Answer for BIR CoW 30 May 2021
HIV cholangiopathy
Findings
Dilated intrahepatic biliary radicles , right hepatic duct , left hepatic duct , common hepatic duct , proximal common bile duct with also involving the ampullary region abrupt narrowing of distal common bile duct for a length of 0.9cm. Multiple segmental narrowing of intrahepatic ducts noted. Gall bladder appears contracted . Main pancreatic duct appears normal
Discussion
Biliary tract disorders in HIV-infected patients are grouped as AIDS cholangiopathy. The spectrum of disorders involved in AIDS cholangiopathy includes acalculous cholecystitis, sclerosing cholangitis, papillary stenosis, lymphoma, Kaposi's sarcoma, and gallstones. Patients are usually affected when the CD4 T lymphocyte count is less than 100/mm3; however, 20% of cases may have findings in which the CD4 T lymphocyte count is greater than 100/mm3. Several pathogens are responsible for the opportunistic infections, Cryptosporidium parvum and cytomegalovirus being the most frequently involved . In two of our patients, Cryptosporidium organisms were identified in bile aspirates. Previously described imaging features of AIDS cholangiopathy rely on cholangiographic patterns that include findings resembling those of primary sclerosing cholangitis and the presence of papillary stenosis, papillary stenosis alone, the isolated presence of findings resembling those of primary sclerosing cholangitis, and segmental extrahepatic bile duct strictures. Biliary strictures are most likely caused by chronic inflammation by one or more opportunistic pathogens . The diagnosis of papillary stenosis is made when a dilated CBD reveals tapering at its terminal portion and when marked and delayed contrast retention occurs during ERCP . On MRCP, we observed dilatation of CBD with distal tapering that was associated with papillary stenosis on ERCP. However, there are no established criteria on MRCP for the diagnosis of papillary stenosis, which is believed to be caused by recurrent inflammation at the papilla by the previously mentioned pathogens. Extrahepatic segmental biliary strictures and primary sclerosing cholangitis-type strictures also develop during the inflammatory process [. In our patient population, we observed the entire previously mentioned spectrum of imaging findings on MRCP. On MRI, we saw thickening and enhancement of the CBD wall as well as enhancement along the intrahepatic bile ducts that is associated with cholangitis . Contrast-enhanced T1-weighted fat-saturated images show the enhancement and thickening of the bile duct walls, which were also seen in our patients. Previously described MRI and MRCP findings of primary sclerosing cholangitis include increased segmental arterial enhancement in the liver parenchyma, predominantly in the periphery . we observed this finding in two patients. Unlike the MRCP findings of primary sclerosing cholangitis, we also observed longer extrahepatic bile duct strictures that have been described as one of the cholangiographic patterns of AIDS cholangiopathy . Biliary strictures in AIDS cholangiopathy are indistinguishable from primary sclerosing cholangitis in the absence of segmental extrahepatic biliary strictures; clinical history may help to distinguish one from the other.
REFERENCES
1. Keaveny AP, Karasik MS. Hepatobiliary and pancreatic infections in AIDS: part one. AIDS Patient Care STDS 1998; 12:347 –357 [Crossref] [Medline] [Google Scholar]
2. Keaveny AP, Karasik MS. Hepatobiliary and pancreatic infections in AIDS: part II. AIDS Patient Care STDS 1998; 12:451 –456 [Crossref] [Medline] [Google Scholar]
3. Lefkowitch JH. The liver in AIDS. Semin Liver Dis 1997; 17:335 –344 [Crossref] [Medline] [Google Scholar]
4. Wilcox CM, Monkemuller KE. Hepatobiliary diseases in patients with AIDS: focus on AIDS cholangiopathy and gallbladder disease. Dig Dis 1998; 16:205 –213 [Crossref] [Medline] [Google Scholar]
5. Miller FH, Gore RM, Nemcek AA, Fitzgerald SW. Pancreaticobiliary manifestations of AIDS. AJR 1996; 166:1269 –1274 [Abstract] [Google Scholar]
Findings
Dilated intrahepatic biliary radicles , right hepatic duct , left hepatic duct , common hepatic duct , proximal common bile duct with also involving the ampullary region abrupt narrowing of distal common bile duct for a length of 0.9cm. Multiple segmental narrowing of intrahepatic ducts noted. Gall bladder appears contracted . Main pancreatic duct appears normal
Discussion
Biliary tract disorders in HIV-infected patients are grouped as AIDS cholangiopathy. The spectrum of disorders involved in AIDS cholangiopathy includes acalculous cholecystitis, sclerosing cholangitis, papillary stenosis, lymphoma, Kaposi's sarcoma, and gallstones. Patients are usually affected when the CD4 T lymphocyte count is less than 100/mm3; however, 20% of cases may have findings in which the CD4 T lymphocyte count is greater than 100/mm3. Several pathogens are responsible for the opportunistic infections, Cryptosporidium parvum and cytomegalovirus being the most frequently involved . In two of our patients, Cryptosporidium organisms were identified in bile aspirates. Previously described imaging features of AIDS cholangiopathy rely on cholangiographic patterns that include findings resembling those of primary sclerosing cholangitis and the presence of papillary stenosis, papillary stenosis alone, the isolated presence of findings resembling those of primary sclerosing cholangitis, and segmental extrahepatic bile duct strictures. Biliary strictures are most likely caused by chronic inflammation by one or more opportunistic pathogens . The diagnosis of papillary stenosis is made when a dilated CBD reveals tapering at its terminal portion and when marked and delayed contrast retention occurs during ERCP . On MRCP, we observed dilatation of CBD with distal tapering that was associated with papillary stenosis on ERCP. However, there are no established criteria on MRCP for the diagnosis of papillary stenosis, which is believed to be caused by recurrent inflammation at the papilla by the previously mentioned pathogens. Extrahepatic segmental biliary strictures and primary sclerosing cholangitis-type strictures also develop during the inflammatory process [. In our patient population, we observed the entire previously mentioned spectrum of imaging findings on MRCP. On MRI, we saw thickening and enhancement of the CBD wall as well as enhancement along the intrahepatic bile ducts that is associated with cholangitis . Contrast-enhanced T1-weighted fat-saturated images show the enhancement and thickening of the bile duct walls, which were also seen in our patients. Previously described MRI and MRCP findings of primary sclerosing cholangitis include increased segmental arterial enhancement in the liver parenchyma, predominantly in the periphery . we observed this finding in two patients. Unlike the MRCP findings of primary sclerosing cholangitis, we also observed longer extrahepatic bile duct strictures that have been described as one of the cholangiographic patterns of AIDS cholangiopathy . Biliary strictures in AIDS cholangiopathy are indistinguishable from primary sclerosing cholangitis in the absence of segmental extrahepatic biliary strictures; clinical history may help to distinguish one from the other.
REFERENCES
1. Keaveny AP, Karasik MS. Hepatobiliary and pancreatic infections in AIDS: part one. AIDS Patient Care STDS 1998; 12:347 –357 [Crossref] [Medline] [Google Scholar]
2. Keaveny AP, Karasik MS. Hepatobiliary and pancreatic infections in AIDS: part II. AIDS Patient Care STDS 1998; 12:451 –456 [Crossref] [Medline] [Google Scholar]
3. Lefkowitch JH. The liver in AIDS. Semin Liver Dis 1997; 17:335 –344 [Crossref] [Medline] [Google Scholar]
4. Wilcox CM, Monkemuller KE. Hepatobiliary diseases in patients with AIDS: focus on AIDS cholangiopathy and gallbladder disease. Dig Dis 1998; 16:205 –213 [Crossref] [Medline] [Google Scholar]
5. Miller FH, Gore RM, Nemcek AA, Fitzgerald SW. Pancreaticobiliary manifestations of AIDS. AJR 1996; 166:1269 –1274 [Abstract] [Google Scholar]
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!