Radiography
Findings
Plain abdominal radiographs are of little use in the diagnosis of choledochal cysts. They offer no specific information related to this diagnosis. In patients presenting with abdominal pain, radiographs are frequently ordered as part of the standard workup. At most, radiographs may suggest displacement of an adjacent hollow viscus, such as the duodenum, by a mass.
Degree of Confidence
Plain abdominal radiographs are not specific for the diagnosis. If a choledochal cyst is suggested on the basis of the history, physical examination findings, and laboratory results, a confirmatory radiologic examination should be performed. The initial examination of choice is abdominal US scanning. This study should be able to depict a cystic mass in the right upper abdominal quadrant with high sensitivity and specificity. If the diagnosis remains equivocal, a CT scan or MRI can be performed.
False Positives/Negatives
False-positive and false-negative rates are high if the diagnosis is based on plain abdominal radiographs. Plain radiographs simply cannot depict choledochal cysts well. Many other masses or inflammatory processes can produce the same clinical picture, which cannot be differentiated by using plain images. Examples of these processes include an acute fluid collection associated with acute pancreatitis, a pancreatic pseudocyst, and choledocholithiasis or cholangitis.
Computed Tomography
Findings
Abdominal CT scanning is useful in the diagnostic algorithm for choledochal cysts. CT scanning is highly accurate and offers a great deal of information that is helpful not only in confirming the diagnosis but also in planning surgical approaches.
Computed tomography scan demonstrates a choledochal cyst involving the intrahepatic portion of the common hepatic duct and the main left hepatic duct.
Computed tomography (CT) CT scan demonstrates the proximity of the duodenum to the distal aspect of a choledochal cyst.
CT scans of a choledochal cyst demonstrate a dilatated cystic mass with clearly defined walls that is separate from the gallbladder. The fact that this mass arises from or actually is the extrahepatic bile duct usually is clear from its location and its relationships to surrounding structures. The cyst is typically filled with bile, which produces waterlike attenuation. Depending on the patient's age and clinical history, the wall of the cyst can appear thickened, especially if multiple episodes of inflammation and cholangitis have occurred.
Most patients with choledochal cysts have undergone abdominal US imaging prior to CT scanning. US findings suggest the diagnosis in most patients and may be conclusive in many. According to Lipsett and colleagues, CT scanning confirms an unclear diagnosis and provides information concerning the relationships of the cyst to surrounding structures.11 These include the portal vein, duodenum, and liver. In addition, CT scanning is superior to US scanning in defining the extent of the cyst in the extrahepatic biliary system and in detecting intrahepatic disease.
CT cholangiography is one of the CT imaging techniques that have been used in the diagnosis of choledochal cysts. Spinzi and colleagues published a case report describing CT cholangiography in the diagnosis of a type I choledochal cyst in an adult.12 The cyst and its extent were demonstrated clearly. The authors did note the need to use retrograde biliary contrast enhancement or intravenous contrast enhancement, and they stated that MR cholangiography may one day obviate CT cholangiography.
Lam and colleagues investigated the use of CT cholangiography versus MR cholangiography in the diagnosis of choledochal cysts in children.13 They examined 14 children and had good results with both techniques.
Satisfactory visualization of the biliary tree was achieved in 93% of patients by using CT cholangiography and in 100% of patients by using MR cholangiography. CT cholangiography adequately depicted 10 (91%) of 11 choledochal cysts, whereas MR cholangiography showed 100% of the cysts. Intrahepatic stones were detected, with sensitivities of 83% and 67% for CT and MR cholangiography, respectively. Specificities were 100% for both. CT cholangiography demonstrated the pancreatic duct and the common pancreatobiliary channel in 64% of patients, whereas MR cholangiography depicted these features in 46%. Postoperatively, CT cholangiography was better for surveillance of the hepaticojejunostomy.
The authors recommended the use of MR cholangiography as the confirmatory imaging study in children with choledochal cysts because it does not require breath holding, is noninvasive, does not require the administration of contrast material, and is not associated with ionizing radiation.
Degree of Confidence
CT scans can be relied on with a high degree of confidence. They provide a great deal of information concerning choledochal cysts and their relationships to surrounding structures. Typically, the only preoperative study that may be considered after a confirmatory CT scan is endoscopic retrograde cholangiopancreatography (ERCP). ERCP often provides additional information concerning the pancreatic duct and the distal extent of the choledochal cyst. ERCP can provide more detailed information concerning the epithelium of the cyst and detect the presence of associated tumors or strictures.
False Positives/Negatives
False-positive and false-negative results are rare. If any question remains concerning the diagnosis after a CT scan, ERCP can be performed. Of note, Kepczyk and colleagues published a case report of a choledochal cyst that mimicked a pancreatic pseudocyst.14 The diagnosis remained in doubt after US and CT scanning. The patient's presenting symptoms were those of acute pancreatitis.
Magnetic Resonance Imaging
Findings
Use of MRI and MRCP techniques is increasing dramatically for the noninvasive diagnosis of biliary and pancreatic diseases. Choledochal cysts are no exception. These cysts appear as large fusiform or saccular masses that may be extrahepatic, intrahepatic, or both, depending on the type of cyst. They produce a particularly strong signal on T2-weighted images. Associated anomalies of the pancreatic duct, its junction with the common bile duct, and the long common channel formed by the 2 are usually well demonstrated on MRI/MRCP images.15,16,17
Miyano and Yamataka used non–breath-hold MRCP to diagnose choledochal cysts in 3 children who presented with abdominal pain and elevated serum amylase levels or with a suspected choledochal cyst on sonograms.7 In all patients, the choledochal cyst was demonstrated clearly. In 2 of 3 patients, APBJs were demonstrated, along with lengthy common channels. The size of the cyst precluded imaging these in the third patient. ERCP also failed to depict the APBJ and common channel in the third patient. The authors concluded that MRCP may be as effective as ERCP in diagnosing choledochal cysts, and it may replace ERCP one day.
Lee and Lee et al compared MRCP and ERCP in 46 patients with various extrahepatic biliary diseases, including choledochal cysts. ERCP was performed in 13 patients, followed by MRCP, and MRCP was performed in 33 patients, followed by ERCP. MRCP findings were diagnostic in 98% of patients. In patients with biliary obstruction, MRCP correctly showed the level of obstruction in 91%, compared with 83% for ERCP (P <.05). The overall diagnostic accuracy rates were similar between the groups.
Sugiyama and colleagues tested the efficacy of MRCP in the diagnosis of APBJ, with results confirmed by means of ERCP.18 APBJ was identified in 82% of patients in whom ERCP had been used to demonstrate the presence of this anomaly. Choledochal cysts were diagnosed in 7 patients. All of the cysts were visualized with MRCP. MRCP studies failed to depict 1 gallbladder carcinoma and 1 case of hyperplasia of the gallbladder mucosa. The authors concluded that MRCP was an accurate technique for the diagnosis of APBJ and choledochal cysts. Yu and colleagues confirmed these results in 2004.19
Irie and associates published the results of using MRCP in the diagnosis of choledochal cysts in 16 patients.20 They found that MRCP defined the proximal bile duct better than ERCP but that defects in the distal common bile duct were missed with MRCP in 2 pediatric patients. APBJ was delineated in all 6 adult patients but was missed in 6 of 10 pediatric patients. The authors concluded that MRCP is an important noninvasive diagnostic study for choledochal cysts but that it should not replace ERCP, especially in children.
Matos and colleagues compared MRCP with ERCP in 8 patients.21 In this series, ERCP and MRCP findings were completely correlated with respect to the type of cyst (type I, n = 7; type IV, n = 1) and the presence of an APBJ and a common channel in all patients. The authors concluded that MRCP provides information equivalent to that derived from ERCP.
Kim, Lim, and colleagues compared MR cholangiography with conventional cholangiography in 13 patients with choledochal cysts.22 MR cholangiography was superior for complete imaging of the cyst (P = .03). Detection rates for the APBJ (P = .641) and bile duct stones (P = .375) were not significantly different. The authors concluded that MR cholangiography was equivalent or superior to conventional cholangiography in the evaluation of choledochal cysts.
De Backer and colleagues described MRI/MRCP findings in a patient with a choledochocele in whom US- and CT-scan findings failed to indicate a diagnosis.23 The MRI techniques revealed a distal common bile duct cyst in the duodenal intramural portion of the duct, which was seen protruding into the duodenal lumen.
Matsufuji and coinvestigators employed secretin-stimulated MRCP to dynamically study pancreatobiliary reflux in patients with known choledochal cysts.24 Six patients were studied. MRCP images were obtained at 1-minute intervals for 15 minutes after the administration of secretin. Increased common bile duct signal intensity was detected in only 50% of these studies. The clinical relevance of this technique is currently not well established.
Degree of Confidence
On the basis of reported results, MRCP appears to be a reliable study in the diagnosis of choledochal cysts, with diagnostic accuracy of 82-100%. In preparation for surgery, particularly when information is needed concerning the location of the APBJ and the length of the common channel, another study, such as ERCP, should be performed.
False Positives/Negatives
Differentiating a choledochal cyst from a pancreatic pseudocyst or other cystic lesions of the pancreas may be difficult using MRI findings. No normal anatomic variants mimic a choledochal cyst.
False-negative findings of the absence of the APBJ and the common pancreatobiliary channel are possible. In addition, small neoplasms of the gallbladder and bile duct may be missed.
Ultrasonography
Findings
US scanning is the initial screening examination of choice in patients with choledochal cysts. Pertinent findings include a cystic extrahepatic mass. Depending on the skill of the operator, the specific type or class of choledochal cyst may be identified. Newer, high-resolution US machines help clinicians make such diagnoses. Furthermore, advances in US technology have enabled ultrasonographers to make the diagnosis in the antenatal period.
US scan findings are diagnostic in many patients; however, in the preoperative period, complementary studies, such as ERCP, CT scans, or MRI/MRCP, may be helpful in delineating details of the surrounding anatomy, the location of an APBJ, and the length of the common pancreatobiliary channel.
Abdominal US scan findings can help in detecting associated conditions and complications of choledochal cysts, such as choledocholithiasis, intrahepatic biliary dilatation, portal vein thrombosis, gallbladder or biliary neoplasms, pancreatitis, and hepatic abscesses.
The importance of abdominal US scanning in this disease process is highlighted by the fact that US scan findings initially suggested the diagnosis in many of the studies dealing with other diagnostic modalities referenced in this article.
In surgical series with a total of 140 patients, US scanning was used early in the diagnostic algorithm.25,26,27 In these studies, US scan findings, although sensitive, were routinely confirmed by using another study or procedure, such as CT scanning, hepatoiminodiacetic acid scanning, or ERCP.
Miyano and Yamataka confirmed that US scanning is the initial radiologic study of choice to be used if a choledochal cyst is suggested.7 The authors recommended the use of CT scanning or MRI to better delineate the relationship of the cyst to the surrounding structures.
Saing and colleagues described their results in 84 patients with choledochal cysts. These authors relied on US scanning to make the initial diagnosis in all patients.28 Follow-up CT scans were obtained in 80% of patients, and the authors used percutaneous transhepatic cholangiograms to evaluate suspected intrahepatic biliary stones or to examine strictures in the postoperative period. ERCP was used in 33% of patients.
Diagnostic sonogram demonstrating a type I choledochal cyst in a 4-month-old child presenting with elevated hyperbilirubinemia and hepatic transaminase levels.
Lee and colleagues reviewed results in 162 pediatric patients undergoing US examinations for biliary tract dilatation.29 A total of 112 patients had choledochal cysts. Mean bile duct diameter was greatest in patients with choledochal cysts versus other pathologies (21.4 vs 10.0 mm for biliary atresia with associated cystic dilatation, 8.5 mm for secondary causes of bile duct dilatation, and 4.4 mm for normal variants, P <.001).
Mackenzie and colleagues reported on 3 children who had a prenatal US diagnosis of extrahepatic cystic masses.30 Biliary atresia with an associated cystic mass was present in 2 patients. A type I choledochal cyst was present in 1 patient. All 3 patients were explored surgically and underwent corrective surgery within 2 weeks. The authors concluded that prenatal US scanning led to accurate diagnoses and expeditious, definitive therapy in all patients. These findings were reinforced in a 2004 study conducted by Chen and colleagues.31
Benhidjeb and colleagues, reporting in 1996, described a new case of antenatal US diagnosis of a choledochal cyst and reviewed 15 previously reported cases.32 The cyst was demonstrated on US examination in the 29th week of gestation. The literature review revealed that this diagnosis has been made using US as early as the 15th week of gestation. The lesions appear as sonolucent cysts in the region of the porta hepatis. The authors stated that the advantage of antenatal diagnosis is that it facilitates early, definitive surgical therapy, precluding the development of complications, such as cholangitis, hepatic abscesses, and cirrhosis.
A 2004 study by Sgro and colleagues described the utility of ultrasound in the prenatal diagnosis of Caroli disease.33
Degree of Confidence
US scanning is sensitive in the demonstration of a cystic lesion in the region of the porta hepatis. If such a lesion is visualized, US scanning frequently may provide enough information to make the diagnosis of a choledochal cyst.
In most patients, a complementary study is necessary. CT scanning and MRI provide detailed information concerning relationships to surrounding anatomic structures. ERCP or percutaneous transhepatic cholangiography provides more anatomic detail regarding the duct.
False Positives/Negatives
No actual normal variants are mimetic of choledochal cysts. Pathologic conditions in the differential diagnosis include pancreatic pseudocysts, hepatic cysts, and biliary atresia with associated cystic masses. At times, differentiating these lesions may be difficult using US scan findings. In this situation, other confirmatory tests are definitely indicated.
Interestingly, Sato and co-investigators reviewed their US findings in 12 patients with choledochal cysts.34 They found that the cyst diameter changed greatly under compression and cautioned against compressing the structures, since this can lead to confusion in the diagnosis.
Nuclear Imaging
Findings
Hepatobiliary scintigraphic modalities are used commonly in the setting of acute cholecystitis and in the investigation of neonatal jaundice. In addition, these techniques are useful in the diagnosis of choledochal cysts.
Nuclear medicine scan of a choledochal cyst. Early image shows most of the radionuclide in the liver.
Kao and co-investigators studied the significance of nonvisualization of the gallbladder on cholescintigraphy in 27 patients with choledochal cysts.35 Nonvisualization of the gallbladder occurred in 18 (67%) of 27 patients at 4 hours after injection of the radionuclide. Most of the patients did not have acute cholecystitis. The authors concluded that nonvisualization of the gallbladder in this patient population is not necessarily indicative of acute cholecystitis and that large choledochal cysts may compress the gallbladder, leading to nonvisualization.
Aigner and colleagues used cholescintigraphy to evaluate postoperative bile flow in infants undergoing cyst resection and hepaticojejunostomy or hepaticoantrostomy.36 A total of 12 patients were examined. No significant differences in bile flow were found between the hepaticojejunostomy group and the hepaticoantrostomy group. Flow was well imaged in all patients. The authors concluded that the method was an effective way to study postoperative bile flow.
Lin and colleagues compared cholescintigraphy (using technetium-99m disofenin) with US scanning in the diagnosis of neonatal jaundice.37 A total of 66 neonates with cholestatic jaundice were entered in the study, which was undertaken principally to determine which modality was superior for differentiating biliary atresia from other causes of neonatal jaundice. Choledochal cysts were present in 3 of the infants. The diagnostic accuracy of cholescintigraphy was superior, but the authors also concluded that US scanning should remain the initial imaging study of choice to exclude such anatomic abnormalities as choledochal cysts.
Johnson and co-investigators used mebrofenin cholescintigraphy to evaluate 58 neonates with jaundice.38 Choledochal cysts were present in 4 of the infants; 3 (75%) of the cysts were detected by cholescintigraphy.
Rajnish and colleagues examined the ability of hepatobiliary scintigraphy to classify choledochal cysts.39 A total of 21 patients with confirmed choledochal cysts (12 type I, 9 type IV) had cholescintigraphic examinations; 2 nuclear medicine specialists were asked to review the scans in an attempt to classify the choledochal cysts according to the Todani classification.
Hepatobiliary scintigraphy correlated with ERCP or surgical diagnosis in 86% of patients. All 12 type I cysts were diagnosed correctly using hepatobiliary scintigraphy. The sensitivity for the diagnosis and classification of type IV cysts was 67% (6 of 9 cases). The difficulty here was the relative inability to diagnose intrahepatic disease in type IV cysts. The authors concluded that hepatobiliary scintigraphy was an effective method to use for help diagnosing type I and type IV choledochal cysts.
Degree of Confidence
Hepatobiliary scintigraphy has reasonably good accuracy in the diagnosis of choledochal cysts. However, it probably should not be relied on as the sole study and should be complemented by ERCP, CT scanning, or MRCP.
False Positives/Negatives
False-negative results can derive from the relative inability to diagnose intrahepatic disease in type IV choledochal cysts. No known normal anatomic variants mimic this disease process.
Angiography
Findings
Angiographic techniques are not part of the standard workup for choledochal cysts; however, angiography studies and angiographic interventions can be instrumental and even lifesaving when major vascular complications associated with choledochal cysts occur. Fortunately, these complications are rare, and most experience has been limited to case reports.
Eliscu and Weiss described the case of a 21-month-old girl who developed melena and hematemesis and presented in shock, with a large, palpable mass in the right upper quadrant.40 The patient underwent emergent upper endoscopy, and blood was seen spurting through the ampulla of Vater. Abdominal US scan findings demonstrated a subhepatic mass. The patient then underwent angiography, which revealed a 1.5-cm aneurysm in the right hepatic artery. This was embolized with steel coils, and the patient was stabilized. Subsequently, the diagnostic workup was completed, and 1 month after embolization, the patient underwent uneventful resection of a choledochal cyst and adjacent abnormal artery.
Kirimlioglu and colleagues published the case of a 31-year-old woman who presented to the hospital with nausea, vomiting, jaundice, and abdominal pain.41 US scan findings demonstrated a choledochal cyst. CT scans showed an adjacent 3-cm X 4-cm mass consistent with a blood vessel compressing the gallbladder and bile duct. A celiac angiogram demonstrated a 5-cm X 5-cm aneurysm of the proper hepatic artery. The patient later went into shock when the aneurysm ruptured; however, she was rushed to the operating room for definitive treatment of the cyst and hepatic artery. The patient completely recovered.
Degree of Confidence
Published case reports indicate that angiography is accurate for the diagnosis of vascular complications associated with choledochal cysts. Beyond this, little can be said because of the paucity of case reports and series.
False Positives/Negatives
No false-positive or false-negative results related to the use of angiography in the evaluation or treatment of choledochal cysts have been described.
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Further Reading
Related eMedicine topics
Choledochal Cysts (from Gastroenterology)
Choledochal Cyst, Surgical Treatment
Caroli Disease (from Radiology)
Caroli Disease (from Pediatrics: General Medicine)
Bile Duct Tumors
Clinical guidelines
ASGE Guideline: The Role of ERCP in Diseases of the Biliary Tract and the Pancreas
Keywords
choledochal cyst, bile duct cysts, Caroli's disease, congenital anomalies of the bile ducts, cystic dilatation, extrahepatic biliary tree, intrahepatic biliary radicles, choledochocele, cystic dilation, cyst dilation, cyst dilatation
