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[Extrahepatic portal hypertension. Azygos-portal disconnection]
Insights
Extrahepatic portal hypertension in children, a condition often caused by portal vein obstruction, can lead to severe esophagogastric varices. Surgical azygos-portal disconnection effectively reduced varices and prevented recurrence in a pediatric patient.
Area of Science:
- Pediatric Gastroenterology
- Vascular Surgery
- Hepatology
Background:
- Extrahepatic portal hypertension is the most common cause of portal hypertension in children, often resulting from portal vein obstruction.
- Esophagogastric varices and upper gastrointestinal bleeding are serious complications of portal hypertension.
Observation:
- A pediatric patient with a progressive course of extrahepatic portal hypertension presented with significant esophagogastric varices and recurrent upper gastrointestinal hemorrhage.
- The patient underwent an azygos-portal disconnection with esophageal transection and gastric fundoplication at age 9.
Findings:
- Postoperative endoscopy revealed a marked reduction in esophageal varices and complete disappearance of gastric varices.
- Eleven months after surgery, the patient remained asymptomatic with no endoscopic evidence of variceal recurrence.
Implications:
- Azygos-portal disconnection is a viable surgical option for managing pediatric extrahepatic portal hypertension and its complications.
- This surgical approach can effectively control variceal bleeding and improve patient outcomes, preventing recurrence.
Abstract:
The partial or complete obstruction of portal vein and or one of its branches is the most frequent cause of portal hypertension in children. A patient with extrahepatic portal hypertension and progressive course is presented, with great development of esophagogastric varices and multiple episodes of upper gastrointestinal hemorrhage. At the age of 9 years he was submitted to an azygos-portal disconnection by abdominal route, with manual transection of the esophagus, covered and protected by a gastric fundoplication. Postoperative endoscopic control demonstrated significant reduction in the size of esophageal varices and disappearance of gastric varices. Eleven months after surgery the patient was asymptomatic and a new endoscopic control showed no recurrence of varices.