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The Budd-Chiari syndrome. Treatment by mesenteric-systemic venous shunts
Insights
Surgical management of Budd-Chiari syndrome using mesocaval shunt (MCS) or mesoatrial shunt (MAS) showed mixed results. While some patients achieved long-term patency and liver recovery, others experienced shunt thrombosis and recurrent ascites.
Area of Science:
- Hepatology
- Vascular Surgery
- Gastroenterology
Background:
- Budd-Chiari syndrome is a rare condition characterized by hepatic venous outflow obstruction, often leading to ascites and liver damage.
- Surgical shunting is a potential treatment option for managing portal hypertension and ascites in Budd-Chiari syndrome.
- Understanding the outcomes of different surgical techniques is crucial for patient management.
Purpose of the Study:
- To evaluate the surgical outcomes of mesocaval shunt (MCS) and mesoatrial shunt (MAS) in patients with Budd-Chiari syndrome.
- To assess the long-term patency rates and clinical efficacy of these shunts.
- To investigate factors influencing patient survival and liver function post-surgery.
Main Methods:
- Retrospective analysis of 12 patients with Budd-Chiari syndrome undergoing surgical decompression.
- Procedures included mesocaval shunt (MCS) in 5 patients and mesoatrial shunt (MAS) in 7 patients.
- Diagnosis confirmed by liver biopsy and hepatic vein catheterization; shunt patency assessed via angiography.
Main Results:
- Four hospital deaths and one late death occurred; causes included shunt thrombosis and liver failure.
- Two patients developed recurrent ascites post-surgery due to shunt thrombosis, managed with a LeVeen shunt.
- Five patients (3 MAS, 2 MCS) had patent shunts with good initial outcomes; two required a second MAS for recurrent ascites.
- Three patients with patent shunts showed significant improvement in liver histology.
Conclusions:
- Surgical shunting for Budd-Chiari syndrome offers potential benefits but is associated with significant morbidity and mortality.
- Mesocaval shunt (MCS) and mesoatrial shunt (MAS) have variable success rates, with some patients experiencing shunt thrombosis and recurrent ascites.
- Successful shunting can lead to remarkable liver recovery in select patients, highlighting the importance of careful patient selection and surgical technique.
Abstract:
Twelve patients with the Budd-Chiari syndrome have been managed surgically. Ten of the patients were female, two were male, with a mean age of 40 years. Three of the patients had polycythemia vera, two had pre-existing cirrhosis, one had ingested estrogens, one had an occult tumor, and in four there were no associated factors. Ten patients presented with ascites and two with bleeding esophageal varices. The diagnosis was confirmed in all 12 patients by liver biopsy and hepatic vein catheterization. Inferior vena cavography revealed the abdominal vena cava to be thrombosed in six patients. The superior mesenteric vein was used to decompress the congested liver in all 12 patients. In five patients, a mesocaval shunt (MCS) was performed and in seven patients, a mesoatrial shunt (MAS) was carried out. There were four hospital deaths (two MCS, two MAS). One late death (MAS) occurred from liver failure following shunt thrombosis. Two additional patients (one MCS, one MAS) re-developed ascites immediately following surgery and angiography revealed a thrombosed shunt. Ascites has been controlled with a LeVeen shunt in these two patients, but liver biopsies showed progression to cirrhosis. The remaining five patients (three MAS, two MCS) did well, and angiography revealed patent shunts. Two of these patients, however, re-developed ascites at 4 and 10 months following MAS and required a second MAS. Follow-up ranges from 6 to 68 months. In three of the patients (two MCS, one MAS) with patent shunts, liver biopsy shows a remarkable return toward normal liver architecture and histology.