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Choledochal cyst associated with polycystic kidney disease: report of a case
T Hasegawa1, M Kim, Y Kitayama
1Department of Surgery, Shirasagi Hospital, Osaka-City, Japan.
Insights
This study details a rare case of type I choledochal cyst in a patient with polycystic kidney disease. The findings suggest pancreaticobiliary maljunction may not cause choledochal cysts in these complex cases.
Area of Science:
- Gastroenterology
- Nephrology
- Medical Genetics
Background:
- Polycystic kidney disease (PKD) is a genetic disorder characterized by numerous cysts in the kidneys.
- Choledochal cysts (CCs) are congenital bile duct dilatations, with Type I being the most common form.
- The association between PKD and CCs is exceptionally rare, prompting investigation into potential etiological links.
Observation:
- A 48-year-old female on hemodialysis for chronic renal failure due to PKD was incidentally found to have a dilated common bile duct.
- Endoscopic retrograde cholangiopancreatography (ERCP) confirmed a spindle-shaped, Type I choledochal cyst without pancreatic duct visualization.
- Surgical resection of the choledochal cyst was performed successfully.
Findings:
- Intraoperative cholangiography revealed no contrast reflux into the pancreatic duct.
- Aspirated bile from the cyst showed no elevated amylase levels.
- These findings indicate that pancreaticobiliary maljunction may not be a contributing factor in this specific case of CC associated with PKD.
Implications:
- The etiology of choledochal cysts in patients with co-existing polycystic kidney disease warrants further investigation.
- Management strategies for choledochal cysts in the context of renal fibropolycystic disease remain controversial.
- This case highlights the importance of considering rare comorbidities in clinical practice and the need for tailored treatment approaches.
Abstract:
We report a very rare case of type I choledochal cyst associated with a polycystic kidney disease. A 48-year-old female had been dependent on hemodialysis for chronic renal failure due to polycystic kidney disease and was incidentally diagnosed to have a dilated common bile duct by an ultrasonography. An endoscopic retrograde cholangiopancreatography showed a spindle-shaped, dilated common bile duct (type I choledochal cyst) without visualization of the pancreatic duct. She underwent a resection of the choledochal cyst. Intraoperative cholangiography showed no reflux of contrast medium into the pancreatic duct. Amylase level of the aspirated bile from the bile duct was not elevated. In the case of choledochal cyst combined with renal fibropolycystic disease, pancreaticobiliary maljunction may not contribute to the etiology of choledochal cyst. In such cases, management of choledochal cyst is still controversial and requires further discussion.