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Rare Case of Budd-Chiari Syndrome in a Young Child: A Diagnostic Conundrum
Yumna Timsaal1, Syed Hasan Ali1, Farheen Malik1
1Internal Medicine, Dow University of Health Sciences, Karachi, PAK.
Insights
Budd-Chiari syndrome (BCS), a rare liver vascular disorder, involves hepatic vein obstruction. This case highlights a pediatric patient diagnosed via CT scan, with liver transplant as a final treatment option.
Area of Science:
- Hepatology
- Vascular Medicine
- Pediatric Gastroenterology
Background:
- Budd-Chiari syndrome (BCS) is an uncommon hepatic vascular disorder characterized by impaired venous outflow from the liver.
- Obstruction typically affects hepatic veins and the inferior vena cava, leading to liver damage.
Observation:
- A 2.5-year-old male child presented with abdominal distension, fever, and watery diarrhea.
- Physical examination revealed anemia and palmar erythema.
- Abdominal computed tomography (CT) scan demonstrated characteristic findings of BCS with caudate lobe hypertrophy.
Findings:
- Empirical antibiotic treatment (cefotaxime, metronidazole, amikacin) was initiated.
- Blood and urine culture reports were negative.
- Characteristic imaging findings confirmed Budd-Chiari syndrome in the pediatric patient.
Implications:
- This case underscores the importance of considering BCS in pediatric patients with suggestive symptoms.
- Prompt diagnostic imaging, such as CT scan, is vital for identifying BCS.
- Liver transplantation may be a necessary last resort for managing advanced or refractory BCS.
Abstract:
Budd-Chiari syndrome (BCS) is an uncommon vascular disorder in which venous thrombosis prevents the venous outflow of the liver. The obstruction is primarily at the level of hepatic veins and inferior vena cava. Here, we present a case of a two-and-a-half-year-old male child who presented with complaints of abdominal distension for two months and fever and watery diarrhea for one month. Physical examination showed the patient was anemic with palmar erythema. He was started on an empirical treatment of cefotaxime, metronidazole, and amikacin. Sensitivity and culture reports for blood and urine samples were negative, but abdominal computed tomography (CT) scan showed characteristic findings for BCS with caudate lobe hypertrophy. After the symptomatic treatment of the patient, a liver transplant was suggested as a last resort.
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