1. + CASE CONFERENCE Sept. 14, 2011 Vincent Patrick Tiu Uy, MD

2. 5 year old male presents to the emergency department with ABDOMINAL PAIN

3. HISTORY OF PRESENT ILLNESS 1 week PTC Intermittent episodes of vague abdominal pain localized at the periumbilical area. The pain was mild and the child can tolerate. Episodes of NBNB emesis x 3 Denies fever, weakness Poor appetite, otherwise hydrated Few hours PTC Pain became continuous, diffused, and severe Graded 10/10 on FACES. Several episodes of vomiting this time bilious No appetite; child refuses to drink anything Child became sicker Abdominal Pain Vomiting x 3-5

4. + History Review of Systems Unremarkable. Most mentioned in the HPI Past Medical History Denies any medical problems, no previous hospitalizations, no previous surgeries, NKDA Family History Denies any medical/surgical problems among immediate family members Social History Child lives in an apartment with parents and siblings. No pets at home. No recent travel. Denies any introduction of new foods. No smokers at home. Child feels safe at home.

5. + Physical Examination General AppearanceAlert and awake, appears to be in moderate pain and prefers to lie down Vital SignsT 97.4 HR 108 RR 22 BP 94/58 SO2 100% RA Head, Eyes, Ears, Nose Throat, Neck NCAT, pinkish conjunctivae, anicteric sclerae, nasal septum midline, TM’s intact, dry oral mucosa, non-hyperemic OP, supple neck, no CLAD Chest and CardiovascularCTAB, +S1/S2, no murmurs Abdominal ExamFlat abdomen, hypoactive bowel sounds, (+) direct tenderness with guarding on 4 quadrants, no palpable masses, (-) rebound, (-) Rovsing’s sign, (-) Psoas sign, (-) Obturator sign, (-) Murphy’s sign *Liver span not mentioned GU/Rectal*Was not performed at the ED ExtremitiesNo edema, no cyanosis, brisk capillary refill Neurologic ExamNo focal neurologic findings

6. + Differentials?

7. + Management at the ED IV line placement + Fluid bolus and then maintenance Laboratories obtained: CBC, CMP, Amylase and Lipase, Coagulation panel, Blood typing, Urinalysis, Blood culture*, Urine Culture* Serial abdominal exams  still with diffuse tenderness and muscle guarding. Diagnostic Imaging: Ultrasound of the abdomen and pelvis

8. + Laboratories Hgb11.7 g/dL Hct33.5% WBC13.2 10^3/uL N50 L40 M7 E2 Na + 135 mEq/L K+K+ 3.5 mEq/L Cl - 103 mEq/L CO 2 23 mEq/L AST111 IU/L ALT44 IU/L Alk Phos159 IU/L Ca 2+ 9.7 mg/dL Glucose216 mg/dL Amylase366 IU/L Lipase904 IU/L Complete Blood CountChemistries

9. + Ultrasound of the Abdomen and Pelvis

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11. + Eventually… Diagnostic imaging: Contrast CT scan of the Abdomen and Pelvis

12. + CT scan of the abdomen

13. + CT SCAN OF THE ABDOMEN

15. + CYSTIC DISEASE OF THE BILIARY TREE

16. + Biliary Duct Cysts Encompasses both intrahepatic and extrahepatic cysts Incidence: 1:1,000 – 1:1,500 Higher incidence in the Asian population Cholangiocarcinoma (9-28%) is the most feared complication. Approximately 67% of patients will be symptomatic by 10 years of age.

17. + Pathogenesis Multifactorial Abnormal Pancreatic-Biliary Junction (APBJ) Pancreatic duct empties into the CBD by >1 cm proximal to the ampulla of Vater. Reflux of pancreatic secretions  weakness of CBD wall Other congenital causes Poor epithelialization and recanalization of the biliary tree Congenital weakness of the ductal walls

18. + Abnormal Pancreaticobiliary Junction

19. + Clinical Presentation Pathologic jaundice and Acholic stools (representing biliary obstruction) Palpable RUQ mass Hepatomegaly Infants

20. + Clinical Presentation Older Children Intermittent bouts of pancreatitis (subclinical) Intermittent biliary obstruction Palpable mass Jaundice

21. + Differential Diagnosis Acute Pancreatitis Biliary Atresia Cholecystitis, Choledolithiasis, Ascending Cholangitis Biliary duct Obstruction Bile duct tumors

22. + Laboratory Work-up No laboratory work-up is specific May suggest a co-diagnosis Elevated LFTs: Cholangitis, Obstruction Bilirubins: Pathologic Jaundice Amylase and Lipase: Pancreatitis WBC Counts: Cholangitis Electrolytes: Hypochloremic, Hypokalemic Alkalosis in vomiting

23. + Imaging Studies Ultrasound Best initial test Can identify the cyst prenatally (2 nd trimester) CT Scan Delineates the anatomy Presence and extent of intrahepatic involvement Usually enough to confirm the diagnosis and plan further management/surgery MRCP Best non-invasive test Able to detect APBJ Useful in planning further management/surgery

24. + Invasive Testing ERCP & PTC If non-invasive tests fail to delineate the anatomy. Helpful in determining if there is an APBJ Delineates any intrahepatic or extrahepatic strictures or stones

25. +

26. + Type I Cyst Most common (50-85%) Type IA saccular dilatation of majority or almost all of the CBD Type IB saccular dilatation of a limited segment. Type IC fusiform dilatation of majority or almost all of the CBD

27. + Type II Cyst Isolated outpouching or a diverticulum protruding from the wall of the CBD May be penduculated (with an adjoining stalk)

28. + Type III Cyst AKA Choledochocoele Arises from the intraduodenal portion of the CBD Choledochoceles can be lined by duodenal or biliary epithelium. Arise embryologically as duodenal duplications involving the ampulla.

29. + Type IV Cyst Second most common (15- 35%) Type IVA multiple dilatations of both intrahepatic and extrahepatic sites. Type IVB multiple dilatations of extrahepatic sites only.

30. + Type V Cyst Multiple dilatations limited to intrahepatic bile ducts only. Also known as Caroli’s Disease

31. Summary of Todani’s Classification TYPE OF CYSTINVOLVEMENTBUZZWORDS Type I aExtrahepaticLarge, majority of the CBD Type I bExtrahepaticSmall, solitary, saccular, segment of the CBD Type I cExtrahepaticFusiform shape, majority of the CBD Type IIExtrahepaticDiverticular, Outpouching, Penduculated Type IIIExtrahepaticCholedochocoele, Intraduodenal, approximates the PD Type IV aExtrahepatic & Intrahepatic Multiple dilatations Type IV bExtrahepaticMultiple dilatations Type V – Caroli’s Disease IntrahepaticMultiple dilatations

32. + Treatment Surgical Cystoduodenostomy and Cystojejunostomy was a thing of the past as it left the cyst wall behind. Total excision is preferred with hepaticojejunostomy (Roux-en-Y)

33. + Current treatment strategies TypeTypical procedureExtra Procedures Type IComplete excision + Roux-en-Y Type IIDiverticular excision with ductoplasty T-tube placement Type III< 3mm: Endoscopic Sphincterotomy > 3mm: Excision (Transduodenal approach) Reimplantation of pancreatic duct Type IVComplete excision of extrahepatic component + Roux-en-Y Intrahepatic components left untouched Lobar excision for intrahepatic components if with stone, strictures, or hepatic abscess or coalescing in one lobe Type VMedical managementLiver transplant if two lobes are affected.

34. + “Extraordinary” Strategies Lilly Technique For cysts that are adherent to the portal vein Can also be done in older patients with repeated cholangitis and marked pericystic inflammation. Liver Transplantation

35. + Adults Versus Children Acquired History of prior biliary surgery, pancreatitis, cholangitis, early/late post-op complications. Vague symptoms Malignant transformation and fibrosis is more common Long term complications 30% Congenital No prior history Classic triad is more common Abdominal pain Jaundice Abdominal mass Fibrosis is rare Long term complications 9.2% ADULTSCHILDREN

36. + Patient Update Transferred and admitted to CHAM Diagnostic MRCP confirms Type IV A Choledochal cysts + Anomalous Pancreaticobiliary Junction (APBJ) Edematous pancreas Underwent surgery to remove the cyst, relieve the pressure on the pancreas Patient tolerated procedure

37. + Easy Pop Quiz! A 9 day old baby girl presents to you with jaundice. The mom noticed yellowing of the skin and eyeballs since she was 4 days old. A work-up was done which showed total bilirubin levels to be elevated at 15 mg/dL. The direct bilirubin was 20% of the TSB. There were no abnormalities in the physical examination. Mom asks you: “What do you think it is?” A. Alagille Syndrome B. Viral Hepatitis C. Biliary Atresia D. Choledochal Cyst E. Sepsis

38. + An even Easier Pop Quiz! A 10 year old boy was brought to the ED with abdominal pain. There was diffuse tenderness and a palpable mass over the right upper quadrant. USG revealed a fusiform choledochal cyst. MRCP was done. The radiologist reading the study mentions an abnormal pancreatico-biliary junction and quizzes the residents: “With the pancreatic duct joining the CBD X cm. before reaching the duodenum.” What is a possible value for X to justify the reading? A. 1 B. 0.5 C. 0.3 D. 1.2 E. 0.8

39. + Sources Ching Shui Huang, et al. Choledochal Cysts: Differences Between Pediatric and Adult Patients. J. Gastrointestinal Surgery (2010) 14:1105-1110 Irie, H., et. al. Value of MRCP in evaluating Choledochal Cysts American Journal of Roentgenology Medscape References Up-to-date

40. + Thank you! LIVER CYSTIC DUCT GALL BLADDER