Speaker: 呂佳興 Supervisor: 蔡宏名老師 Dr. 孫明祥醫師 Intern seminar Speaker: 呂佳興 Supervisor: 蔡宏名老師 Dr. 孫明祥醫師

Personal identifies Name: 林xx Age : 6 years old Gender: female Date of admission: 2005/02/04

Present illness Referred to　Dr.林炳文，abdominal echo, MRI, blood test was done. No fever, no jaundice. Intermittent abdominal pain especially after lunch 2005/01/31 2 months ago 4 months ago 1 month ago Referred to Dr.陳肇真 Admitted then. Visited GI LMD

Past history 1. Neonatal jaundice s/p phototherapy 2. No history of surgical operation 3. No other major medical problems 4. No known drug allergy Family history: Not contributory

Physical exam-1 Consciousness: clear Vital sign: stable and within normal range. HEENT: conjunctiva: not pale sclera anicteric light reflex +/+ Neck: supple, no LAP, no JVE

Physical exam-2 Chest: clear breathing sound in both lungs Heart sound: regular, no murmur Abdomen: soft, normal active bowel sound peri-umbilical pain described by patient. Diffuse tenderness when palpated. Extremities: freely movable, no edema

Lab Data item result Normal range GOT 23 5-40 GPT 10 5-55 Bil-T 0.4 0.2-1.4 Bil-D 0.1 <0.4 ALK-P 178 110 GGT 80

Echo on 2005/01/03

What’s your findings

1.Normal liver parenchymal echo pattern without abnormal focal space-occupying lesions is found. 2.The GB is well distended with normal wall thickness. 3.There is a dilated outpouching of extrahepatic duct with some stones. The IHDs are patent. The PV is patent. 4.The pancreas is normal in size and echogenicity. 5.The spleen is normal in size. 6.No abnormal lymph node enlargement is found. 7.Bil. kidneys are unremarkable. 看完ｅｃｈｏ上報告的形容，我們再來看MRI驗証一下

MRI image

MRCP

What’s your finding

Diagnosis Sonography: Clinical information: choledocal cyst IMP:R/o choledochal cyst with some stones.

MRI with and without contrast 1. MR cholangiography showed marked dilated CHD and proximal to middle CBD,about 35mm in caliber. The distal CBD and IHDs are preserved. The pancreatic duct is not dilated. GB is distended with smooth in wall. 2. The liver,spleen and both kidneys are normal in size and shape. 3. The pancreas is normal in size. 4. The stomach is not distended,but no evidence of abnormality. 5. No para-aortic lymph node can be seen. IMP: Choledochal cyst, type I.

Operation note

Pathology report Pathology: choledocal cyst +chronic cholecystitis CBD fluid: amylase: 91500u/L lipase: 479375u/L bil-T/D: 546/0 mg/dL

Post-op care No fever, no jaundice WBC: 8.1 Seg: 72.9 % CRP: 54 Serum biochemistry within normal range. MBD on post-op day three

1.Normal liver parenchymal echo pattern without abnormal focal space-occupying lesions. 2.The GB is absent. 3.The biliary system is patent. The PV is patent. 4.The pancreas is normal in size and echogenicity. 5.The spleen is normal in size. 6.No abnormal lymph node enlargement is found. 7.The Bil. kidneys are normal in size and position. 8.The echogenicity of bil. kidneys is not increased. 9.The bil. renal parenchymal thickness is preserved. 10.No abnormal hyperechoic focus in bil. collecting systems. 11.No abnormal dilatation of the bil. collecting systems. 12.No abnormality over aorta and IVC areas. IMP:s/p cholecystectomy. 孫明祥醫師-

Differential diagnosis 1. obstructive bile duct dilatation 2. hepatic artery aneurysm 3. biloma 4. pseudocysts and cysts of abdomen

Differential diagnosis 1. obstructive bile duct dilatation 1. obstruction site (stone, stenosis) 2. smoothly dilated ducts 3. dilated intra-hepatic ducts 4. markedly elevated alkaline phosphatase.

Differential diagnosis 2. hepatic artery aneurysm 1. hepatic artery origin 2. medical history may hints 3. color doppler to detect blood flow.

Differential diagnosis 3. biloma 1. history 2. location

Differential diagnosis 4. pseudocysts and cysts 1. trace the cyst origin, see if any communicating with the biliary system 2. history 3. stone is uncommon in cysts

Choledocal cyst Dilatation of bile ducts. Old name of biliary cyst Specially referring to extra-hepatic cysts Prevalence: 1/2000,000~~1/1,3000 much higher in Asian countries female: male: 3:1

Choledocal cyst-- Etiology congenital V.S. acquired infectious agents reflux of pancreatic enzymes long common channel genetic factors, and biliary autonomic dysfunction. abnormal pancreaticobiliary junction (APBJ) is present in about 70 percent of patients some may show family history

Stone abnormal pancreaticobiliary junction

dilatation of extra-hepatic duct accounts for 50- 85% Type I dilatation of extra-hepatic duct accounts for 50- 85%

Type II Saccular outpouching of the common bile duct.

Type III Type III is also named choledochocele wide mouth dilation of the common duct at its confluence with the duodenum. Lined with duodenum epithelium Not associated with biliary tract carcinoma

Type IV Second common type 33% Cystic dilatation of both intra and extra hepatic ducts

Type V multiple intrahepatic cysts with no extrahepatic component Termed caroli’s disease when associated with hepatic fibrosis and medullary sponge kidney Caroli’s disease is autosomal recessive. Malignancy 7%

Clinical findings Clinical triad: RUQ mass 30-60% jaundice 80% abdominal pain >90% Gallstones, cholangitis, cholecystitis, pancreatitis, biliary peritonitis. Biliary cirrhosis. abscess Cholangiocarcinoma 0.7% at first 10 years 6.8% at 20 years old 14.3% over age 20 increase even further through life, up to 50%

Image findings 1. dilated bile duct seen from echo, CT, MRI, or cholangiography. 2. abnormal pancreaticobiliary junction may be seen (elongated, dilated) 3. colangiography is the best test for choledocal cysts.

treatment Roux-en-Y hepaticojejunostomy (draining in old days) Type IV may require partial hepatectomy Long term complications anastomose stricture(25%) and it’s consequence intrahepatic stone

references 1. Lawrence W. Way: Way Current Surgery 2. uptodate online database 3. Harrison online 4. Radiographics. 2001;21:895-910 Cystic Focal Liver Lesions in the Adult: Differential CT and MR Imaging Features

Thanks for your attention