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CASE CONFERENCE

Sept. 14, 2011Vincent 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 3Denies fever, weaknessPoor appetite, otherwise hydrated

Few hours PTC

Pain became continuous, diffused, and severeGraded 10/10 on FACES. Several episodes of vomiting this time biliousNo appetite; child refuses to drink anythingChild became sicker

Abdominal PainVomiting x 3-5

+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.

+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

+Differentials?

+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

+Laboratories

Hgb 11.7 g/dL

Hct 33.5%

WBC 13.2 10^3/uL

N 50

L 40

M 7

E 2

Na+ 135 mEq/L

K+ 3.5 mEq/L

Cl- 103 mEq/L

CO2 23 mEq/L

AST 111 IU/L

ALT 44 IU/L

Alk Phos 159 IU/L

Ca2+ 9.7 mg/dL

Glucose 216 mg/dL

Amylase 366 IU/L

Lipase 904 IU/L

Complete Blood Count Chemistries

+Ultrasound of the Abdomen and Pelvis

+Ultrasound of the Abdomen and Pelvis

+Eventually…

Diagnostic imaging: Contrast CT scan of the Abdomen and Pelvis

+CT scan of the abdomen

+CT SCAN OF THE ABDOMEN

+

CYSTIC DISEASE OF THE BILIARY TREE

+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.

+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

+Abnormal Pancreaticobiliary Junction

+Clinical Presentation

Pathologic jaundice and Acholic stools (representing biliary obstruction)

Palpable RUQ mass

Hepatomegaly

Infants

+Clinical Presentation

Older Children Intermittent bouts of pancreatitis (subclinical) Intermittent biliary obstruction Palpable mass Jaundice

+Differential Diagnosis

Acute Pancreatitis

Biliary Atresia

Cholecystitis, Choledolithiasis, Ascending Cholangitis

Biliary duct Obstruction

Bile duct tumors

+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

+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

+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

+

+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

+Type II Cyst

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

May be penduculated (with an adjoining stalk)

+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.

+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.

+Type V Cyst

Multiple dilatations limited to intrahepatic bile ducts only.

Also known as Caroli’s Disease

Summary of Todani’s ClassificationTYPE 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

+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)

+Current treatment strategiesType 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-YIntrahepatic 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.

+“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

+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%

ADULTS CHILDREN

+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

+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

+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

+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

+Thank you!

LIVER

CYSTIC DUCT

GALL BLADDER