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CASE CONFERENCE

CASE CONFERENCE. Sept. 14, 2011 Vincent Patrick Tiu Uy, MD. 5 year old male presents to the emergency department with ABDOMINAL PAIN. HISTORY OF PRESENT ILLNESS.

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CASE CONFERENCE

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  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 Intermittent episodes of vague abdominal painlocalized 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 1 week PTC Abdominal Pain Vomiting x 3-5 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 Few hours PTC Emergency Room

  4. History

  5. Physical Examination

  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 Complete Blood Count Chemistries

  9. Ultrasound of the Abdomen and Pelvis

  10. Ultrasound of the Abdomen and Pelvis

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

  12. CT scan of the abdomen

  13. CT SCAN OF THE ABDOMEN

  14. CYSTIC DISEASE OF THE BILIARY TREE

  15. 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.

  16. 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

  17. Abnormal Pancreaticobiliary Junction

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

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

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

  21. 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

  22. Imaging Studies • Ultrasound • Best initial test • Can identify the cyst prenatally (2nd 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

  23. 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

  24. 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

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

  26. 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.

  27. 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.

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

  29. Summary of Todani’s Classification

  30. 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)

  31. Current treatment strategies

  32. “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

  33. Adults Versus Children ADULTS 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%

  34. 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

  35. 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?” • Alagille Syndrome • Viral Hepatitis • Biliary Atresia • Choledochal Cyst • Sepsis

  36. 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? • 1 • 0.5 • 0.3 • 1.2 • 0.8

  37. 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

  38. Thank you! LIVER CYSTIC DUCT GALL BLADDER

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