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Objective:
Ectopic varices are relatively rare portosystemic shunts caused by portal hypertension and occur outside of the gastroesophageal region. They exhibit diverse symptoms and account for 5% of variceal bleeding cases. Due to the lack of standardized guidelines, selecting the appropriate treatment can be challenging. The efficacy of transjugular intrahepatic portosystemic shunt (TIPS) for ectopic variceal bleeding remains debated. This systematic review addresses the outcomes of TIPS in ectopic variceal bleeding.

Materials and Methods:
A comprehensive search through PubMed, Scopus, Web of Science, and Embase was conducted until January 16, 2023, using relevant keywords. Case reports and case series with under 10 patients, specifically focusing on TIPS for ectopic variceal management, were included. Exclusion criteria included review articles, trials, observational studies with more than 10 patients, and non-English publications. Two reviewers independently screened titles, abstracts, and full texts. Data extraction covered patient demographics, medical history, diagnostic findings, prior treatments, TIPS details, post-TIPS management, complications, and mortality. The quality assessment followed the Joanna Briggs Institute checklist for case reports.

Results:
This systematic review evaluated 43 studies involving 50 patients with ectopic variceal bleeding undergoing TIPS procedures. Patient demographics showed a mean age of 54.3 years, evenly distributed gender, and two pregnant cases. Alcoholic liver disease (48%) and hepatitis C infection (28%) were common causes of portal hypertension. Ascites and splenomegaly were reported in 24% and 16% of the patients, respectively. Three patients did not have bleeding, of whom one presented with anal pain. One patient had hematuria after cystoplasty, and two had vaginal bleeding. Rectal, oral, and stomal variceal bleeding accounted for 62%, 16%, and 22% of the patients, respectively. Ectopic varices were mainly located in the duodenum (28%) and rectum (26%) regions. Concurrent or prior gastroesophageal varices were seen in 50%. Of patients, 60% required transfusions during hospitalization. Complications affected 40% of the patients, encephalopathy in seven and rebleeding in eleven, followed by worsened liver function, respiratory failure, pneumonia, arrhythmia, thrombocytopenia, edema, and cerebral infarcts. TIPS treatments involved various stent sizes, most commonly 10 mm, and concurrent embolization was performed in 21 patients. Mean follow-up lasted 12 months, and 12 needed further intervention. Mortality after TIPS was 18%, owing to diverse complications.

Conclusion:
Despite complications and a notable mortality rate, favorable outcomes were observed in almost half of the patients with ectopic variceal bleeding managed with TIPS. Further research is warranted to refine strategies and improve patient outcomes.