Teaching Case

History of Crohn's disease, recurrent episodes of cholangitis,intrahepatic lithiasis

The patient had a history of Crohn's disease, treated conservatively for several years, as well as a mild sclerosis of the sacroiliac joints. He suffered from recurrent episodes of cholangitis for several months. An ultrasonographic examination at that time revealed intrahepatic lithiasis of the subdiaphragmatic liver segment. An ERCP was performed and demonstrated multiple radiolucent stones of relative small size, impacted in the 7th and 8th liver segment, while all other bile ducts were free of disease (Fig 1a). Segmental liver resection was initially proposed, but the patient refused. Percutaneous treatment was decided as an alternative. Percutaneous transhepatic cholangiography confirmed the endoscopic findings, showing multiple lithiasis and strictures in almost every bile duct of the subdiaphragmatic segments (Fig 1b). Subsequently a 8 Fr biliary external draining catheter was introduced and replaced by a 12 Fr catheter 3 days later (Fig 1c). Daily lavage with Ursodeoxycholic acid for stone dissolution was performed through the catheter. However, after one week no progress was noted, and so this kind of treatment was terminated. Mechanical lithotomy with help of special baskets and stone-extraction balloons, combined with balloon dilatations of all stenotic segments was performed for several weeks (Fig 2a,b). In total, 8 lithotripsy sessions took place during a two-month period until total clearance was achieved (Fig 3a). Cholangioscopy was also undertaken for three indications. First for guiding electrohydraulic lithotripsy of relative larger calculi, second for controlling all segmental branches for residual stones and third for checking the patency of the dilated ducts. Follow-up ultrasonographic examinations revealed a relative hyperechogenic bile duct wall during the next 2 years, without evidence of new stones (Fig 3b).

Crohn's disease is a non-specific inflammatory disease of the bowel, which can be complicated with biliary disease. Such secondary biliary inflammation can lead to recurrent cholangitis and strictures. These strictures can cause formation of multiple small stones which are usually pigmented. Rarely, we have encountered small cholesterol stones, so that we decided to use Ursodeoxycholic acid solutions for one week before starting mechanical lithotripsy. If lithiasis occurs in several intrahepatic biliary segments, the management of the patient is difficult. Surgical treatment can be achieved only by segmental resection of the affected area. Intrahepatic impacted stones proximal to stenotic ducts are not easily accesible by endoscopy, so that this treatment option usually fails. Post-inflammatory strictures are often difficult to be dilated due to fibrotic changes. Recurrence of previously dilated strictures is a common late complication of percutaneous treatment. Biliary calculi can recurr months or years after lithotomy, due to two main reasons; first, not every stone or fragment is always removed and second, despite total clearance, new stones can be formated because of recurrent stenosis or due to other lithogenic causes. Cholangioscopy with direct visual control of every affected bile duct can be very useful for excluding residual lithiasis. Instruments of 9,9 Fr outer diameter are very flexible and optimal for this purpose. Regular follow-up with ultrasonography is essential for discovering recurrent stones or dilated ducts. This follow-up examination should be performed by the same radiologist who can compare with the previous imaging findings. Otherwise "normal" findings like residual dilatation or hyperechogenic bile duct walls with or without posterior shadow, can be falsely recognized as abnormal.