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Choledochal malformations: Lessons learnt in Johannesburg
A Grieve1, D Harrison, C Westgarth-Taylor
1Department of Paediatric Surgery, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa. a.grieve@me.com.
Insights
Choledochal malformations (CMs) are rare congenital conditions that can cause jaundice and other complications. Specialized pediatric surgical care is essential for managing CMs and improving long-term patient outcomes.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Congenital Abnormalities
Background:
- Choledochal malformations (CMs) are rare congenital conditions.
- They can manifest from antenatal screening through adulthood.
- Associated pancreaticobiliary malunion is a known predisposing factor.
Purpose of the Study:
- To review the experience with choledochal malformations (CMs).
- To improve patient care for this population at the University of the Witwatersrand.
Main Methods:
- Retrospective record review of patients with CMs.
- Study period: January 2010 to May 2017.
- Included patients managed by the Department of Paediatric Surgery.
Main Results:
- 35 patients underwent surgery for CMs.
- 83% presented with jaundice; median bilirubin: 167 mmol/L.
- Type IV CMs showed trends towards younger age and higher bilirubin/GGT levels.
Conclusions:
- Choledochal malformations (CMs) can cause significant morbidity and malignancy.
- Specialized care is crucial for improving long-term outcomes.
- Further research may elucidate specific management strategies.
Background:
Choledochal malformation (CM) is a well-described and relatively rare condition. CMs may present on antenatal ultrasound screening, through childhood and into adulthood. The aetiology is not well understood but the association with a pancreaticobiliary malunion predisposes to the development of CMs.
Objectives:
To review the experience of CMs in the Department of Paediatric Surgery, University of the Witwatersrand, Johannesburg to improve our patient care for this population.
Methods:
After institutional ethics approval, a retrospective record review was conducted of patients presenting with CM to the Department of Paediatric Surgery, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg. All patients managed between January 2010 and May 2017 were included.
Results:
A total of 35 patients underwent surgery for CMs and 2 patients were excluded from the study owing to incomplete records. Most of our patients (83%) presented with jaundice and a median (interquartile range) bilirubin level of 167 (32 - 234) mmol/L. In our cohort of patients those with type IV lesions presented at a younger age and with higher bilirubin and gamma-glutamyl transferase levels, although this finding was not statistically significant.
Conclusion:
Although uncommon, CMs may lead to significant morbidity and malignancy. Specialised care is necessary to improve longterm outcomes in these patients.
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