Choledochal cysts

Choledochal cysts
Classification and external resources
Different types of choledochal cysts
ICD-10	Q44.4
ICD-9	751.69
DiseasesDB	2527
eMedicine	article/172099 article/366004 article/934267
MeSH	D015529

Choledochal cysts are congenital conditions involving cystic dilatation of bile ducts.^[1] They are uncommon in western countries^[2] but not as rare in East Asian nations like Japan and China.

Signs and symtoms

Most of them present in 1st year of life; adult presentation is rare and usually at this stage is associated with complication . Classic triad of intermittent abdominal pain, jaundice, and a right upper quadrant abdominal mass is found only in minority of patients.

Diagnosis

Direct Hyperbilirubinemia

Types

They were classified into 5 types by Todani in 1977.^[3]

Classification was based on site of the cyst or dilatation. Type I to IV has been subtyped.

• Type I: Most common variety (80-90%) involving saccular or fusiform dilatation of a portion or entire common bile duct (CBD) with normal intrahepatic duct.
• Type II: Isolated diverticulum protruding from the CBD.
• Type III or Choledochocele: Arise from dilatation of duodenal portion of CBD or where pancreatic duct meets.
• Type IVa: Characterized by multiple dilatations of the intrahepatic and extrahepatic biliary tree.
• Type IVb: Multiple dilatations involving only the extrahepatic bile ducts.
• Type V or Caroli's disease: Cystic dilatation of intra hepatic biliary ducts.

Treatment

Choledochal cysts are treated by surgical excision of the cyst with the formation of a roux-en-Y anastomosis to the biliary duct. Future complications include cholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree.

References

1. ^ "choledochal cyst" at Dorland's Medical Dictionary
2. ^ Liu YB, Wang JW, Devkota KR, et al. (2007). "Congenital choledochal cysts in adults: twenty-five-year experience". Chin. Med. J. 120 (16): 1404–7. PMID 17825168. 
3. ^ Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K (1977). "Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst". Am. J. Surg. 134 (2): 263–9. doi:10.1016/0002-9610(77)90359-2. PMID 889044.