Biliary Cysts: A Review and Simplified Classification Scheme

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Key points

  • Various biliary cystic conditions that have been considered together really represent markedly different disorders in terms of embryologic and anatomic considerations, and they also carry different risks of both neoplastic and nonneoplastic complications.

  • Modern imaging techniques, as well as advances in operative technique and our ability to manage patients in the perioperative period, have likely altered and simplified the necessary classification schemes and treatment algorithms.

Classification schemes

Under most circumstances, when one is writing an article such as this, one would begin with a description of the anatomy and physiology of the organs in question. It is a time-honored tradition and usually makes sense. In the case of biliary cysts or congenital choledochal malformations it is probably not so useful, perhaps even counterproductive. By definition, all of the cystic abnormalities either are or derive from anatomic variations. Furthermore, some of the defects may be the direct

Cause

There are a few theories about the cause of choledochal cysts. Agreement on them is far from unanimous. The fact that the anatomic and physiologic aberrations exist more so in some of the subtypes than in others leads to the increased confusion on the topic.

The main theme in most of the theories is one of an altered pressure-compliance situation. Essentially, something happens that makes the biliary structure more distensible, therefore more compliant, and this may or may not be associated with

Risk of malignancy

Irwin and Morrison17 first reported the occurrence of malignancy and stones within a biliary cyst in 1944. Flanigan in 197718 reported a series of patients with biliary carcinomas, in whom slightly more than half had malignancy within the cyst itself (Fig. 8), and the others were distributed throughout the biliary system.18 This finding led many to believe that there is a field defect associated with the presence of biliary cysts. I recommend that this assertion be taken with a large pinch of

Type I cysts

Type I biliary cysts, or extrahepatic biliary cysts, are uncommon but not unheard of in the general population. They are the most common of the biliary cysts. Their incidence is estimated at 1:100,000 to 1:150,000 in Western countries and higher in Japan, at 1:1,000.22 The knowledge of their presence dates back to Vater in 1723.23 Douglas in 185224 suggested the congenital nature of the cyst. The clinical presentation of patients with type I biliary cysts may include abdominal pain, jaundice,

Type II cysts

Type II cysts, or biliary diverticula, are rare. They are so rare that, in my opinion, it would be best to discontinue including them in the discussion of biliary cysts. The differential diagnosis includes duplication of the gallbladder. Intersphincteric diverticula may represent a herniation of bile duct mucosa and, therefore, be false diverticula akin to sigmoid diverticula, whereas the more proximal diverticula are believed to be congenital true diverticula. The only published cholangiograms

Type III cysts

Type III cysts, or choledochoceles, are located within the duodenal wall. The term choledochocele was coined by Wheeler in 1940.36 It is believed to be the second least common of the biliary cysts, although I think that we should reserve judgment on that. The differential diagnosis includes duodenal duplications and other cystic abnormalities of adjacent anatomic structures. Further confusing the topic is that some investigators include cystic abnormalities with duodenal mucosa37 as well as

Type IV cysts

Type IV biliary cysts are classified separately, because multiple cysts are present, at least one of which must be extrahepatic. If the multiple cysts are extrahepatic and intrahepatic, they are considered type IVa cysts, and if the multiple cysts are all extrahepatic, they are considered type IVb cysts. This is probably an unnecessary classification, because it adds little to help us. In the category of the type IVb cysts, everything that can be said about type I cysts also applies without

Type V cysts

Caroli disease is also known as communicating ectasia of the intrahepatic bile ducts. It is a rare autosomal-recessive disorder described by Caroli and others in 1958.41 As mentioned earlier, it is a result of embryologic ductal plate malformations that occur during remodeling. The resultant defect can cause fibrosing and scarring of bile ducts large or small. When the scarring is limited to the smaller duct, the effect may be negligible, but when the scarring involves the larger ducts, the

A simplified approach

I submit to you, dear reader, that from this discussion, we have enough information to generate a simpler approach to biliary cystic disease than is currently used. In no way do I wish to denigrate the work of all those who have toiled before us. After all, it was their work that allowed us to study these disorders and learn what we have. Now, it is time to make use of that effort. I am not the first nor will I likely be the last to suggest an alternative classification scheme, but I provide

Summary

There is much known, and there is much unknown, about biliary cystic disease. Several classification schemes have been developed and modified over the last 70 years or so. What seems to be true is that the various biliary cystic conditions that have been considered together represent markedly different disorders in terms of embryologic and anatomic considerations, and they also carry different risks of both neoplastic and nonneoplastic complications. Modern imaging techniques, as well as

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