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Pediatric choledochal cysts: diagnosis and current management
Kevin C Soares1, Seth D Goldstein2, Mounes A Ghaseb3
1Division of Surgical Oncology, Department of Surgery, The Johns Hopkins University School of Medicine, Blalock 688, 600 N. Wolfe Street, Baltimore, MD, 21287, USA.
Insights
Choledochal cysts are rare congenital biliary tract disorders. Management involves surgical excision, with generally excellent outcomes but a persistent risk of malignancy.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Medical Imaging
Background:
- Choledochal cysts (CC) are rare congenital anomalies.
- Anomalous pancreaticobiliary union (APBDU) is a suspected contributing factor.
- CCs are associated with inflammation, hepatitis, cholangitis, and pancreatitis.
Purpose of the Study:
- To review the etiology, classification, diagnosis, and management of choledochal cysts.
- To highlight the association between CC and malignancy.
- To discuss current diagnostic and therapeutic approaches.
Main Methods:
- Review of existing literature on choledochal cysts.
- Discussion of diagnostic modalities including CT, MRI, ultrasound, and MRCP.
- Emphasis on surgical management including cyst excision and reconstruction.
Main Results:
- Todani classification (1977) categorizes CC into five types; Types I and IV are most common and linked to malignancy.
- Diagnosis often occurs in childhood, presenting with nonspecific abdominal pain.
- MRCP has largely replaced PTC and ERCP for diagnosis.
Conclusions:
- Prompt surgical excision is the standard management for choledochal cysts.
- Minimally invasive techniques show promise in pediatric cases.
- While prognosis is good, malignancy risk persists post-excision.
Abstract:
Choledochal cysts are rare congenital disorders first described by Vater and Ezler in 1723. Their exact etiology remains incompletely understood; however, an anomalous pancreaticobiliary union (APBDU) and subsequent reflux of biliary contents into the biliary tree are thought to play a role. Accordingly, APBDU-associated choledochal cyst patients are significantly more likely to have evidence of hepatitis, cholangitis or pancreatitis and pathologically confirmed inflammation. In 1977, Todani and colleagues modified the original Alonso-Lej classification to include five types of CC. Type I and IV are the most common and most likely to be associated with malignancy. The majority of choledochal cysts are diagnosed in childhood. Clinical presentation varies and most often consists of nonspecific abdominal pain. Diagnosis is typically accomplished using multimodality imaging techniques including computed tomography, magnetic resonance imaging, ultrasound and MRCP. The use of diagnostic PTC and ERCP in CC has been largely replaced by MRCP. Appropriate management consists of prompt, complete cyst excision followed by restoration of biliary enteric continuity when necessary. Minimally invasive CC resection in the pediatric population has demonstrated acceptable outcomes. Prognosis is generally excellent; however, malignancy risk remains higher than the general population even after complete surgical excision.