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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
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
Abdominal Pain
Vomiting x 3-5
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
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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.
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Physical Examination
General Appearance
Alert and awake, appears to be in moderate
pain and prefers to lie down
Vital Signs
T 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 Cardiovascular
CTAB, +S1/S2, no murmurs
Abdominal Exam
Flat 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
Extremities
No edema, no cyanosis, brisk capillary refill
Neurologic Exam
No focal neurologic findings
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Differentials?
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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
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Laboratories
Complete Blood Count
Chemistries
Hgb
11.7 g/dL
Na+
135 mEq/L
Hct
33.5%
K+
3.5 mEq/L
WBC
13.2 10^3/uL
Cl-
103 mEq/L
N
50
CO2
23 mEq/L
L
40
AST
111 IU/L
M
7
ALT
44 IU/L
E
2
Alk Phos
159 IU/L
Ca2+
9.7 mg/dL
Glucose
216 mg/dL
Amylase
366 IU/L
Lipase
904 IU/L
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Ultrasound of the Abdomen and Pelvis
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Ultrasound of the Abdomen and Pelvis
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Eventually…

Diagnostic imaging: Contrast CT scan of the Abdomen and
Pelvis
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CT scan of the abdomen
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CT SCAN OF THE ABDOMEN
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CYSTIC DISEASE OF THE
BILIARY TREE
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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.
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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
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Abnormal Pancreaticobiliary
Junction
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Clinical Presentation
Infants

Pathologic jaundice and
Acholic stools (representing
biliary obstruction)

Palpable RUQ mass

Hepatomegaly
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Clinical Presentation

Older Children

Intermittent bouts of pancreatitis (subclinical)

Intermittent biliary obstruction

Palpable mass

Jaundice
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Differential Diagnosis

Acute Pancreatitis

Biliary Atresia

Cholecystitis, Choledolithiasis, Ascending Cholangitis

Biliary duct Obstruction

Bile duct tumors
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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
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Imaging Studies

Ultrasound



CT Scan
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


Best initial test
Can identify the cyst prenatally (2nd trimester)
Delineates the anatomy
Presence and extent of intrahepatic involvement
Usually enough to confirm the diagnosis and plan further
management/surgery
MRCP
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

Best non-invasive test
Able to detect APBJ
Useful in planning further management/surgery
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Invasive Testing

ERCP & PTC

If non-invasive tests fail to delineate the anatomy.

Helpful in determining if there is an APBJ
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Delineates any intrahepatic or extrahepatic strictures or stones
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Type I Cyst

Most common (50-85%)

Type IA saccular dilatation of
majority or almost all of the
CBD
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Type IB saccular dilatation of a
limited segment.

Type IC fusiform dilatation of
majority or almost all of the
CBD
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Type II Cyst

Isolated outpouching or a
diverticulum protruding from
the wall of the CBD

May be penduculated (with an
adjoining stalk)
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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.
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Type IV Cyst

Second most common (1535%)

Type IVA multiple dilatations
of both intrahepatic and
extrahepatic sites.

Type IVB multiple dilatations
of extrahepatic sites only.
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Type V Cyst

Multiple dilatations limited to
intrahepatic bile ducts only.

Also known as Caroli’s Disease
Summary of Todani’s Classification
TYPE OF CYST
INVOLVEMENT
BUZZWORDS
Type I a
Extrahepatic
Large, majority of the CBD
Type I b
Extrahepatic
Small, solitary, saccular,
segment of the CBD
Type I c
Extrahepatic
Fusiform shape, majority of the
CBD
Type II
Extrahepatic
Diverticular, Outpouching,
Penduculated
Type III
Extrahepatic
Choledochocoele,
Intraduodenal, approximates
the PD
Type IV a
Extrahepatic &
Intrahepatic
Multiple dilatations
Type IV b
Extrahepatic
Multiple dilatations
Type V – Caroli’s
Disease
Intrahepatic
Multiple dilatations
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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)
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Current treatment strategies
Type
Typical procedure
Extra Procedures
Type I
Complete excision +
Roux-en-Y
Type II
Diverticular excision with
ductoplasty
T-tube placement
Type III
< 3mm: Endoscopic
Sphincterotomy
> 3mm: Excision
(Transduodenal approach)
Reimplantation of
pancreatic duct
Type IV
Complete 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 V
Medical management
Liver transplant if two
lobes are affected.
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“Extraordinary” Strategies


Lilly Technique
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For cysts that are adherent to the portal vein
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Can also be done in older patients with repeated cholangitis and
marked pericystic inflammation.
Liver Transplantation
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Adults Versus Children
ADULTS
CHILDREN

Acquired

Congenital

History of prior biliary
surgery, pancreatitis,
cholangitis, early/late post-op
complications.

No prior history
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Classic triad is more common
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
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Vague symptoms
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Abdominal pain
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Jaundice
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Abdominal mass
Malignant transformation and
fibrosis is more common
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Fibrosis is rare
Long term complications 30%

Long term complications 9.2%
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Patient Update

Transferred and admitted to CHAM
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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
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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
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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
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Sources

Ching Shui Huang, et al. Choledochal Cysts: Differences
Between Pediatric and Adult Patients. J. Gastrointestinal
Surgery (2010) 14:1105-1110
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Irie, H., et. al. Value of MRCP in evaluating Choledochal Cysts
American Journal of Roentgenology
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Medscape References
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Up-to-date
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Thank you!
LIVER
CYSTIC
DUCT
GALL
BLADDER