Table 3.
Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | |
1 - 2 mo | |||||
Ultrasound | L normal, | L normal, | L normal, | L normal, | L enlarged, |
BD normal, | BD normal, | BD normal, | BD normal, | hyperechoic, | |
GB sludge | GB sludge | GB sludge | GB sludge | BD normal, | |
GB contracted, | |||||
splenomegaly | |||||
ERC | Stenoses and loss of IHBD, | - | IHBD irregular, | - | - |
CBD normal, | CBD normal, | ||||
GB normal | GB normal | ||||
MRT/MRCP | - | - | - | L normal, BD | - |
normal, GB normal | |||||
4 - 6 mo | |||||
Ultrasound | L cirrhosis, | L inhomogeneous, | L inhomogeneous with | - | L enlarged, |
hyperechoic areas | |||||
alongside the IHBD, | BD normal, | ||||
BD normal, | BD normal | BD normal, | BD normal, | ||
GB stones | GB normal, | splenomegaly | |||
ERC | - | IHBD: multifocal short | - | IHBD irregular, | |
strictures and dilatations, | beaded | ||||
CBD normal, | appearance, | ||||
GB normal (Figure 2) | CBD normal, | ||||
GB normal | |||||
Liver histology | Substantial cholestasis; | Portal tract inflammation, | Canalicular bile thrombi; | - | - |
inflammation of the portal | occasionally lymphocytes | edematously swollen | |||
tracts, liver lobules, and | in bile duct epithelium; | portal tracts; | |||
sporadically the bile ducts; | several necrotic foci with | inflammatory infiltrates, | |||
feathery degeneration of | foamy macrophages in | esp. around bile ducts; | |||
hepatocytes; | the liver acini; | occasionally feathery | |||
fibrosis around bile ducts; | bridging fibrosis; | degeneration of hepatocytes; | |||
regenerative bile duct | regenerative bile duct | minimal fibrosis | |||
proliferations | proliferations | ||||
12 - 24 mo | |||||
Ultrasound | L inhomogeneous cirrhosis | L enlarged, inhomogeneous, | L cirrhosis, | L cirrhosis, | - |
hyperechoic areas segment | |||||
7/8, IHBD slightly dilated, | IHBD slightly dilated, | ||||
BD normal, | CBD normal, | BD normal, | CBD normal, | ||
GB stones and sludge, | GB stones, | GB normal, | GB normal, | ||
splenomegaly, | splenomegaly | splenomegaly | splenomegaly | ||
distinct ascites | |||||
ERC | stenoses and loss of IHBD, | IHBD: loss and stenoses | - | IHBD: multifocal | - |
of right-sided bile ducts, | high-grade strictures and | ||||
a long, stretched running | dilatations on the left side, | ||||
left bile duct (suitable to | bile ducts on the right | ||||
liver hypertrophy), | side not presentable, | ||||
CBD normal, | CBD normal, | CBD normal | |||
GB normal | GB stones | ||||
MRT/MRCP | - | L atrophy of right liver, | L macronodular cirrhosis, | - | - |
hypertrophy of left liver; | |||||
reduced signal intensity of | |||||
segments 2 and 3 and | |||||
partly 7 and 8; | |||||
IHBD segmentally dilated, | |||||
CBD pseudoobstruction, | CBD pseudoobstruction, | ||||
GB stones, splenomegaly | splenomegaly | ||||
Liver histology | No cholestasis; | - | Occasionally canalicular | Substantial cholestasis (bile | |
complete liver cirrhosis | cholestasis; mild | thrombi in dilated canaliculi); | |||
portal inflammation | occasionally lymphocytes in | ||||
(predominantly | the epithelium of bile ducts; | ||||
lymphocytes); some | numerous regenerative | ||||
regenerative bile duct | bile duct proliferations in the | ||||
proliferations; mild | periphery of portal tracts; | ||||
fibrosis; (sampling error ?) | cirrhosis (Figure 4) |
BD: bile ducts; CBD: common bile duct; GB: gallbladder; IHBD: intrahepatic bile ducts; L: liver.