Abstract
Choledochal cysts are rare congenital dilations of the biliary tree that can present with non-specific symptoms such as abdominal pain, jaundice, cholelithiasis and pancreatitis. Although most commonly identified in children, they can be found in the adult population.
The incidence of choledochal cysts in the western population is 1:100,000–1: 150,000 live births. The rate is remarkably higher in Asian populations, with approximately two-thirds of cases occurring in Japan.
Cystic disease of the biliary tree was first described in 1723, and the first classification system for choledochal cysts was proposed by Alonso-Lej et al. in 1959 Contemporary classification was created by Todani et al. in 1977 (Figure 1).
The aetiology of the choledochal cyst remains unclear. The most accepted theory of cyst origin supposes the influence of an abnormal pancreatobiliary junction when the common channel of the pancreatic and bile duct exists. This theory postulates that the exceptionally long common channel allows mixing of the pancreatic and biliary juices, resulting in the activation of pancreatic enzymes, inflammation, and deterioration of the biliary duct wall, leading toits dilatation. The origin of type-V choledochal cysts probably differs from that of the other types of choledochal cysts, and is possibly due to dysfunctional remodelling of the ductal plate during embryogenesis.
The relationship between choledochal cysts and carcinogenesis is important. The choledochal cyst is apremalignant state in which cancer occurs not only more often but also 10–15 years earlier than in the normal population. The overall risk of cancer has been reported to be 10–15%, and it increases with age.
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Corresponding Author
Dr Tanmay Anand Surgical Resident
DOI: https://dx.doi.org/10.18535/jmscr/v6i5.30
