[Clinical characteristics of regional portal hypertension in children presenting with isolated gastric fundal varices].
This retrospective case series describes eight children with regional portal hypertension presenting as isolated gastric fundal varices, including cases associated with acute leukemia and retroperitoneal neuroblastoma, and reports improvement in seven followed patients after treatment directed primarily at the underlying disease.
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This retrospective case series describes eight children with regional portal hypertension presenting as isolated gastric fundal varices, including cases associated with acute leukemia and retroperitoneal neuroblastoma, and reports improvement in seven followed patients after treatment directed primarily at the underlying disease.
Research significance
The reported observations support an association between treating the underlying pancreatic, splenic, or retroperitoneal disorder and improvement of regional portal hypertension and fundal varices; it may be inferred—but is not established—that early recognition and etiology-directed management could reduce bleeding risk in affected pediatric oncology patients.
Source abstract
Objective: To investigate the clinical features of regional portal hypertension (RPH) in children whose primary presentation was isolated gastric fundal varices. Methods: This case series study reviewed clinical data from 8 children diagnosed with RPH complicated with isolated gastric fundal varices at Beijing Children's Hospital, Capital Medical University from June 2017 to January 2026. Data on demographics, clinical manifestations, laboratory findings, diagnosis, and treatment were extracted and analyzed for etiology, clinical characteristics, and outcomes. Results: Among 8 children there were 6 males and 2 females, with a diagnostic age of 2.2-11.5 years. The underlying etiologies were pancreatic in 6 children (5 with severe pancreatitis complicated by pancreatic pseudocyst, and 1 with pancreatic involvement secondary to acute leukemia), all involving pancreatic body and tail. One child was of splenic origin (disseminated tuberculosis with splenic involvement), and 1 child was retroperitoneal (secondary to retroperitoneal neuroblastoma). Presenting features included abdominal pain (7 children), vomiting (6 children), and gastrointestinal bleeding (2 children, 1 of whom with isolated hematemesis, another 1 with hematemesis and melena). Splenomegaly occurred in 7 children. Ascites observed in 5 children, was attributed to the primary disease rather than to RPH. All 8 children had normal platelets, 3 children presented with leukopenia and anemia, which were attributed to the primary disease and secondary lesions. All 8 children had normal liver function, and coagulation function, but all demonstrated splenic vein abnormalities. All 8 children received treatment targeting at the underlying etiology. Somatostatin was administered to 5 children, and hemostatic agents to the 2 children with gastrointestinal bleeding. No child underwent endoscopic intervention. The follow-up ranged from 2 months to 8 years; 1 child with tuberculosis was lost to follow-up. The remaining 7 children showed symptomatic relief and improvement or complete resolution of gastric fundal varices. Conclusions: Regional portal hypertension often manifests as isolated gastric fundus varices and splenomegaly, and may be accompanied by gastrointestinal bleeding. Pancreatic, splenic, and retroperitoneal etiologies should be vigilanted. Individualized treatment strategies focused on the primary disease are crucial for improving pediatric RPH outcomes.