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The long-term prognosis of congenital portosystemic venous shunt
Insights
Congenital portosystemic venous shunts (PSVS) are rare but can cause hepatic encephalopathy (HE). Surgical closure may prevent HE in patients with shunts exceeding 60% ratio.
Area of Science:
- Vascular Surgery
- Pediatric Gastroenterology
- Hepatology
Background:
- Congenital portosystemic venous shunts (PSVS) are rare vascular anomalies.
- PSVS can lead to serious complications like hepatic encephalopathy (HE).
- The natural history and surgical indications for PSVS remain poorly defined.
Purpose of the Study:
- To review cases of congenital PSVS in Japan.
- To analyze the relationship between PSVS, shunt ratio, and HE development.
- To evaluate potential indications for surgical intervention.
Main Methods:
- Retrospective review of 51 congenital PSVS cases in Japan.
- Classification of shunts into intrahepatic and extrahepatic types.
- Analysis of patient demographics, clinical presentation, and diagnostic findings.
Main Results:
- 12 patients (23.5%) presented with HE.
- HE frequency increased in patients over 60 years.
- Children with HE had shunt ratios >60%; HE did not occur with shunt ratios <30%.
- Hypergalactosemia was noted in 20/28 (71.4%) patients under 15 years.
- Some intrahepatic shunts showed spontaneous closure.
Conclusions:
- A shunt ratio exceeding 60% may indicate a need for surgical PSVS closure to prevent HE.
- Congenital PSVS management requires careful consideration of shunt characteristics and HE risk.
- Further research is needed to define optimal surgical timing and criteria.
Abstract:
Congenital portosystemic venous shunt (PSVS), considered to be a rare disease, can lead to hepatic encephalopathy (HE). With improvements in diagnostic imaging techniques, the number of infants and children with documented PSVS has increased. The natural course of the disease and indications for surgical closure of the shunt vessel have not been well defined. We reviewed 51 cases of congenital PSVS in Japan; 34 patients had an intrahepatic PSVS, and 17 had an extrahepatic PSVS. There were 12 patients with HE at the time of diagnosis. The frequency of HE increased in subjects over 60 years of age. Children with HE had a shunt ratio exceeding 60%. When the shunt ratio was less than 30%, HE did not occur. Twenty of 28 patients under the age of 15 years had hypergalactosemia at the time of neonatal screening. Part of the congenital intrahepatic PSVS spontaneously closed. Surgical closure of a PSVS may be an approach expected to prevent HE when the shunt ratio exceeds 60%.