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Portal hypertension in children. An experience of 33 cases
Insights
This study reviews 33 children with portal hypertension, finding good prognoses for extra-hepatic portal obstruction (EHB) and variable outcomes for intra-hepatic obstruction (IHB) based on hepatic severity and surgical approach. Surgical intervention is key for managing this condition.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Surgical Interventions
Background:
- Portal hypertension in children presents diverse etiologies, including extra-hepatic portal obstruction (EHB) and intra-hepatic obstruction (IHB).
- Understanding the specific causes, such as portal vein blockages in EHB and post-hepatitis cirrhosis or congenital abnormalities in IHB, is crucial for effective management.
- Long-term outcomes and treatment efficacy necessitate detailed review.
Purpose of the Study:
- To review the pathology, treatment, and long-term progress of pediatric patients diagnosed with portal hypertension.
- To compare the outcomes and prognoses between children with extra-hepatic portal obstruction (EHB) and intra-hepatic obstruction (IHB).
- To evaluate the impact of different surgical procedures on survival rates and recurrent bleeding in children with portal hypertension.
Main Methods:
- Retrospective review of 33 pediatric patients with portal hypertension.
- Classification of patients into extra-hepatic portal obstruction (EHB) and intra-hepatic obstruction (IHB) groups based on lesion location.
- Analysis of surgical treatments, patient survival, and incidence of recurrent bleeding following various operative procedures.
Main Results:
- Out of 33 children, 20 had EHB (portal vein block) and 13 had IHB (post-hepatitis cirrhosis or congenital abnormality).
- Surgical treatment was performed in 32 patients, with a generally good prognosis and long survival observed in the EHB group.
- In the IHB group, survival was contingent on hepatic disorder severity, with lieno-renal anastomosis showing the lowest incidence of recurrent bleeding compared to splenectomy alone or with devascularisation.
Conclusions:
- Extra-hepatic portal obstruction (EHB) in children generally carries a favorable prognosis with surgical intervention.
- Outcomes for intra-hepatic obstruction (IHB) are significantly influenced by the underlying hepatic condition and the chosen surgical approach.
- The selection of surgical procedure, particularly lieno-renal anastomosis, plays a critical role in minimizing recurrent bleeding and improving long-term survival in pediatric portal hypertension.
Abstract:
The pathology, treatment and progress of 33 children with portal hypertension are reviewed. There were 20 patients with extra-hepatic portal obstruction (EHB) and 13 with intra-hepatic obstruction (IHB). The lesion in all the EHB was a block in the portal vein: in IHB it was a post-hepatitis cirrhosis in two cases and in the others a congenital abnormality. Treatment was surgical in 32 patients. The prognosis in EHB is good and long survival after operation was the rule. In IHB the survival depended upon the type and severity of the hepatic disorder and the incidence of recurrent bleeding. The frequency of recurrent bleeding was found to vary with the operation performed being greatest after splenectomy alone or with devascularisation, and least after lieno-renal anastomosis. The follow-up ranged from one year to more than 28 years.
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