Abstract

Background: The coexistence of extrahepatic portal vein thrombosis (EHPVT) and sickle-cell disease (SCD) was documented in a young adult patient who was presented with vague gastrointestinal symptoms, including right upper quadrant abdominal pain associated with fever and jaundice. Diagnosing EHPVT in children can be challenging due to ambiguous presentation and the lack of sufficient literature, particularly in those with SCD. Case Presentation: A five-year-old boy with homozygous sickle-cell (SS) disease presented to the emergency department with subjective fever and abdominal pain. The patient was managed conservatively with intravenous (IV) fluids, antibiotics, and analgesics. After admission, the patient was found to have hypoalbuminemia, anemia, and thrombocytopenia. Abdominal ultrasound showed moderate ascites with bowel loop thickness and preserved vasculature. Later, the boy developed increased abdominal pain. Abdominal computed tomography (CT) with contrast showed hepatic, portal, superior mesenteric and splenic veins thrombosis. Conclusion: Venous thrombosis is rare in SS disease, and its occurrence in this disease raises the possibility of a common pathogenesis and adds another pathology to the causes of abdominal pain in sickle-cell disease.

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