RUQ Pain in Pregnancy: A Case of a Choledochal
Cyst
Jennifer Torpey, Year III
Gillian Lieberman, MD
Harvard Medical School
Beth Israel Deaconess Medical Center
March 2010
Agenda
• Our Patient A.B.
• RUQ Anatomy and Differential Diagnosis of
Acute RUQ pain
• Choledochal
Cysts
• Menu of Tests and Images from Companion
Patients
• Summary and Follow‐up of our Patient
Our Patient: Initial Presentation
• A.B. is a 38 year old G2P1 at 23 weeks who
presented with 2‐3 days of RUQ pain that continued
to worsen
– Pain is dull, feels like pressure, is not worsened or
ameliorated with eating or activity
– No fever, chills, nausea, vomiting, abdominal trauma or
sick contacts, no change in bowel habits
– No loss of fluid, no vaginal bleeding, minimal contractions
q2 min which subsided, + fetal movement
Our Patient: Past Medical History
• Obstetric Hx: SVD x1, no complications
– Benign pre‐natal course for current pregnancy
• Medical Hx: Denies
• Surgical Hx: ? Open removal of
gallbladder/cyst at age 13 in China
• Medications: None
• Allergies: None
• Denies tobacco, alcohol and drug use
• T 98.9, BP 103/68, HR 81, RR 20
• Gen: NAD, mildly uncomfortable
• Abd: Gravid, moderately distended
– marked tenderness to palpation in RUQ and R‐side,
fullness appreciated but unable to palpate borders
due to tenderness
– No rebound or guarding
– Fundus
palpable 1 cm below the umbilicus
• Labs: ALT 6, AST 21, alk
phos
47, Tbili
0.2
– WBC 7.2, Hg 12, Hct
34.6, Plt
271
Our Patient: Physical Exam
Before moving on with the case let’s
review:
1)
The anatomy of important structures in
the right upper quadrant
2)
The differential diagnosis of Acute RUQ
pain
3)
Preferable Imaging Modalities in
Pregnancy
Anatomy of the Biliary
Tree
http://gallstoneflush.com/images/biliary%20tract.JPG
Differential Diagnosis of
Acute RUQ Pain
• Gallbladder Disease:
– Cholecystitis
– Cholangitis
– Choledocholithiasis
• Hepatitis
• Hepatomegaly
• Retroperitoneal
appendicitis
• Malignancy:
– Hepatocellular
carcinoma
– Cholangiocarcinoma
– Liver metastases
– Gastric cancer
– Metastatic cancer
– Lymphoma
Gillian Lieberman, MD. Primary Care Radiology: Radiologic Assessment
of Abdominal Pain. Eradiology.bidmc.harvard.edu
Imaging Modalities in Pregnancy
• Preferable to Avoid Ionizing Radiation
– Plain films, CT, ERCP, nuclear medicine
• Common Tests:
– Ultrasound: sound waves
• Pros: Inexpensive, good for identifying fluid
• Cons: Requires skilled technologist, limited view
– MRI: electromagnetic radio waves
• Pros: use up to 1.5 Tesla, good soft tissue differentiation
• Cons: should not use gadolinium as it crosses the
placenta, expensive
• Special Tests:
– MRCP: special MRI for imaging the bile and pancreatic ducts
Now it is time to review our patient’s
imaging.
The first step: RUQ ultrasound
Let’s look at a normal ultrasound first.
Normal
RUQ Ultrasound
Right hepatic duct
Left hepatic duct
www.medison.ru/uzi/img/p401.jpg
RUQ ultrasound
Our Patient: RUQ Ultrasound
PACS, BIDMC
There is a very large anechoic
structure found inferior to the liver.
Our Patient: RUQ Ultrasound
•
13.3 x 10.8 x 12.7 cm cystic structure with layering echogenic
material,
which most likely represents a markedly distended gallbladder
•
Gallbladder neck and presumed dilated cystic duct are markedly
tortuous, containing multiple echogenic
foci, compatible with gallstones
•
Common bile duct cannot be imaged due to tenderness limiting
examination
•
There is no peripheral intrahepatic
biliary
dilation but evaluation of
the central biliary
tree is limited
• No gallbladder wall thickening or definite pericholecystic
fluid
• Normal hepatopetal
flow is seen in the main portal vein.
To better define this abnormal fluid
collection we should look at our
patient’s MRI …
Our Patient: MRI of Abdomen/Pelvis
What Do You See?
PACS, BIDMC
CORONAL SSFSE (HASTE) MRI
SAGITTAL SSFSE (HASTE) MRI
Our Patient: MRI of Abdomen/Pelvis
PACS, BIDMC
*
*
*
*
*
*
* Liver, * Cystic structure, * Fetus
CORONAL SSFSE (HASTE) MRI
SAGITTAL SSFSE (HASTE) MRI
Our Patient: MRI Findings
• Massively dilated common bile duct which measures up to 11 x
13 cm in transaxial
diameter
• Massive dilation of the central intrahepatic
bile ducts with
numerous filling defects within the ducts consistent with stones• Pancreatic head is seen to be splayed about the distended CBD
• Duodenum is displaced laterally and posteriorly
to the dilated
duct• No definite obstructing stone or mass is identified, but the
distortion of the duodenum and pancreatic anatomy limits
definitive evaluation• Gallbladder is collapsed and displaced anterior to the dilated
CBD• ‐
Intrauterine pregnancy identified, no gross abnormalities
visualized
DIAGNOSIS: Type I or IVb Choledochal Cyst
Choledochal
Cysts: The Basics
• Rare, congenital dilatations of the biliary
tract
– Intrahepatic
and extrahepatic
• Risk Factors:
– Female predominance
– more common in Asia
• Complications:
– Recurrent cholangitis, Choledocholithiasis
– Biliary
stricture, Recurrent acute pancreatitis
– Malignant transformation: 15% risk of developing
cholangiocarcinoma
Todani
Classification
for Choledochal
Cysts
Type I
Fusiform or cystic
dilations of the
extrahepatic biliary tree
Type II
Saccular diverticulum
of the extrahepatic
biliary tree
Type III
Bile duct dilatation
within the duodenal
wall (choledochocele)
>50% of choledochal cysts 5% of choledochal cysts 5% of choledochal cysts
Brunicardi, FC, Andersen DK, Billiar TR, Dunn DL, Hunter JG, Matthews JB, Pollock RE:
Schwartz’s Prinicples of Surgery, 9th Edition: http://www.accessmedicine.com
Todani
Classification ‐
Continued
Type IVa
Multiple cysts present,
intra and extrahepatic
(Caroli’s disease)
Type IVb
Multiple cysts
present, extrahepatic
only
Type V
Intrahepatic biliary
cysts only
5-10% of choledochal cysts 1% of choledochal cysts5-10% of choledochal
cysts
Brunicardi, FC, Andersen DK, Billiar TR, Dunn DL, Hunter JG, Matthews JB, Pollock RE:
Schwartz’s Prinicples of Surgery, 9th Edition: http://www.accessmedicine.com
Imaging to Determine Management of
Choledochal
Cysts
• Type I, II and IV cysts will be visible on RUQ US
– Types III (duodenal) and V (intrahepatic) will not
• HIDA scan to determine continuity with biliary
tract
– Excellent for extrahepatic
cysts, difficult for intrahepatic
cysts
• CT scans and MRI provide better intrahepatic
visualization
– Better for surrounding structures, evaluation of malignancy
• TREATMENT: Surgery
• Cyst excision, Roux‐en‐Y hepaticojejunostomy
• Others: Cholecystectomy, Intrahepatic
cyst resection
Let’s look at some companion patients for
more examples of choledochal
cysts.
Companion Patient #1 – RUQ US
Herman and Siegel
- Neonate born to
19 yo G2P1
- Identified Type I
Choledochal Cyst
in utero
- Thought to be
ovarian cyst early
on in pregnancy
Cyst, * Dilated intrahepatic ducts
Cyst Cyst
*
RUQ ultrasound
Companion Patient #2 – RUQ US
• Choledochal
cyst
identified on RUQ US
• Polypoid
mass at
proximal region of
cyst
• Pathology confirmed
cholangiocarcinoma
Cyst
Polypoid
Mass
Liver
Lee HK, Park SJ et al
RUQ ultrasound
Companion Patient #3 ‐
MRCP
Dilated intrahepatic
ducts
Choledochal
cyst
Haciyanli et al
• 28 yo female
• Recurrent
episodes of RUQ
pain
• MRCP images
best defined the
type and extent of
the choledochal cyst
compared to US and
CT images
• Surgery performed
•Patient doing well
NB: MRCP images do not require contrast as bile serves as a
natural contrast material.
Companion Patient #4 ‐
MRI
• 19 yo
G1P0 at 22 wks
• Presented with RUQ pain
• Found Type I choledochal
cyst filled with stones
• Underwent CCY, Roux‐
en‐Y
hepaticojejunostomy,
and cyst excision
• Healthy baby born at 40
weeks gestation
• MRI used for diagnosis
* Fetus* Choledochal
Cyst
Conway. Choledochal cyst during pregnancy. Am J Obstet Gynecol 2009.
SAG T1-weighted MRI
Companion Patient #5: HIDA scan
http://www.imagingpathways.health.wa.gov.au/includes/dipmenu/a_chol/image.html
This NORMAL HIDA scan shows radiotracer in the liver after 5
minutes (left image) and radiotracer in the gallbladder and duodenum
after 45 minutes (right image).
HIDA Anterior view
Companion Patient #6: ERCP
PACS, BIDMC
*
* Choledochal
Cyst• NB: ERCP is an invasive procedure, seldom used
today for diagnosis of choledochal cysts.
• 50 yo male with abnormal liver
function tests and abnormal anechoic
structure found on RUQ US
•ERCP for CBD stent placement
ERCPERCP
Let’s get back to our patient …
Our Patient: Intermittent History
• Recovered from acute episode of RUQ pain
• Decided to hold off on surgery until
postpartum, if possible
• Healthy baby boy delivered at 37 weeks
• Imaging for surgical planning: CT and MRCP
– Identified Type I vs. IVb
choledochal
cyst
– Planned surgery: cyst excision, roux‐en‐y
hepaticojejunostomy, intrahepatic
stone removal,
liver biopsy
Our Patient: Imaging for Surgical Planning
CT MRCP
* Choledochal
Cyst
Liver
*
Liver
PACS, BIDMC
*
C+ COR CT
Our Patient’s Surgery:
Roux‐en‐Y Hepaticojejunostomy
Jejunum
Duodenum
Jejunojejunostomy
Hepaticojejunostomy
Roux En “Y”
Percutaneous
transhepatic stents
Our Patient ‐
Follow Up
• Recovered slowly from
surgery
• Baby boy is doing well
• Followed closely by
hepatology
and surgery
• Two weeks post‐op CT
– Dilated intrahepatic
ducts *
– Pneumobilia
*
PACS, BIDMC
* *
C+ COR CT
Summary
• Imaging is essential for diagnosis of RUQ pain
and surgical planning
• Imaging modalities should be chosen carefully
in pregnancy to avoid harm to the fetus
– Good choices include ultrasound, MRI and MRCP
• Other RUQ imaging options include: CT, HIDA
scan, ERCP
• Choledochal
cysts are rare but have serious
complications and should be removed
surgically
Acknowledgements
• Dr Gillian Lieberman
– BIDMC Core Radiology Clerkship Director
• Dr Jean‐Marc Gauguet
– BIDMC Radiology Resident and “Big Sib”
• Maria Levantakis
– BIDMC Core Radiology Clerkship Administrator
References
• Conway W., Campos G. and Gagandeep
S. “Choledochal
Cyst During Pregnancy: The patient’s first pregnancy was
complicated by congenital anomaly.”
Images in Obstetrics: AJOG.
May 2009. 200 (5). 588e1‐e2
• Normal RUQ US www.medison.ru/uzi/img/p401.jpg
• Herman T., Siegel MJ. “Neonatal Type I Choledochal
Cyst.”
Journal of Perinatology. 27, 453–454 (1 July 2007)
http://www.nature.com/jp/journal/v27/n7/fig_tab/7211759f1.html
• Haciyanli
M., Genc
H., et al. “An Adult Choledochal
Cyst – the MRCP Findings: Report of a Case.”
Surg
Today (2008)
38:1056–1059
• Wiseman K., Buczkowski
A., et al. “Epidemiology, Presentation, Diagnosis and Outcomes of Choledochal
Cysts in Adults in an
Urban Environment.”
American Journal of Surgery 189 (2005) 527–531.
• Lee HK, Park SJ et al. “Imaging Features of Adult Choledochal
Cysts: a Pictorial Review.”
Korean J Radiol
2009;10:71‐80
• Sokol
Ronald J, Narkewicz
Michael R, "Chapter 21. Liver & Pancreas" (Chapter). Hay WW, Jr., Levin MJ, Sondheimer
JM,
Deterding
RR: CURRENT Diagnosis & Treatment: Pediatrics, 19e: http://www.accessmedicine.com.ezp‐
prod1.hul.harvard.edu/content.aspx?aID=3404306.
• Oddsdottir
Margret, Pham Thai H, Hunter John G, "Chapter 32. Gallbladder and the Extrahepatic
Biliary
System" (Chapter).
Brunicardi
FC, Andersen DK, Billiar
TR, Dunn DL, Hunter JG, Matthews JB, Pollock RE: Schwartz's Principles of Surgery, 9e:
http://www.accessmedicine.com.ezp‐prod1.hul.harvard.edu/content.aspx?aID=5026661.
• Kruskal
J, Levine D, Wilkins‐Haug
L, Barss
V. “Diagnostic Imaging Procedures During Pregnancy”
UpToDate. Sept 2009.
http://utdol.com/online/content/topic.do?topicKey=maternal/2119
• Singham
J, Yakada
EM, Scudamore
CH. “Choledochal
Cysts. Part 2 of 3: Diagnosis.”
Can J Surg, Vol. 52, No. 6, December
2009. 506‐511
• Lieberman, G. “Primary Care Radiology: Radiologic Assessment of Abdominal Pain.”
Primary Care Radiology Module.
Eradiology.bidmc.harvard.edu
• Diagnostic Imaging Pathways. Department of Health: Government of
Western Australia.
http://www.imagingpathways.health.wa.gov.au/includes/dipmenu/a_chol/image.html
RUQ Pain in Pregnancy: A Case of a Choledochal Cyst
Agenda
Our Patient: Initial Presentation
Our Patient: Past Medical History
Our Patient: Physical Exam
Slide Number 6
Anatomy of the Biliary Tree
Differential Diagnosis of �Acute RUQ Pain
Imaging Modalities in Pregnancy
Slide Number 10
Normal RUQ Ultrasound
Our Patient: RUQ Ultrasound
Our Patient: RUQ Ultrasound
Slide Number 14
Our Patient: MRI of Abdomen/Pelvis�What Do You See?
Our Patient: MRI of Abdomen/Pelvis
Our Patient: MRI Findings
Choledochal Cysts: The Basics
Todani Classification �for Choledochal Cysts
Todani Classification - Continued
Imaging to Determine Management of Choledochal Cysts
Slide Number 22
Companion Patient #1 – RUQ US
Companion Patient #2 – RUQ US
Companion Patient #3 - MRCP
Companion Patient #4 - MRI
Companion Patient #5: HIDA scan
Companion Patient #6: ERCP
Slide Number 29
Our Patient: Intermittent History
Our Patient: Imaging for Surgical Planning
Our Patient’s Surgery:�Roux-en-Y Hepaticojejunostomy
Our Patient - Follow Up
Summary
Acknowledgements
References