Percutaneous Biliary Intervention in Patients with Primary Sclerosing Cholangitis

Fig. 15.1

(a–c) The typical steps of percutaneous transhepatic drainage. Needle passes are performed with injection of contrast until the biliary system is identified, often from access of a relatively central duct. A peripheral duct is then selected for puncture that will optimize drainage of multiple liver segments (a). An .018-in. torqueable guide wire is passed through the needle into the biliary system. This permits placement of a catheter that is used to advance a wire into the bowel (b). After serial dilation of the transhepatic tract and sometimes the ducts themselves, a percutaneous biliary drainage catheter is placed through the liver and into the small bowel (c)

After the tract matures, typically 2 weeks, dominant strictures are evaluated with both brush cytology including fluorescence in situ hybridization (FISH) and forceps biopsy under fluoroscopic guidance. When these results are negative but suspicion for cancer remains, transhepatic cholangioscopic evaluation is planned. Transhepatic fine-needle aspiration biopsy is no longer performed due to concern for peritoneal seeding and exclusion from future transplantation.

Balloon dilation of both dominant strictures and long intrahepatic strictures is then performed at 6-week intervals followed by biliary catheter exchange until symptoms improve. Unlike endoscopy where intervention is usually limited to hilar and extrahepatic duct strictures, peripheral strictures may be treated without additional morbidity, as many strictures in the opposite lobe reachable by contralateral access or adjacent lobe by ipsilateral access are dilated. Stricture dilation with long inflation times, utilizing high pressure (20 atm) balloons of 4–6 mm diameter intrahepatically or 10 mm at the hilum or extrahepatically, is followed by placement of an 8 Fr. internal-external drainage catheter. In patients with PSC, larger drains often obstruct downstream ducts, and serial drain enlargement, as is often employed when treating other benign strictures, is not appropriate. Three-month evaluation for drain removal is planned; clinical response in addition to stricture appearance dictates removal. Because most extrahepatic strictures are successfully managed with endoscopy, percutaneously placed metallic stents are not part of the treatment paradigm in PSC patients.

Post-procedurally, patients are admitted for pain control and treatment of cholangitis. Patients will have drain-related pain for 2–3 weeks that often requires oral narcotics. Management requires patient education and training, particularly in the first 6 weeks when pain may be severe and drain complications frequently arise.

Results

Percutaneous Transhepatic Cholangiography for PSC Diagnosis

Cholangiographic findings in PSC are not specific. The differential includes a long list of secondary causes of biliary inflammation and obstruction that result in similar bile duct abnormalities. Interpretation of cholangiography is therefore made in conjunction with the clinical presentation (most often in young and middle-aged serologically negative males with inflammatory bowel disease) of cholestasis (elevation of alkaline phosphatase and gamma-glutamyl transferase) without identification of secondary cause. Small duct PSC, a PSC variant, can have a similar clinical presentation but normal cholangiography.

MacCarty et al. described the cholangiographic findings in 86 patients with PSC in whom secondary causes of cholangitis were excluded and compared them to the cholangiographic findings in 82 patients with bile duct carcinoma and 16 patients with primary biliary cirrhosis (PBC) [8]. PSC was characterized by multifocal, short, annular strictures of both the intra- and extrahepatic bile ducts alternating with normal or slightly dilated segments to produce a “beaded” appearance. Confluent long strictures were found with advanced disease (Fig. 15.2). A specific finding, occurring in 27 % of patients, was diverticulum of the extrahepatic duct with or without associated band-like strictures. The lack of extrahepatic disease in PBC patients can differentiate PBC from PSC. Typical patient populations are also different as PBC is most likely to present in young to middle-aged female patients with positive serology (anti-mitochondrial antibody). Bile duct cancer in patients without PSC tended to be focal or multifocal at presentation.

Fig. 15.2

A catheter placed into the left duct (segment 2) has been injected in a patient who has undergone prior hepatico-jejunostomy. Cholangiography demonstrates a typical appearance of severe PSC with multiple short strictures of the intrahepatic bile ducts that result in a “beaded” appearance (thin black arrows). Some strictures appear confluent (white arrow). Despite severe disease there is not significant biliary ductal dilation. All liver segments can be identified although abnormal, except for segment 8. The anastomosis is not well visualized on this image, but an extrahepatic diverticulum is suggested (thick arrow)

Interpretation of cholangiograms requires a methodical segmental duct assessment. Isolated segmental or lobar ducts can be readily missed without careful assessment. Dominant strictures, defined by Stiehl et al., include an extrahepatic stricture of ≤1.5 mm in the common bile duct or ≤1.0 mm in the hepatic duct within 2 cm of the bifurcation [9].

There are no definitive cholangiographic features of malignancy, although perihilar location for CCA is most common. The suspicion of cancer is increased with discovery on MRI/MRC of a new dominant stricture, focal bile duct thickening or irregularity, and venous phase enhancement of an associated mass [6]. Clinical signs include new evidence of biliary obstruction, worsening enzymes, and elevation of CA19-9 greater than 100 U/mL in the absence of cholangitis [6]. Early stricture recurrence after dilation is also a suggestive feature.

Percutaneous Transhepatic Drainage and Stricture Dilation

One of the first descriptions of PTD and stricture dilation in PSC patients by May et al. described an experience at the Mayo Clinic in 14 PSC patients with dominant strictures presenting with intractable pruritus and jaundice or recurrent episodes of bacterial cholangitis [10]. Access was transhepatic (n = 9) or via T-tube (n = 5). PTD was completed and followed, after 24–48 h, with stricture dilation utilizing short inflation times of less than one minute of 10 atm PTA balloons ranging in size from 5 to 8 mm for intrahepatic and 6–10 mm for extrahepatic strictures. Internal-external drainage catheters of 10–14 Fr. were placed. Strictures were dilated in all 14 patients. Treatment resulted in a decrease in the number of cholangitic episodes. In nine patients (64 %) with recent onset of jaundice (<6 months), a decrease in bilirubin was seen. PTD was complicated by bacteremia or cholangitis in 5 (36 %) patients. One-third of patients with symptomatic resolution initially had symptom and stricture recurrence at 6–18 months.

Skolkin et al. described attempted PTD and stricture dilation in 15 PSC patients, utilizing transhepatic access (n = 13) or indwelling T-tubes (n = 2) [11]. In all patients, alkaline phosphatase was three times normal, and ten of these patients had bilirubin elevation. Clinical indications included jaundice, pruritus, or a progressive rise in alkaline phosphatase and/or bilirubin. All patients had multifocal, intrahepatic strictures, and a majority also had confluence or extrahepatic strictures. Stricture dilation was performed serially with 4–10 mm PTA balloons with inflation pressures of 6–10 atm for 5 min. Five 14 Fr. internal-external drainage catheters were placed with exchange at 6–8 weeks for an average of 4.5 months. PTD was successful in 14 patients, 13 of which improved symptomatically. An average of 9 h in 3.7 sessions was required to accomplish these results. Five of 13 patients developed recurrence, four were retreated, and one underwent liver transplantation. A majority of patients demonstrated biochemical improvement or no change, after drain removal compared with before drain placement, with recurrent enzyme elevation after 10 months on average. Fever complicated all procedures. One patient experienced arterial bleeding secondary to a pseudoaneurysm. Escalation of care from complications occurred in 7 (47 %) patients. There were no deaths.

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