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Updated: Aug 5, 2026

Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats
Published on: August 1, 2018
The aetiology, presentation and natural history of extra-hepatic portal venous obstruction
Insights
Extra-hepatic portal vein obstruction frequently causes hypertension in children and adults. While surgery may control severe bleeding, conservative management is often preferred due to higher mortality rates in surgical cases.
Area of Science:
- Gastroenterology
- Pediatric Gastroenterology
- Vascular Surgery
Background:
- Extra-hepatic portal vein obstruction (EHPVO) is a significant cause of portal hypertension, particularly in childhood.
- While often presenting in pediatric patients, a notable proportion of cases manifest in adulthood.
Purpose of the Study:
- To analyze the clinical presentation, outcomes, and management strategies for extra-hepatic portal vein obstruction.
- To compare the efficacy and mortality associated with surgical versus conservative management of variceal bleeding in EHPVO patients.
Main Methods:
- Retrospective analysis of 97 patients diagnosed with extra-hepatic portal vein obstruction.
- Review of patient demographics, clinical presentation (hematemesis, splenomegaly, ascites), precipitating factors, and treatment outcomes.
Main Results:
- 57% of patients presented before age 15, but 43% presented in adulthood. Hematemesis was the most common symptom in both groups.
- Ascites was more prevalent in adults (69%) and associated with increased mortality (p<0.01). Portal-systemic encephalopathy affected 45% of ascites patients.
- Variceal hemorrhage caused 19 deaths; infection caused 5 deaths. Surgical intervention for bleeding had a higher mortality rate than conservative management.
Conclusions:
- Extra-hepatic portal vein obstruction requires careful management, with a significant number of cases presenting in adulthood.
- Conservative management is generally associated with better outcomes than surgical intervention for variceal bleeding in EHPVO.
- Surgery should be reserved for refractory cases where bleeding cannot be controlled by medical means.
Abstract:
Extra-hepatic obstruction of the portal vein is a well known cause of hypertension in childhood and 55 out of the 97 patients (57 per cent) seen with this condition presented before they were 15 years old. However, nearly half our cases (43 per cent) presented in adult life. Haematemesis was the commonest mode of presentation in both the adult and childhood group although splenomegaly was common, especially in the children. The severity and frequency of haematemesis increased during puberty in the children. Twelve children and three adults have now not bled for between five and 30 years, 10 of them ceasing spontaneously and five following surgery. Intra-abdominal infection or septicaemia precipitated the portal venous obstruction in 38 patients (39 per cent), though the exact cause of such obstruction in patients who had no cirrhosis was obscure in about half the cases. Ascites was present in 18/51 (35 per cent) of the children and 24/35 (69 per cent) of the adults. Its presence was associated with an increased mortality (p less than 0.01). Nineteen out of 42 (45 per cent) patients with ascites also had portal-systemic encephalopathy. Twenty-four patients died, nine having presented during childhood, variceal haemorrhage was responsible for death in 19 and infection in five. Sixty-four patients underwent 114 operations for variceal haemorrhage. Mortality was greater in the surgical group compared with those managed conservatively. Surgery is therefore indicated only in the rare case where bleeding cannot be controlled by medical means.
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