Choledochal cysts are classified by the portion of the bile duct affected, according to specialists at the University Center for Fetal Medicine. Type I, fusiform dilation of the common bile duct, accounts for up to 90 percent of all choledochal cysts and makes up the majority of cysts diagnosed prenatally. Choledochal cysts in infants are rare but are diagnosed predominantly in females. Associated anomalies are rare with choledochal cysts.
Prenatal care consists of serial ultrasounds to assess maternal and fetal well-being. Delivery at a tertiary care center is advised to provide immediate and complete postnatal evaluation by a specialized team experienced in the management and treatment of both choledochal cysts and biliary atresia. Abdominal ultrasound should be performed. Postnatal magnetic resonance cholangiopancreatography (MRCP) may be helpful by providing a noninvasive and detailed 3D image of the entire biliary tree.
Once the diagnosis of a choledochal cyst has been confirmed, timing of the surgical repair depends on gestational age, size, clinical condition and presence of symptoms.
Prognosis for patients after surgical therapy for type I, II or III choledochal cyst is excellent. The long-term complications of cirrhosis, portal hypertension and malignant transformation are reduced by complete cyst resection and reconstruction.
The majority of patients with type IV cysts will have resolution of their dilated intrahepatic ducts after surgical correction and enjoy an excellent long-term outcome. In patients with Caroli’s disease (type V), intrahepatic dilatations do not resolve. This leads to recurrent cholangitis, fibrosis, portal hypertension and hepatic failure. Recently, liver transplantation has been successful in the treatment of these patients.