In this podcast, Dr. Alex Bondoc and Dr. Rae Hanke discuss the intricacies of choledochal cysts. Contributing editor: Dr. Rod Gerardo.
Choledochal Cyst
– Definition: Congenital cystic dilation of the biliary tree
– Epidemiology: Western World (1/100,000), Eastern Asia (1/13000), 3-4x more in females than makes
– Genetics: PKD1 for type 5
– Pathophysiology:
o Embryologic (type 1 and 4): pancreatico biliary duct junction malunion long common channel reflux back of pancreatic enzymes causes changes
– Todani classification
Type I (MCC) | Type II | Type III | Type IV (a/b) | Type V |
Embryologic Etiology |
|
| Embryologic Etiology | Genetic Component |
Fusiform or saccular dilation of CBD | Diverticulum off CBD | Choledochocele of CBD in wall of duodenum. Involves sphincter of Odi | IVa: cysts intra and extra hepatic IVb: cysts extra hepatic only | Inhtrahepatic cysts |
8% Lifetime risk of hepatobiliary malignancy. Even after resection. MCC Cholangiocarcinoma |
|
| 8% Lifetime risk of hepatobiliary malignancy. Even after resection. MCC Cholangiocarcinoma |
|
§ Type 6: dilation of cystic duct—not part of classification scheme
– Presentation:
o Prenatal: on week 20 ultrasounds. Should establish care at a fetal center.
§ Need to follow closely
§ Hepatobiliary disease is associated—cystic biliary atresia must be ruled out
§ If diagnosed prenatally >4.5cm at 20w scan, high symptom burden. Should have earlier intervention
o Younger (infants): incidental on axial/US for other causes
o Children: cholangitis s/s, fevers, jaundice, palpable RUQ mass. Charcot’s triad—very uncommon
§ Giant cysts can perforate and present with biliary ascites
– Differential diagnosis for Child with Jaundice
o Pancreatic biliary tree
o Stone disease
o Choledochal cyst
– Work up:
o Physical exam jaundice/scleral icterus. Palpable abdominal mass
o Labs LFT, indirect and direct bili, CBC (for infection/cholangitis)
o Imaging abd ultrasound, usually followed by CT scan
o Extraneous Testing: do not need liver biopsy for work up in children. But will need in a neonate/infant to find biliary atresia variant
o ERCP/MRCP: If need to know anatomic details get MRCP. If disease into the hilum or intrahepatic, this can be helpful. Will identify variant biliary anatomy also. ERCP decision is based on type of cyst, and imaging findings.
– Treatments
o Non surgical options—none
o Surgical options—
§ If acute (infective) presentation: cool them off, treat underlying process. After recovery, schedule for surgery
§ If incidental presentation: schedule elective operation
§ Goals of surgery: complete excision of the cyst, but limited by type
Type I | Type II | Type III | Type IV (a/b) | Type V |
Remove as much of the duct as possible, even into the pancreas. | Remove diverticulum at neck, repair CBD
Do not leave T tube | ERCP w/ sphincterotomy if simple.
Duodenal wall involvement trans duodenal resection. Remember: Biopsy the mucosal wall of the cyst. | Remove as much of the duct as possible, even into the pancreas.
Type IVb: May require transplant or lobectomy | Resect: if adequate liver remnant Transplant: if diffuse involvement |
o Surgical technique Details for Type I and Type IVa
§ <2yo subcostal incision, >2yo upper midline incision. Mobilize the cyst from the hilar structures. IOC needed to understand where distal cyst occurs to not leave anything behind. Then resect, and close CBD. Reconstruct with hepatico J RNY. 15-20cm from LOT, JJ in 2 layers. Hepatico J w/ interrupted absorbable suture. Close the small bowel mesentery.
– Post op Considerations:
o enteric drainage w/ NGT due to JJ plan to remove NGT at 24hrs
o await ROBF
o Keep drains to follow for biliary leak from HJ until eating regular diet
o Start Choleretic (actigal) and cholangitis ppx (bactrim for 3-6m post op).
– Long term Complications: sbo adhesions, cholangitis, reflux gastritis.
o Need life long follow up due to possibility of malignancy.
Intro and outro tracks are adapted from “I dunno” by grapes, featuring J Lang, Morusque. Artist URL: ccmixter.org/files/grapes/16626
