|ECR 2018 / C-2422|
|Beyond the typical biliary system diseases: Unusual conditions of the biliary tract|
Findings and procedure details
A 43-year-old female presents with right upper abdominal pain for the past 2 months. The patient underwent abdominal US and MRCP, Fig. 1. What are the main findings? What is your differential diagnosis?
Recurrent pyogenic cholangitis, also known as oriental cholangiohepatitis, is characterized by multiple episodes of cholangitis with the formation of intrahepatic pigmented stones.
The etiology is poorly understood but is commonly attributed to parasitic infection by Clonorchis sinensis in endemic areas such as East Asia, or bacterial infection.
The clinical presentation consists of abdominal pain, fever and jaundice.
Key imaging findings:
- On US: bile duct dilatation with the presence of intraluminal echogenic stones is commonly seen, preferably in the left lobe of the liver.
- CT and MRI show disproportionate dilatation of extrahepatic and central intrahepatic bile ducts with multiple low T2 filling defects from stones.
- In some instances complications such as hepatic abscess (20%) and cholangiocarcinoma (6%) may occur.
The management usually involves a multidisciplinary approach including endoscopic, surgical and interventional radiology therapy.
A 46-year-old male presents with right upper abdominal pain for the past 3 days and abnormal liver function tests. Patient underwent abdominal US and MR cholangiography, Fig. 4. What are the main findings? What is your differential diagnosis?
Answer: Fig. 5.
Mirizzi syndrome (MS) is a form of obstructive jaundice secondary to stone(s) impacted in the neck of the gallbladder or the cystic duct causing narrowing of the common hepatic duct.
It is a rare complication of gallstones in 0.1-0.7% of the cases. Patients with MS have a 25% risk of gallbladder cancer.
Patients usually present with symptoms of cholecystitis or choledocholithiasis.
Two types of MS are commonly recognized:
- Type I representing simple obstruction of the common hepatic duct (CHD).
- Type II in which erosion of the wall of the CHD results in formation of a cholecysto-choledochal fistula.
Key imaging findings:
US, CT and MR/MRCP are routinely used, the latter having the highest sensitivity for MS.
- An impacted stone in the gallbladder neck or junction of the CHD and cystic duct is commonly seen, with upstream biliary dilatation. Inflammatory changes of the gallbladder are commonly associated, Fig. 6.
ERCP with stent placement may be a temporary option prior to surgery, Fig. 7. Definitive treatment is surgical with cholecystectomy or partial cholecystectomy. In type II MS, the remnant of the gallbladder is used to close a cholecysto-choledochal fistula.
A 35-year-old male with history of chronic hepatitis C presents with severe right upper abdominal pain, nausea and mild hematemesis for the past 3 days. Patient underwent abdominal US, CT and MR, Fig. 8 and Fig. 9 . What are the main findings? What is your differential diagnosis?
Hemorrhagic cholecystitis is a rare complication of acute cholecystitis (8-12% of cases) with high mortality rate.
It commonly occurs due traumatic, coagulopathy, malignancy or iatrogenic causes.
Hemorrhage is caused by mucosa infarction and erosion secondary to gallbladder wall inflammation.
Clinical presentation may mimic an uncomplicated acute cholecystitis. Hemobilia may rarely manifest as hematemesis or melena.
Key imaging findings, Fig. 12:
- On US: Distended gallbladder with focal wall thickening and irregularity, intraluminal membranes and/or avascular nonmobile echogenic intraluminal mass.
CT findings include high attenuation material within the gallbladder lumen with possible fluid-fluid level. Active contrast extravasation, a finding indicating active bleeding may be seen on the arterial phase.
MR demonstrates hemorrhagic component within the gallbladder lumen characterized by hyperintense signal on T1WI and variable signal on T2WI (depending on oxygenation state of hemoglobin).
Urgent cholecystectomy is the treatment of choice to prevent complications such as perforation.
A 62-year-old female with history of gallstones presents to the emergency department with right upper abdominal pain, mild jaundice, nausea and vomiting for the past 2 days. Patient underwent abdominal US and CT of the abdomen, Fig. 13. What are the main findings? What is your differential diagnosis?
Answer: Fig. 14
Xanthogranulomatous cholecystitis is a rare form of chronic cholecystitis characterized by intramural accumulation of lipid-laden macrophages, also known as xanthogranulomas.
It occurs as a sequela of rupture of Rokintansky-Aschoff sinuses or small ulceration in the mucosa, followed by extravasation of inspissated bile and mucin into the wall of the gallbladder causing an inflammatory reaction and the formation of intramural nodules.
Predominantly affects elderly women (>60 years old) and they usually present with signs and symptoms of cholecystitis. Complications occur in up to one third of the patients and include gallbladder perforation, abscess formation and fistula formation.
Key imaging findings, Fig. 15:
- Diffuse or focal gallbladder wall thickening (3-25mm), with or without definition of the wall margin is the main hallmark.
- On US, the visualization of intramural hypoechoic nodules corresponds to small intramural abscesses or foci of xanthogranulomatous inflammation. Other findings include pericholecystic fluid or intraluminal stones.
- CT demonstrates multiple hypoattenuating intramural nodules and bands.
- MR demonstrates multiple T2 hyperintense and T1 hypointense intramural nodules that may demonstrate signal drop out on out-of-phase images.
- Findings such as extension into the adjacent liver and the presence of lymphadenopathy can be seen in up to one third of the patients and may mimic gallbladder cancer.
The preferable treatment is open cholecystectomy due to the extent of inflammation and the high preoperative suspicion of gallbladder cancer.
A 68-year-old diabetic woman with history of gallstones presents with epigastric pain, nausea and vomiting for the past 5 days. Patient underwent initial abdominal radiograph followed by CT of the abdomen, Fig. 16. What are the main findings? What is your diagnosis?
Bouveret syndrome is a rare variant of gallstone induced ileus causing gastric outlet obstruction by an impacted stone in the distal stomach or proximal duodenum.
Gallstone ileus complicates 0.3-0.5% of all cases of cholelithiasis and less than 3% of theses cases present as Bouveret syndrome. Mortality is being estimated in up to 12% of the cases due to unfamiliarity with this condition.
Commonly occurs in elderly females and presents with nonspecific signs and symptoms such as nausea, vomiting and epigastric pain.
Key imaging findings:
Presence of Rigler’s triad is the main hallmark and consists of bowel obstruction, pneumobilia and ectopic gallstone.
Abdominal radiography may demonstrate signs of bowel obstruction and migratory calcified gallstone.
CT and MRI are more sensitive in demonstrating the Rigler’s triad and localizing the site of the fistula. MRCP can be useful in identifying isoattenuating stones not seen on CT.
Endoscopy is the first line of treatment, given the high number of poor surgical candidates. In case of endoscopic failure, enterolithotomy alone is usually performed.
An 18-month-old male presents with right upper quadrant mass and intermittent jaundice for the past 6 months. Patient underwent US and MR of the abdomen, Fig. 19. What are the main findings? What is your differential diagnosis?
Choledochal cysts (CC) are rare congenital cystic dilatations of the biliary tract of uncertain etiology.
Incidence is higher in Asia and female population.
CC are usually present in the first decade of life. Common presentations include abdominal pain, jaundice and right upper quadrant mass. A wide variety of complications may occur such as biliary stones, cholangitis, pancreatitis, secondary biliary cirrhosis and cholangiocarcinoma.
Key imaging findings:
- MRCP is the imaging of choice for CC diagnosis, classification and treatment planning.
Five types of CC are described according to Todani’s classification system:
- Type I: fusiform dilatation of the extrahepatic duct. This is the most common type of CC (80-90% of cases), Fig. 22.
- Type II: true diverticula of the common bile duct (CBD).
- Type III: Dilatation of the intraduodenal portion of the CBD.
- Type IV: Involvement of both intrahepatic and extrahepatic bile ducts.
- Type V: Intrahepatic bile duct dilatation without underlying obstruction or extrahepatic bile duct involvement, also known as Caroli’s disease Fig. 23.
Treatment depends on the type of CC and extent of biliary involvement. Complete surgical resection of the cyst is usually performed for extrahepatic CC and segmental hepatectomy is recommended for localized intrahepatic involvement. Liver transplant is preferable for Caroli’s disease (Type V CC).
A 78-year-old male presents with history of multiple episodes of diarrhea, chills and abdominal pain after cholecystectomy. Patient underwent US (not shown) and MRI of the abdomen, Fig. 24. What are the main findings? What is your diagnosis?
Answers: Fig. 25.
Biliary enteric fistulas are rare (less than 1% of cases) and usually present in patients with chronic gallstone disease.
Cholecystoduodenal fistulas are the most common type followed by cholecystocolic fistulas (biliary-colonic fistulas).
Biliary-colonic fistulas may occur spontaneously secondary to choledocholithiasis o colonic diverticulitis,and very rarely as a complication of cholecystectomy (0.06-0.14% of cases).
Most patients present with nonspecific symptoms such as chronic diarrhea, nausea, vomiting and right upper quadrant pain, with or without peritoneal signs.
Key imaging findings:
ERCP has been recognized as the best tool for the diagnosis, although it is invasive and has associated complications.
MRCP has been proposed as the best noninvasive imaging study to delineate the biliary tree anatomy and localize the site of the fistula.
Treatment consists of cholecystectomy with resection of the fistulous tract. In iatrogenic cases such as after cholecystectomy, biliary reconstruction is commonly required with hepaticojejunostomy.
A 33-year-old female presents with abdominal pain, jaundice and fever 2 days after surgery. Patient underwent abdominal US, CT and MR, Fig. 26. What are the main imaging findings? What is your diagnosis?
Iatrogenic biliary injuries have been increasing in incidence, over the past few decades, given the rising number of hepatobiliary surgical procedures such as laparoscopic cholecystectomy (1% of cases).
Most common causes are due to slippage of the cystic duct ligature, leaks from the gallbladder bed or leak from an accessory or anomalous bile duct.
Patients commonly present with nonspecific symptoms such as abdominal pain, nausea, vomiting and fever.
Key imaging findings:
US and CT usually demonstrate free or loculated fluid.
Hepatobiliary scintigraphy can provide useful information and demonstrate the presence of free or contained leakage. Its main limitation is poor spatial resolution.
MRCP is the most useful noninvasive imaging study to delineate the exact site of biliary injury and characterize the fluid collections.
ERCP is highly accurate although it cannot visualized leaks from ducts discontinuous from the central biliary tree.
Management of these injuries often require a multidisciplinary team. Small asymptomatic bilomas tend to resolve spontaneously and larger loculated peri or intrahepatic fluid collection often require imaging-guided percutaneous drainage. ERCP is highly effective in the management of bile duct injuries (success over 90%), except in cases of complete circumferential transection of the biliary duct. In such cases, surgical repair is need it.
A 40-year-old male complains of abdominal pain, nausea and vomiting for the past 5 days. The patient has a history of prior cholecystectomy 8 months ago. Patient underwent abdominal US (image not shown) and CT evaluation, Fig. 29 . What are the main imaging findings? What is your diagnosis?
Answer: Fig. 30.
Lost gallstones are a common complication of laparoscopic cholecystectomy due to perforation of the gallbladder and spillage of bile and stones into the abdominal cavity.
Risk factors include presence of cholecystitis, advanced patient age, presence of pigmented stones, number of stones greater than 15 and resident participation.
Latest literature review reports an incidence of 10-35% of laparoscopic cholecystectomy cases. Subsequent abscess formation occurs in 0.6-2.9% of the cases.
Other complications include wound infection, fibrosis, small bowel obstruction, fistulas and cutaneous sinus formation.
Clinical presentation varies and may occur days to months after surgery, making it difficult to be associated to prior surgical procedure.
Key imaging findings:
Diagnostic workup generally involves CT imaging, as it is the best tool for visualizing a spilled stone and an associated abscess. US and barium studies are less frequently used.
Treatment usually involves drainage of the abscess and stone removal.
A 59-year-old male presents with six months of progressive malaise, fatigue, weight loss and leukocytosis. Patient underwent US, CT and MRI of the abdomen, Fig. 31. What are the main imaging findings? What is your differential diagnosis?
Gallbladder carcinoma is an uncommon malignancy with poor prognosis.
Adenocarcinoma is the most common type of gallbladder malignancy (90% of the cases), followed by adenosquamous/squamous cell carcinoma (1.7-12% of the cases).
Risk factors include female gender, elderly population, Mirizzi syndrome, chronic cholelithiasis and porcelain gallbladder.
Patients usually present with nonspecific symptoms such as right upper quadrant pain and jaundice. 50% of the cases are found incidentally after cholecystectomy.
Key imaging findings:
- Usually presents as a mass completely occupying or replacing the gallbladder lumen, focal or diffuse wall thickening or an intraluminal polypoid lesion.
- Invasion of the adjacent liver and biliary tree, biliary obstruction and/or lymph node metastases are common associated findings in advanced cases.
- US is usually the first imaging study, but it is limited at detecting early lesions.
- CT or MR are widely preferred for further characterization of potentially malignant gallbladder lesions and metastasis evaluation.
Surgical candidates will generally undergo cholecystectomy, resection of hepatic margins bordering the gallbladder fossa and en bloc nodal dissection. Adjuvant radiation therapy and systemic chemotherapy have shown improved survival. For unresectable cases, adjuvant or palliative chemotherapy and percutaneous interventions are usually recommended.