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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

CASE CONFERENCE

Sept. 14, 2011

Vincent Patrick Tiu Uy, MD



HISTORY OF PRESENT ILLNESS ABDOMINAL PAIN

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


History
History ABDOMINAL PAIN


Physical examination
Physical Examination ABDOMINAL PAIN


Differentials
Differentials? ABDOMINAL PAIN


Management at the ed
Management at the ED ABDOMINAL PAIN

  • 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


Laboratories
Laboratories ABDOMINAL PAIN

Complete Blood Count

Chemistries




Eventually
Eventually… ABDOMINAL PAIN

  • Diagnostic imaging: Contrast CT scan of the Abdomen and Pelvis


Ct scan of the abdomen
CT scan of the abdomen ABDOMINAL PAIN


Ct scan of the abdomen1
CT SCAN OF THE ABDOMEN ABDOMINAL PAIN



Biliary duct cysts
Biliary Duct Cysts ABDOMINAL PAIN

  • 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.


Pathogenesis
Pathogenesis ABDOMINAL PAIN

  • 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



Clinical presentation
Clinical Presentation ABDOMINAL PAIN

Infants

  • Pathologic jaundice and Acholic stools (representing biliary obstruction)

  • Palpable RUQ mass

  • Hepatomegaly


Clinical presentation1
Clinical Presentation ABDOMINAL PAIN

  • Older Children

    • Intermittent bouts of pancreatitis (subclinical)

    • Intermittent biliary obstruction

    • Palpable mass

    • Jaundice


Differential diagnosis
Differential Diagnosis ABDOMINAL PAIN

  • Acute Pancreatitis

  • Biliary Atresia

  • Cholecystitis, Choledolithiasis, Ascending Cholangitis

  • Biliary duct Obstruction

  • Bile duct tumors


Laboratory work up
Laboratory Work-up ABDOMINAL PAIN

  • 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


Imaging studies
Imaging Studies ABDOMINAL PAIN

  • 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


Invasive testing
Invasive Testing ABDOMINAL PAIN

  • 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


Type i cyst
Type I Cyst ABDOMINAL PAIN

  • 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


Type ii cyst
Type II Cyst ABDOMINAL PAIN

  • Isolated outpouching or a diverticulum protruding from the wall of the CBD

  • May be penduculated (with an adjoining stalk)


Type iii cyst
Type III Cyst ABDOMINAL PAIN

  • 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.


Type iv cyst
Type IV Cyst ABDOMINAL PAIN

  • Second most common (15-35%)

  • Type IVA multiple dilatations of both intrahepatic and extrahepatic sites.

  • Type IVB multiple dilatations of extrahepatic sites only.


Type v cyst
Type V Cyst ABDOMINAL PAIN

  • Multiple dilatations limited to intrahepatic bile ducts only.

  • Also known as Caroli’s Disease



Treatment
Treatment ABDOMINAL PAIN

  • 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)



Extraordinary strategies
“Extraordinary” Strategies ABDOMINAL PAIN

  • 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


Adults versus children
Adults Versus Children ABDOMINAL PAIN

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%


Patient update
Patient Update ABDOMINAL PAIN

  • 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


Easy pop quiz
Easy Pop Quiz! ABDOMINAL PAIN

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


An even easier pop quiz
An even Easier Pop Quiz! ABDOMINAL PAIN

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


Sources
Sources ABDOMINAL PAIN

  • 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


Thank you
Thank you! ABDOMINAL PAIN

LIVER

CYSTIC DUCT

GALL BLADDER


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