1. Management of Type I Choledocal Cysts Ashrith R Amarnath, MD

2. Case Presentation: CW  16 y/o F p/w acute onset of epigastric pain with nausea & vomiting 3 prior episodes that resolved spontaneously  No significant PMHx  Exam: NAD, afebrile, VSS, no jaundice Abd: soft, ttp in RUQ & epigastric area, ND, +BS, no palpable masses/hernias  Labs CBC/CMP/Amy/Lip within normal limits

3. Case Presentation: CW  Imaging CT A/P: dilated CBD to 6.5cm, no other abnormalities MRCP: Confirmed dilated extrahepatic bile ducts to head of pancreas c/w Type I choledochal cyst

5. Case Presentation: CW  Treatment Open excision of choledocal cyst with Roux-en-Y hepaticojejunostomy & cholecystectomy  Post-op Course Uncomplicated & discharged on POD #4

6. Background  Anatomical description first published in 1723 by Vater & Ezler  Dilation of the extrahepatic or intrahepatic biliary tree, or both  Incidence is 1/13000 – 1/2000000 live births  More prevalent in Asia with 33-50% reported cased from Japan  Female to Male ratio is 4:1  Cholangiocarcinoma occurs in 9-28% of cases

7. Pathophysiology  No unifying etiology  Several theories Abnormal recanalization of the primitive bile duct cords Anomalous junction of the pancreatic & CBD (40-80%)

8. Classification  Five types of cysts Type I (80-90%) Type II Type III (choledochocele) Type IVA Type IVB Type V (Caroli Disease)

9. Classic Example  Presentation Abdominal pain, jaundice, & RUQ mass (< 50%)  Workup RUQ U/S +/- CT MRCP vs ERCP  Treatment Surgical excision of cyst & biliary-enteric reconstruction

10. Complications & Prognosis  Overall morbidity is <10% Cholangitis Stricture Pancreatitis Bile leak Intraabdominal abscess  Prognosis is excellent however still ~2% risk of cholangiocarcinoma

11. Pediatric vs Adult  Presentation Jaundice (71%) vs RUQ Pain (91%)  Imaging U/S in both population (93% vs 72%)  Complication rate Adult (41%) > Peds (6%)

12. Advances in Treatment  Laparoscopic Surgery First done in 1995 by Farello et al Comparable operative times, morbidity, & mortality Shorter hospital stay, decreased pain, & better cosmesis Requires greater technical skill  Robotic Surgery

13. References  Brunicardi, F. Charles, Dana K. Andersen, Timothy R. Billiar, David L. Dunn, John G. Hunter, and Raphael E. Pollock. Schwartz's Principles of Surgery, 8/e (Schwartz's Principles of Surgery). New York: McGraw-Hill Professional, 2004. Print.  Edil, B. H., J. L. Cameron, S. Reddy, Y. Lum, P. A. Lipsett, H. Nathan, T. M. Pawlik, M. A. Choti, C. L. Wolfgang, and R. D. Schulick. "Choledochal Cyst Disease in Children and Adults: A 30-Year Single-Institution Experience." J Am Coll Surg 206.5 (2008): 1000-005. Print.  Greenfield's Surgery Scientific Principles And Practice (with Solutions Package). Philadelphia: Williams & Wilkins, 2005. Print.  Meehan, J. J., S. Elliott, and A. Sandler. "The robotic approach to complex hepatobiliary anomalies in children: preliminary report." J Ped Surg 42 (2007): 2110-114. Print.  Palanivelu, C., M. Rangarajan, R. Parthasarathi, V. Amar, and P. Senthilnathan. "Laparoscopic Management of Choledochal Cysts: Technique and Outcomes - A Retrospective Study of 35 Patients from a Tertiary Center." J Am Coll Surg 206.6 (2008): 839-45. Print.  Townsend, Courtney M., R. Daniel Beauchamp, B. Mark Evers, and Kenneth L. Mattox. Sabiston Textbook of Surgery: Expert Consult Premium Edition: Enhanced Online Features and Print (Sabiston Textbook of Surgery: The Biological Basis of Modern Practicsurgical Practice). Philadelphia: Saunders, 2007. Print.