Diagnostic Considerations
Important considerations
Choose the procedure that is appropriate for the patient. Know the limitations of the procedure. The presence of dense adhesions, advanced liver disease, unexpected cancer, or a severely inflamed gallbladder or encountering troublesome bleeding should prompt assessment of the need to convert a laparoscopic cholecystectomy to an open procedure.
Special concerns
Postcholecystectomy syndrome
The persistence, recurrence, or development of pain following the removal of the gallbladder is referred to as the postcholecystectomy syndrome. It occurs in 10%-15% of the 600,000 cholecystectomies performed annually in the United States.
A retained common duct stone can be identified in 0.3%-18% of cases. A history of recurrent biliary-type pain associated with nausea, vomiting, fever, and chills is suggestive of a retained common duct stone, and sonographic evidence of ductal dilation is supportive of this diagnosis. An intraoperative cholangiogram is very useful to exclude a retained common duct stone.
Biliary leaks or fistula occur in 0.1%-0.4% of all gallbladder operations. Leakage from the cystic duct stump is most common, but it may (1) emanate from accessory ducts along the gallbladder fossa, (2) be the result from an injury to a major extrahepatic duct, or (3) follow T-tube removal after open cholecystectomy and common bile duct exploration. In the past, these were associated with a mortality rate of about 30%.
Benign biliary strictures occur with a frequency of 0.1%-0.8% of all gallbladder operations. This usually is the result of an operative injury to the bile duct. The first type results from a surgical misadventure such as inadvertent duct ligation or placing clips on the bile duct. Only 10% of patients present with pain and jaundice in the first week, and another 60% present within 3 months of the surgery. The second type results either from ischemic injury or from crush injury from clamps. Many months or years may pass before mild cholestasis or symptoms develop when biliary sludge or stones form proximal to a stricture.
Cystic duct remnants are a rare and controversial cause of postcholecystectomy pain. The long cystic duct stump promotes bile stasis within which microlithiasis or stones may form. The passage of this material through the papilla is believed to cause intermittent biliary-type pain. In extremely rare instances, a neuroma or granuloma of the cystic duct stump can cause pain.
Sphincter of Oddi dysfunction
Sphincter of Oddi dysfunction is an uncommon, probably overdiagnosed, cause of biliary-type pain. The 2 subtypes are papillary stenosis and sphincter of Oddi dyskinesia. The former is a mechanical problem, and the latter is a functional problem.
Papillary stenosis is a mechanical problem involving an inflammatory stenosis of the duodenal papilla of Vater. It may be caused by choledocholithiasis, ascariasis, sclerosing cholangitis, pancreatitis, iatrogenic instrumentation of the duodenal papilla, peptic duodenitis, and Crohn disease involving the duodenum and cholesterolosis of the papilla. It is diagnosed manometrically when the sphincter of Oddi manometry reveals a basal sphincter pressure greater than 40 mm Hg that does not decrease in response to cholecystokinin (CCK) or amyl nitrate. It also may be diagnosed surgically via the inability to pass a #3 Bakes dilator through the sphincter or during endoscopic retrograde cholangiopancreatography (ERCP) via an inability to advance a 5F cannula retrograde over a wire placed into the bile duct.
Endoscopic sphincterotomy alleviates biliary-type pain in most, but not all, patients with papillary stenosis.
The term sphincter of Oddi dysfunction has been used to describe a clinical syndrome of biliary or pancreatic obstruction related to mechanical or functional abnormalities of the sphincter of Oddi.
Sphincter of Oddi dysfunction is diagnosed by a sphincter manometry study revealing a basal pressure greater than 40 mm Hg that is responsive to CCK or amyl nitrate. Other supportive features may include phasic wave contractions with a peak amplitude greater than 220 mm Hg, contraction duration greater than 8 seconds, and frequency of greater than 10 per minute. An increased percentage of retrograde contractions have also been cited. Abnormal sphincter motility is uncommon, identified in 1% of 454 patients following cholecystectomy; 14% of those were diagnosed with a postcholecystectomy syndrome.
HIV-associated cholangiopathy
Several biliary tract abnormalities are associated with human immunodeficiency virus (HIV) infection, but the CD4+ lymphocytes are usually below 100 cells per microliter before these problems develop. These include acalculous cholecystitis, papillary stenosis, and a sclerosing cholangitis–like picture. The causes include opportunistic infections, such as infection with cytomegalovirus, Cryptosporidium species, Enterocytozoon bieneusi, and Mycobacterium species, or neoplasms, such as Kaposi sarcoma and non-Hodgkin lymphoma. Liver-associated enzymes usually show a cholestatic picture, without significant hyperbilirubinemia, unless a neoplasm obstructs the bile duct. Medical therapy to date has been ineffective in treating opportunistic infections.
Ursodeoxycholic acid (UDCA) has been used in the treatment of primary sclerosing cholangitis, a disorder with similar intrahepatic changes to AIDS cholangiopathy. This observation has led to its experimental use in patients with acquired immunodeficiency syndrome (AIDS) cholangiopathy; results in a small number of patients have found an improvement in symptoms and a fall in the levels of serum alkaline phosphatase and gamma-glutamyl transpeptidase. The authors recommend giving UDCA in a dose 300 mg 3 times daily, primarily in patients who have intrahepatic ductal disease and markedly elevated liver function test results.
Vanishing bile duct syndrome
This is a rare syndrome affecting the intrahepatic bile ducts. Most patients are asymptomatic, but some may present with pruritus and, rarely, jaundice. The alkaline phosphatase level usually is elevated, along with gamma-glutamyltransferase (GGT), which may exceed 600 IU/L. Obtaining a wedge liver biopsy often is necessary to make the diagnosis. The cause of ductopenia may be related to developmental abnormalities of the intrahepatic biliary system, as in Alagille syndrome. On physical examination, frontal bossing and triangular facies may be noted, and tests that are more specific can reveal butterfly vertebrae and posterior embryotoxon of the eye. Progressive familial intrahepatic cholestasis is another type, caused by a genetic mutation of the membrane transporter for phospholipids (MDR3).
Other types are acquired and are associated with cystic fibrosis, systemic mastocytosis, histiocytosis-X, Hodgkin disease, and drug-induced hepatotoxicity. Patients without a clear association are diagnosed with idiopathic adult ductopenia.
Differential Diagnoses
-
Biliary Cirrhosis
-
Biliary Colic
-
Bilirubin, Impaired Conjugation
-
A normal postcholecystectomy cholangiogram.
-
Biliary disease. In a patient with persistent elevation of liver-associated enzymes, the contrast medium entering the biliary ductal system preferentially enters the cystic duct.
-
Biliary disease. Even when the catheter is advanced to the proximal common hepatic duct, contrast dye preferentially fills the cystic duct and gallbladder rather than allowing visualization of the intrahepatic ductal system (same patient as in previous image).
-
Biliary disease. In this image, the common bile duct is occluded with a balloon-tipped catheter. Contrast material fills the intrahepatic ductal system to reveal diffuse intrahepatic sclerosing cholangitis.
-
Biliary disease. Common bile duct stones are among the most frequent problems occurring in the biliary system. In this cholangiogram, the stones line up like peas in a pod.
-
Biliary disease. After a biliary sphincterotomy, a balloon-tipped catheter is used to remove the stones one by one.
-
Biliary disease. This clearing cholangiogram shows a common bile duct free of filling defects and good flow into the duodenum. The stones are visible as filling defects in the duodenal bulb.
-
Biliary disease. A patient with pancreatic cancer developed jaundice during his treatment. The cholangiogram shows a stricture in the distal common bile duct.
-
Biliary disease. A patient with pancreatic cancer developed jaundice during his treatment (same patient as in previous image). To palliate the jaundice, the biliary stricture is dilated and stented with a 10F plastic stent. Note the contrast dye flowing down the stent.
-
Biliary disease. This computed tomography scan of the abdomen shows a large tumor mass in the head of the pancreas. The brightly colored object within the mass is the biliary stent placed by endoscopic retrograde cholangiopancreatography.
-
Biliary disease. This abdominal computed tomography scan shows mild intrahepatic biliary ductal dilatation.
-
Biliary disease. Abdominal computed tomography scanning in a patient with jaundice revealed polycystic liver disease.
-
Biliary disease. Findings on this endoscopic retrograde cholangiopancreatogram exclude extrahepatic biliary obstruction but demonstrate that the intrahepatic biliary ductal system is splayed by multiple hepatic cysts.
-
Biliary disease. This cholangiogram shows a choledochal cyst. Fusiform dilatation of the entire extrahepatic bile duct is present.
-
A 92-year-old woman had recurrent abdominal pain and jaundice. A right upper quadrant ultrasonogram showed a dilated biliary duct with no stones. She had a previous Roux-en-Y surgery that made endoscopic retrograde cholangiopancreatography impossible. Critical aortic stenosis increased the risk of most interventions. This percutaneous cholangiogram, performed under conscious sedation in the operating room, revealed a large stone missed by the ultrasonogram. It was removed successfully with percutaneous choledochoscopy and electrohydraulic lithotripsy.
-
Biliary disease. This cholangiogram shows a stone too large to deliver through a standard biliary sphincterotomy.
-
Biliary disease. Here, a mechanical lithotripter is used to grab a stone too large to deliver through a standard biliary sphincterotomy and crush it into small pieces (same patient as in previous image). The smaller pieces then are removed with a balloon-tipped catheter.
-
Biliary disease. A patient had malignant strictures of the biliary system palliated with metal mesh stents. Unfortunately, the tumor grew through the metal mesh to reobstruct the biliary system.
-
Biliary disease. A patient had malignant strictures of the biliary system that were palliated with metal mesh stents, but the tumor grew through the metal mesh to reobstruct the biliary system (same patient as in previous image). In this image, after a wire was passed through the lumen, a balloon-dilating catheter was passed into the metal mesh stents and inflated to enlarge the lumen.
-
Biliary disease. A patient had malignant strictures of the biliary system that were palliated with metal mesh stents, but the tumor grew through the metal mesh to reobstruct the biliary system (same patient as in previous image). After a wire was passed through the lumen, a balloon-dilating catheter was passed into the metal mesh stents and inflated to enlarge the lumen. In this image, two plastic stents were passed into the intrahepatic ductal system to again palliate the obstruction.
