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Published on: September 13, 2022
Primary choledocholithiasis after cholecystectomy without hyperbilirubinaemia in a child with sickle cell disease
Ashley Inez Thrower1, Annette Roberts2, Joshua P Lee3
1Internal Medicine and Pediatrics, Duke University, Durham, North Carolina, USA.
Insights
This study highlights a rare case of primary choledocholithiasis in a child with sickle cell disease (SCD) post-cholecystectomy. Normal bilirubin levels can mask this serious biliary tract condition in SCD patients.
Area of Science:
- Pediatric Gastroenterology
- Hematology
- Hepatobiliary Medicine
Background:
- Children with sickle cell disease (SCD) are prone to hepatobiliary issues due to chronic hemolysis and sickling.
- Choledocholithiasis in pediatric SCD typically arises from gallstones, but primary stones can occur.
- Prior cholecystectomy and normal bilirubin levels can impede diagnosis of biliary disease in SCD.
Purpose of the Study:
- To report an uncommon instance of primary choledocholithiasis in a pediatric patient with SCD.
- To emphasize the diagnostic challenges posed by normal bilirubin levels and a history of cholecystectomy.
Main Methods:
- Case report detailing clinical presentation, diagnostic workup, and management.
- Review of relevant literature on biliary tract pathology in pediatric sickle cell disease.
Main Results:
- A child with SCD and a history of cholecystectomy presented with abdominal pain and primary choledocholithiasis.
- Diagnostic delays were noted due to normal conjugated bilirubin levels.
Conclusions:
- Primary choledocholithiasis is a rare but possible diagnosis in children with SCD, even after cholecystectomy.
- Healthcare providers must maintain a high index of suspicion for biliary disease in SCD patients with abdominal pain, irrespective of bilirubin levels or prior surgery.
Abstract:
Children with sickle cell disease (SCD) may have hepatobiliary pathology secondary to chronic haemolysis and intrahepatic sickling during vaso-occlusive crisis. Choledocholithiasis in children with SCD is usually secondary to cholelithiasis but, less commonly, can be due to primary choledocholithiasis. A known history of cholecystectomy may lead to a delay in diagnosis of biliary tract pathology in sickle cell patients presenting with abdominal pain. Normal conjugated bilirubin levels may further stray healthcare providers away from considering biliary disease in these patients. Here, we report an unusual case of primary choledocholithiasis in a child with SCD after cholecystectomy with normal conjugated bilirubin at the time of presentation.
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