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, * FetusCORONAL 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

CystConway. 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