Life-Threatening Esophageal Variceal Hemorrhage in a 7-Year-Old Boy with Massive Portal Vein Enlargement Due to Congenital Arterioportal Fistula

BACKGROUND Arterioportal fistulas (APFs) are abnormal connections between the arterial and portal venous systems, leading to portal hypertension (PH) and symptoms such as gastrointestinal bleeding, splenomegaly, and hepatic pain. Symptoms typically appear by the age of 2 years in about 75% of cases. CASE REPORT A 7-year-old boy with an asymptomatic APF developed life-threatening complications following a Clostridium difficile infection. He initially had chronic diarrhea, abdominal pain, weight loss, and anorexia for 3 weeks, despite normal liver enzymes. After antibiotic and antifungal treatment, his condition worsened, resulting in severe anemia and hemorrhagic shock due to variceal bleeding. Further evaluations revealed significant PH secondary to the APF. Intensive care involved blood transfusions, fluid resuscitation, and high-frequency ventilation. Emergency embolization successfully reduced PH and controlled bleeding. After stabilization, the patient was transferred for further care. A week later, a color Doppler ultrasound (CD-US) detected a thrombus in the left portal vein, which decreased by the 19-day follow-up. Spleen size reduction indicated decreased portal pressure. The presence and reduction of the thrombus and alleviation of PH symptoms indicate therapeutic success. Ten months after embolization, the patient remained asymptomatic, with normal liver function and no thrombus on follow-up imaging. CONCLUSIONS Early diagnosis and intervention are crucial in managing congenital APF in children. Severe variceal bleeding triggered by exacerbated PH due to a Clostridium difficile infection demonstrates the complications of APF. Endovascular treatment was highly effective, resulting in significant improvement. The recommended diagnostic approach includes initial computed tomography angiogram and CD-US, followed by digital subtraction angiography with possible intervention, and monitoring with CD-US.