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Noncirrhotic portal hypertension: differing patterns of disease in children and adults
Insights
Noncirrhotic portal hypertension (NCPH) presents differently in children and adults. Age stratification is crucial for predicting survival and portal-systemic encephalopathy (PSE) risk after surgery for variceal hemorrhage.
Area of Science:
- Gastroenterology
- Hepatology
- Surgical Gastroenterology
Background:
- Noncirrhotic portal hypertension (NCPH) is a rare condition.
- Operative portal decompression is used for variceal hemorrhage control.
Purpose of the Study:
- To investigate distinct patterns of NCPH in pediatric and adult populations.
- To evaluate the impact of age on outcomes after portal decompression surgery.
Main Methods:
- Retrospective review of 25 patients with histologically verified NCPH.
- Analysis of clinical presentation, portal vein status, and outcomes (mortality, portal-systemic encephalopathy).
- Histological examination of liver biopsy specimens.
Main Results:
- Children (n=14, avg age 8.5) had no operative mortality and low rates of portal-systemic encephalopathy (PSE) (7%).
- Adults (n=11, avg age 56) had 18% operative mortality and high rates of PSE (56%).
- Portal vein occlusion was more common in children (71%) than adults (35%). No consistent histological differences were found between age groups.
Conclusions:
- Age is a significant factor in NCPH, influencing outcomes after portal decompression.
- Age stratification may improve prognostic accuracy for survival and PSE incidence.
- Further research into NCPH pathogenesis and treatment is warranted.
Abstract:
Of 440 patients who underwent operative portal decompression to control variceal hemorrhage, 25 (6%) had histologically verified noncirrhotic portal hypertension (NCPH). A review of these patients identified two distinct patterns of disease. Fourteen children with an average age of 8.5 years were treated with no deaths at operation. Occlusion of the portal vein (PV) was demonstrated preoperatively or intraoperatively in 10 children (71%). Follow-up for 12 to 207 months showed that no children died of a liver-related illness and only one child (7%) developed portal-systemic encephalopathy (PSE). In contrast, 11 adults with an average age of 56 years were treated with an operative mortality rate of 18%. Occlusion of the PV was demonstrated angiographically in four adults (35%). Of nine surviving adults who were followed for 12 to 164 months, five adults (56%) manifested PSE. Intraoperative liver biopsy specimens were analyzed by light microscopy in all cases. With the possible exception of sinusoidal fibrosis and dilatation of intrahepatic portal venous radicles, no histologic discriminators of adult forms versus childhood forms were identified regardless of the status of the PV. We conclude that age stratification in patients with NCPH may provide important prognostic data regarding survival rates and the incidence of PSE after portal-systemic shunting.