| Choledochal cysts | |
|---|---|
| Other names | Bile duct cyst |
 | |
| Different types of choledochal cysts | |
| Specialty | Medical genetics  |
Choledochal cysts (a.k.a.bile duct cyst) arecongenital conditions involvingcystic dilatation ofbile ducts.[1] They are uncommon in western countries[2] but not as rare in East Asian nations like Japan and China.
Most patients have symptoms in the first year of life. It is rare for symptoms to be undetected until adulthood, and usually adults have associated complications. The classic triad of intermittent abdominal pain,jaundice, and aright upper quadrantabdominal mass is found only in minority of patients.[citation needed]
In infants, choledochal cysts usually lead to obstruction of the bile ducts and retention of bile. This leads to jaundice and anenlarged liver. If the obstruction is not relieved, permanent damage may occur to the liver - scarring andcirrhosis - with the signs ofportal hypertension (obstruction to the flow of blood through the liver) andascites (fluid accumulation in the abdomen). There is an increased risk of cancer in the wall of the cyst.[citation needed]
In older individuals, choledochal cysts are more likely to cause abdominal pain and intermittent episodes of jaundice and occasionally cholangitis (inflammation within the bile ducts caused by the spread of bacteria from the intestine into the bile ducts).Inflammation of the pancreas also may occur. The cause of these complications may be related to either abnormal flow of bile within the ducts or the presence of gallstones.[citation needed]
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.
Choledochal cysts are treated by surgical excision of the cyst with the formation of aroux-en-Y anastomosis hepaticojejunostomy/ choledochojejunostomy to thebiliary duct. Future complications includecholangitis and a 2% risk of malignancy, which may develop in any part of the biliary tree. A recent article published in the Journal of Surgery suggested that choledochal cysts could also be treated with single-incision laparoscopic hepaticojejunostomy with comparable results and less scarring. In cases of saccular type of cyst, excision and placement of T-shaped tube is done.[citation needed] The first peroral endoscopic resection of a choledochal cyst ( typ lll, choledochocele ) was performed by Deyhle in 1973. ( Deutsche medizinische Wochenschrift 99, 71 - 72 especially 83 - 83, Januar 1974
Currently, there is no accepted indication for fetal intervention in the management of prenatally suspected choledochal cysts.[6][7]