Choledochal cysts

Choledochal cysts
Synonyms	Bile duct cysts
Pronounce	N/A
Specialty	N/A
Symptoms	Abdominal pain, jaundice, palpable mass
Complications	Cholangitis, pancreatitis, biliary cirrhosis, cholangiocarcinoma
Onset	Congenital
Duration	N/A
Types	Type I, II, III, IV, V
Causes	Congenital malformation
Risks	N/A
Diagnosis	Ultrasound, CT scan, MRI, ERCP
Differential diagnosis	N/A
Prevention	N/A
Treatment	Surgical excision
Medication	N/A
Prognosis	Generally good with treatment
Frequency	Rare
Deaths	N/A

Choledochal cysts are congenital anomalies of the bile ducts characterized by cystic dilatation of the biliary tree. These cysts can lead to complications such as cholangitis, pancreatitis, and even cholangiocarcinoma if left untreated.

Classification

Choledochal cysts are classified into five main types based on the Todani classification:

• Type I: Fusiform dilatation of the common bile duct.
• Type II: True diverticulum of the bile duct.
• Type III: Cystic dilatation of the intraduodenal portion of the bile duct (choledochocele).
• Type IV: Multiple cysts, which can be intrahepatic or extrahepatic.
• Type V: Cystic dilatation of the intrahepatic bile ducts, also known as Caroli disease.

Pathophysiology

The exact cause of choledochal cysts is not well understood, but they are believed to result from an abnormality in the pancreaticobiliary junction, leading to reflux of pancreatic enzymes into the bile duct and subsequent inflammation and dilatation.

Clinical Presentation

Patients with choledochal cysts often present in childhood, but symptoms can also appear in adulthood. Common symptoms include:

• Abdominal pain
• Jaundice
• Palpable abdominal mass

Complications such as cholangitis, pancreatitis, and biliary cirrhosis can occur if the condition is not treated.

Diagnosis

Diagnosis of choledochal cysts is typically made using imaging studies:

• Ultrasound: Often the first imaging modality used.
• CT scan: Provides detailed images of the cysts.
• MRI: Particularly MRCP (Magnetic Resonance Cholangiopancreatography) is useful for visualizing the biliary tree.
• ERCP: Endoscopic Retrograde Cholangiopancreatography can be both diagnostic and therapeutic.

Treatment

The treatment of choice for choledochal cysts is surgical excision. This typically involves:

• Complete resection of the cyst
• Reconstruction of the biliary tract, often with a Roux-en-Y hepaticojejunostomy

Early surgical intervention is recommended to prevent complications and malignancy.

Prognosis

With appropriate surgical treatment, the prognosis for patients with choledochal cysts is generally good. However, regular follow-up is necessary to monitor for potential complications.

Also see

• Biliary atresia
• Cholangiocarcinoma
• Pancreatitis
• Cholangitis