crucial, as mentioned in the study by 1.
- Regular monitoring of liver function, ammonia levels, and clinical signs is essential throughout treatment, with orthotopic liver transplantation being the only treatment option when there is an absent portal vein or with intractable portosystemic encephalopathy, as suggested by 1. The prognosis varies based on shunt complexity, patient age, and presence of other anomalies, but many patients show significant improvement with appropriate management, highlighting the importance of early intervention to prevent progressive liver damage, as implied by the study by 1.
From the Research
Diagnosis and Management of Congenital Intrahepatic Portosystemic Shunt
- Congenital intrahepatic portosystemic shunts are rare vascular malformations that create an abnormal connection between portal and systemic veins, resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system 2.
- The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts 2, 3.
- Diagnosis relies on imaging, and prenatal diagnosis is possible 2, 4.
- Spontaneous closure of the shunt is possible in some anatomic forms during the first year of life 2, 4.
Treatment Options
- Radiologic or surgical closure of the shunt may prevent, resolve, or stabilize complications 2, 3.
- Endovascular closure is the first option for treatment when possible 2.
- Interventional radiology plays a key role in the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the shunt 2.
- Surgery is reserved for patients who are not eligible for radiologic procedure and those requiring liver transplantation 5.
Natural History and Prognosis
- Congenital intrahepatic portosystemic shunt is a benign, self-limiting condition in which no correlation between the size of the shunt and the blood ammonia level to the outcome of the shunt was found 4.
- Shunt closure was observed in all children within a mean of 114.31 ± 115.05 days (median 84) 4.
- None of the children had any hepatic or other sequelae 4.
- The size of the shunt increases with age, and older persons are more prone to the effect of toxic metabolites 6.