Choledochal cysts

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CholedochalCysts : 

CholedochalCysts And Imaging the Biliary Tree Karl Langberg

Choledochal Cysts : 

Choledochal Cysts Rare congenital abnormality of the biliary tree Dilation of a portion of the bile duct Affects 1 in 13,000 to 1 in 2,000,000 Present in girls 3 times more than boys Causes 2%-5% of extrahepatic neonatal jaundice 20% become symptomatic in adulthood

Presentations & Associations : 

Presentations & Associations Intermittent RUQ pain Distention/ inflammation of a portion of the biliary tree Jaundice Direct Hyperbilirubinemia Abdominal Mass Dilated extrahepatic bile duct Increased risk for Cholangiocarcinoma Consequence of cholestasis Classic Triad

Work Up & Diagnosis : 

Work Up & Diagnosis Choledochal cysts usually diagnosed by Imaging Screening is done with ultrasound Follow up with Endoscopic Retrograde Cholangiopancreatography (ERCP) Magnetic Resonance Cholangiopancreatography (MRCP) is an increasingly common, accurate (96%), diagnostic modality.

ERCP : 

ERCP Endoscopic technique Has both diagnostic and theraputic roles

ERCP : 

ERCP Injects contrast into the biliary tree to visualize the anatomy in real time with fluoroscopy

Slide 9: 

A) D) C) B)

Benefits and Risks : 

Benefits and Risks Interventions by ERCP can avoid the need for surgery Complications occur in 5%-7% of patients. They include: Pancreatitis (4%), Hemorrhage (1%), Cholangitis (1%), Perforation (0.5%) and Death (0.1%) ERCP’s use as a diagnostic measure is decreasing due to a less invasive way to image the biliary tree

MRCP : 

MRCP Noninvasive MR imaging of the biliary tree

Quick MR basics : 

Quick MR basics Hydrogen nuclei have a magnetic orientation MR uses a giant magnet to line all of the H atoms in the same orientation Rf pulses return the atoms back to equilibrium, releasing energy into the surrounding tissues The rate of this energy dissipation is different for different tissues

Study Types : 

Study Types Different remagnetization properties of the H atoms in certain tissues can be analyzed with two main study types This allows for different pictures of the same tissue to tell certain properties of that tissue.

T1 : 

T1 Fluid appears dark Fat appears light CSF Grey mater White mater

T2 : 

T2 Fluid appears light Fat appears dark CSF White mater Grey mater

MRCP : 

MRCP Heavily T2 weighted study Easily images the relatively stationary bile that fills the biliary tree Provides high quality picture of patient’s anatomy Has no theraputic role but is non invasive

Classification of Choledochal Cysts : 

Classification of Choledochal Cysts There are 5 subtypes of choledochal cysts. Classified based on morphology of the dilatation

Normal Anatomy : 

Normal Anatomy ERCP Endoscope MRCP Intrahepatic Bile Duct Gall Bladder Extrahepatic Bile Duct Sphincter of Odi Hilar confluence

Case Presentation : 

Case Presentation 61 year old Asian woman CC: RUQ pain, nausea, alternating diarrhea and constipation HPI: Sx occurred intermittantly 4-5 years ago. Choledochal cyst diagnosed by ultrasound. Sx have become constant. PMH: Gall bladder cancer (has been removed), breast cancer with bone metastasis, Heart disease, sleep apnea, diabetes, HTN, 5 cm Ascending Aortic Aneurism.

Continued : 

Continued An ERCP was performed

˜ : 

˜

ERCP : 

ERCP Diffuse dilatation of common bile duct Distended up to the left hepatic duct No strictures No contrast dye filling defects

MRI with MRCP : 

MRI with MRCP Performed to confirm diagnosis

MRI with MRCP : 

MRI with MRCP

MRI with MRCP : 

MRI with MRCP

MRI with MRCP : 

MRI with MRCP

MRI with MRCP : 

MRI with MRCP

MRI with MRCP : 

MRI with MRCP

MRI with MRCP : 

MRI with MRCP

Diagnosis- TypeI Choledochal cyst : 

Diagnosis- TypeI Choledochal cyst Most common classification 80%-90% of Choledochal Cysts

Slide 33: 

X X X X X X X X X

Treatment : 

Treatment Surgical excision and reanastomosis Roux-en-Y Hepaticojejunostomy Must weight pros and cons of surgery vscholangiocarcinoma risk and symptoms Drain cyst of biliary sludge

Other Types : 

Other Types Type 2 Diverticulum of CBD Type 3 Cyst of the intraduodenal duct Choledococele

Other Types : 

Other Types Type 4 Both intra and extrahepatic involvement Type 5 Intrahepatic involvement only Caroli’s disease

Shout Outs : 

Shout Outs Special Thanks to Dr. Diego Martin Dr. Bobby Kalb Dr. Jimmy Costello

Refrences : 

Refrences Sokol Ronald J, Narkewicz Michael R, "Chapter 21. Liver & Pancreas" (Chapter). Hay WW, Jr., Levin MJ, Sondheimer JM, Deterding RR: CURRENT Diagnosis & Treatment: Pediatrics, 19e: http://www.accessmedicine.com/content.aspx?aID=3404306. MorteleKoenraad J, "Chapter 9. State-of-the Art Imaging of the Gastrointestinal System" (Chapter). Greenberger NJ, Blumberg RS, Burakoff R: CURRENT Diagnosis & Treatment: Gastroenterology, Hepatology, & Endoscopy: http://www.accessmedicine.com/content.aspx?aID=6201030. Carr-Locke David L, "Chapter 35. Endoscopic Retrograde Cholangiopancreatography (ERCP)" (Chapter). Greenberger NJ, Blumberg RS, Burakoff R: CURRENT Diagnosis & Treatment: Gastroenterology, Hepatology, & Endoscopy: http://www.accessmedicine.com/content.aspx?aID=6203876. Greenberger Norton J, Paumgartner Gustav, "Chapter 305. Diseases of the Gallbladder and Bile Ducts" (Chapter). Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, Loscalzo J: Harrison's Principles of Internal Medicine, 17e: http://www.accessmedicine.com/content.aspx?aID=2874111. Chen Michael Y, Bradbury Michelle S, "Chapter 1. Scope of Diagnostic Imaging" (Chapter). Chen MYM, Pope TL, Jr., Ott DJ: Basic Radiology: http://www.accessmedicine.com/content.aspx?aID=2270000. ShethKetan R, Bonnor Ricardo M, Pappas Theodore N, "Chapter 45. Biliary Tract" (Chapter). Zinner MJ, Ashley SW: Maingot's Abdominal Operations, 11th Edition: http://www.accesssurgery.com/content.aspx?aID=130494 Wikipedia: Choledochal cysts, for some images