IPEG 2024 Round Table Discussion from East Asia - Dr. Shoko Ogawa
With Dr. Shoko Ogawa
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What the experts said
The patient was a 6-year-old girl with no significant medical history who presented with abdominal pain and nausea
Laboratory studies showed jaundice and elevated hepatic transaminase
MRI showed very large and distended extra and intrahepatic bile ducts
The diagnosis was Todani type 4A choledochal cyst
Laparoscopic cholecystostomy for biliary drainage was performed on hospital day 9 due to continued elevated bilirubin
The patient developed cholangitis on day 20-29 with obstructed cholecystic duct observed by fluoroscopy
Percutaneous transhepatic cholangio drainage was placed but cholangitis recurred afterwards
Laparoscopic excision of extrahepatic duct and hepaticojejunostomy was performed on day 54
The inflammatory change around the biliary duct was intensive during the operation
During dissection along the common hepatic duct, the anterior wall was opened
The intrapancreatic duct was dissected bluntly and the distal end was oversewn
There was no significant postoperative complication and the patient did well with no elevated tumor markers or suspicious imaging for years
The patient developed intrahepatic cholangiocarcinoma with hilar lymphatic metastasis 6.5 years after the surgery
The patient underwent left hepatectomy with lymphadenectomy and adjuvant chemotherapy
The carcinoma recurred 4 months after hepatectomy and the patient died 8 months after hepatectomy
Biliary drainage can be beneficial in cases of giant choledochal cysts with jaundice and elevated transaminases before definitive surgery
Literature review of giant choledochal cysts found mostly case reports and no evidence advocating giant size as a risk factor for biliary malignancy
Most audience members would try primary surgery first for large cysts without symptoms rather than drainage
The moderator may choose open surgery over laparoscopic for large cysts with severe cholangitis or pancreatitis as a contraindication to minimally invasive surgery
Experience with giant choledochal cysts up to 15 centimeters in diameter in patients 10-15 years old can be managed laparoscopically
Preoperative drainage is not needed and can lead to complications like cholangitis; proceeding straight to operation is preferred
Laparoscopic surgery is feasible for giant cysts by performing intraoperative decompression through aspiration after introducing trocars
For very large cysts, the lower edge may reach the pelvis level or lower, requiring trocar placement at lower levels to visualize the cyst first
Strategy is to place only one trocar initially, aspirate the cyst for reduction, then place other trocars in comfortable positions for the operation
Current practice is to perform laparoscopic choledochal cyst excision around 3-4 months of age, even in neonates with prenatal diagnosis and large cysts
Strategy for prenatally diagnosed cysts: if liver function tests show no jaundice and normal enzymes after birth, can wait 2-3 months; if jaundice and elevated enzymes present, operate earlier even in neonatal period
In Korea, malignant transformation after choledochal cyst surgery occurs between ages 40-60 with cumulative incidence of approximately 8%
There was no malignant sign in the pathology results of the primary surgery
The recurrence occurred in the intrahepatic duct
Most pediatric surgeons do not choose hepatectomy for primary surgery because it is too extensive
Residual intrahepatic biliary duct dilation remained even with follow-ups after the primary surgery
There were no suspicious image findings by ultrasound or MRI during regular follow-up for 6.5 years before malignancy was detected
Most malignant transformation occurs in patients who had initial operation after 5 years old or older
Malignant transformation is rare if patients had first operation in infancy or at 1-2 years old or less
Close follow-up is especially needed for patients who had initial operation after 5 years old or even 10 years old
ERCP is not used for follow-up in the presented center; follow-up is done by echo and MRI
ERCP follow-up is impossible after hepaticojejunostomy, though possible after hepaticoduodenostomy