Hippokratia 2016, 20(1):93
Kavvadas G1, Chamzin A2, Dourakis SP1
12nd Department of Internal Medicine, Athens University Medical School, 2Endoscopic Surgery Unit, Hippokratio Hospital, Athens, Greece
Key words: Choledochal cyst, polycystic kidney disease, magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography
A 73-year-old man with free past medical history presented to the emergency department with 2-weeks duration of obstructive jaundice and no other symptoms. Magnetic resonance cholangiopancreatography (MRCP) (Figure 1) revealed extensive saccular dilatation of the common bile duct up to 6.5 cm and coexistence of saccular dilatations of the central intrahepatic bile ducts. That image was compatible with type IV-A congenital choledochal cyst. Magnetic resonance imaging of the upper abdomen revealed the coexistence of multiple scattered, cortical, exophytic and peripelvic renal cysts, the largest of which measured about five cm. Furthermore, a transient liver elastography was performed which demonstrated a liver stiffness of 40.3 kPa and a gastroscopy that detected three esophageal varices. Cirrhosis was attributed to the asymptomatic chronic cholestasis. No cause of chronic liver disease (HBV, HCV, etc) was founded. The patient was not diabetic and had not abdominal fat. An endoscopic retrograde cholangiopancreatography was performed, which confirmed the MRCP findings and was followed by endoscopic sphincterotomy with the use of balloon catheter. Surgical treatment is required in most cases due to high malignant transformation rate. We have chosen solely endoscopic sphincterotomy because he was already cirrhotic with portal hypertension and had a high surgical risk. The patient and his family totally agreed to this management after been informed. The patient was discharged from the hospital clinically improved with gradually decreasing bilirubin concentration.
Figure 1: Saccular dilatation of common bile duct and coexistence of saccular dilatations of the central intrahepatic bile ducts on magnetic resonance cholangiopancreatography (MRCP) imaging.
Choledochal cyst is a rare entity that represents about 1% of benign biliary disease1. In 60% of patients, the diagnosis is made in the first decade of life2, and the most common symptoms are abdominal pain, fever, vomiting and jaundice3. The paradox of this case is that the patient reached the age of 73 years without any symptoms ever, even though he had a large choledochal cyst in combination with the development of liver cirrhosis probably associated with polycystic kidney disease.
Conflict of interest
1. Jabłońska B. Biliary cysts: etiology, diagnosis and management. World J Gastroenterol. 2012; 18: 4801-4810.
2. Söreide K, Körner H, Havnen J, Söreide JA. Bile duct cysts in adults. Br J Surg. 2004; 91: 1538-1548.
3. Zheng X, Gu W, Xia H, Huang X, Liang B, Yang T, et al. Surgical treatment of type IV-A choledochal cyst in a single institution: children vs. adults. J Pediatr Surg. 2013; 48: 2061-2066.