Download RUQ Pain in Pregnancy: A Case of a Choledochal Cyst

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
Our Patient: Physical Exam
• 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
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
Left hepatic duct
Right hepatic duct
RUQ ultrasound
www.medison.ru/uzi/img/p401.jpg
Our Patient: RUQ Ultrasound
There is a very large anechoic
structure found inferior to the liver.
PACS, BIDMC
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?
CORONAL SSFSE (HASTE) MRI
PACS, BIDMC
SAGITTAL SSFSE (HASTE) MRI
Our Patient: MRI of Abdomen/Pelvis
*
*
*
*
*
*
CORONAL SSFSE (HASTE) MRI
* Liver, * Cystic structure, * Fetus
SAGITTAL SSFSE (HASTE) MRI
PACS, BIDMC
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
Type II
Type III
Fusiform or cystic
dilations of the
extrahepatic biliary tree
Saccular diverticulum
of the extrahepatic
biliary tree
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
Type IVb
Type V
Multiple cysts present,
intra and extrahepatic
(Caroli’s disease)
Multiple cysts
present, extrahepatic
only
Intrahepatic biliary
cysts only
5-10% of choledochal cysts
5-10% of choledochal
cysts
1% 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
Cyst
Cyst
- Neonate born to
19 yo G2P1
- Identified Type I
Choledochal Cyst
in utero
*
- Thought to be
ovarian cyst early
on in pregnancy
RUQ ultrasound
Cyst, * Dilated intrahepatic ducts
Herman and Siegel
Companion Patient #2 – RUQ US
Liver
Cyst
Polypoid
Mass
• Choledochal cyst identified on RUQ US
• Polypoid mass at proximal region of cyst
• Pathology confirmed cholangiocarcinoma
RUQ ultrasound
Lee HK, Park SJ et al
Companion Patient #3 ‐ MRCP
• 28 yo female
Dilated intrahepatic
ducts
Choledochal
cyst
• 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.
Haciyanli et al
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
SAG T1-weighted MRI
* Choledochal Cyst
* Fetus
Conway. Choledochal cyst during pregnancy. Am J Obstet Gynecol 2009.
Companion Patient #5: HIDA scan
HIDA Anterior view
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).
http://www.imagingpathways.health.wa.gov.au/includes/dipmenu/a_chol/image.html
Companion Patient #6: ERCP
• 50 yo male with abnormal liver
function tests and abnormal anechoic
structure found on RUQ US
•ERCP for CBD stent placement
*
ERCP
ERCP
• NB: ERCP is an invasive procedure, seldom used
today for diagnosis of choledochal cysts.
* Choledochal Cyst
PACS, BIDMC
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
Liver
MRCP
Liver
*
*
C+ COR CT
* Choledochal Cyst
PACS, BIDMC
Our Patient’s Surgery:
Roux‐en‐Y Hepaticojejunostomy
Percutaneous
transhepatic stents
Hepaticojejunostomy
Duodenum
Roux En “Y”
Jejunum
Jejunojejunostomy
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 *
C+ COR CT
PACS, BIDMC
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
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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