Cystic duct cysts are a rare congenital anomaly. While the other bile duct cysts (choledochus and the intrahepatic bile ducts) are classified according to the classification described by Tadoni, there is no classification method described by the cystic duct cysts, although it is claimed that the cystic duct cysts may constitute a new “Type 6” category. Only a limited number of patients with cystic duct cysts have been reported in the literature. The diagnosis is usually made in the neonatal period or during childhood. The clinical symptoms are nonspecific and usually include pain in the right upper quadrant and jaundice. The condition may also present with biliary colic, cholangitis, cholelithiasis, or pancreatitis. In our case, the abdominal ultrasonography (US) performed on a 6-year-old female patient who presented with pain in the right upper quadrant pointed out an anechoic cyst at the neck of the gall bladder. Based on the magnetic resonance cholangiopancreatography (MRCP) results, a cystic dilatation was diagnosed in the cystic duct. The aim of this case-report presentation was to discuss the US and MRCP findings of the cystic dilatation of cystic duct, which is an extremely rare condition, in the light of the literature information.
Cysts of the bile duct are rarely observed congenital anomalies characterised by cystic dilatations of the intrahepatic and/or extrahepatic bile ducts. Cystic duct cysts are even more seldom than the other types of choledochal cysts [
A 6-year-old female patient presented to the Paediatrics Clinic with abdominal pain. Her routine blood count and biochemical tests were normal. The abdominal ultrasonography (USG) has noted an approximately 16 × 29 mm anechoic cyst adjacent to the gall bladder (Figure
Ultrasound image of the cystic duct cyst: (a) the cystic duct cyst at the anterior aspect of the portal vein (white arrow); (b) the cystic duct cyst (white arrow) with the anteriorly located gall bladder (asterisk).
Cystic dilatation of the cystic duct was determined in coronal MRCP and T2-weighted MR images. ((a), (b)) Cystic dilatation is observed in the cystic duct in MRCP images (arrow). The intrahepatic bile ducts and the choledochus are normal. The connection of the dilated cystic duct segment with both gall bladder (c) and choledochus (d) could be determined (arrows).
Choledochal cysts are rarely observed congenital anomalies characterized by cystic dilatations of the intrahepatic and/or extrahepatic bile ducts. It is a very rare condition and its incidence during the neonatal period has been reported between 1/100.000 and 1/150.000 in the western societies. The frequency is observed to increase in Asian societies and especially in Japan [
Only a limited number of cystic duct cysts have been reported in the literature and a classification method for these cysts is yet to be developed. Choledochal cysts were initially classified by Alonso-Lej et al. in 1959 [
The diagnosis is usually made during the neonatal period or the childhood; only 20–30% of the patients are diagnosed in adulthood [
The primary treatment in case of choledochal cysts is surgery including cyst enterostomy, cyst excision, or hepatic jejunostomy. Surgeries conducted during early childhood are more successful because inflammation or malignant changes may accompany the cysts in adulthood. Surgery should not be delayed because it may pave the way to malignancies. Any concurrent anomalies of the pancreaticobiliary junction increase the risk of malignancy due to the long-term exposure of the epithelium to the pancreatic enzymes. Therefore, the cyst should be completely excised. In 50% of the adults with bile duct cysts, the diagnosis is made based on symptoms including jaundice, cholecystitis, cholangitis, or pancreatitis as well as during the surgery to excise malignancies [
In conclusion, cystic duct cysts are a rare congenital anomaly usually diagnosed in the neonatal period or during childhood. While they may remain asymptomatic, they may also cause symptoms including nonspecific abdominal pain and jaundice as well as serious symptoms such as biliary colic, cholangitis, cholelithiasis, or pancreatitis. The differential diagnosis of the condition includes dilatations due to gall stones, postoperative scars, or pancreatic pseudocysts. Because MRCP is an easily performed method that can detect any concurrent pathologies and does not involve ionizing radiation, we are for the opinion that it is useful in the diagnosis of cystic duct cysts. The treatment of this condition is surgical and surgery should not be delayed because it may pave the way to malignancies.
The authors declare that there is no conflict of interests regarding the publication of this paper.