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|Year : 2010 | Volume
| Issue : 4 | Page : 287-289
Recurrent cholangitis in the tropics: Worm or cast?
PA Jain1, VV Gandhi1, P Desai2, NH Doctor1
1 Department of Surgical Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, India
2 Department of Medical Gastroenterology, Surat, India
|Date of Submission||08-Jan-2010|
|Date of Decision||01-Mar-2010|
|Date of Acceptance||14-Jun-2010|
|Date of Web Publication||7-Oct-2010|
N H Doctor
Department of Surgical Gastroenterology, Jaslok Hospital and Research Centre, Mumbai
Source of Support: None, Conflict of Interest: None
The development of biliary casts is very rare, especially in non-liver transplant patients. The etiology of these casts is uncertain but several factors have been proposed which lead to bile stasis and/or gallbladder hypo-contractility and promote cast formation. Here, we report a 54-year-old male, with diabetes and ischemic heart disease, who presented with recurrent attacks of cholangitis. Magnetic resonance cholangiopancreatography revealed linear T1 hyperintense and T2 hypointense filling defects in the right and left hepatic ducts extending into the common hepatic duct, and a calculus in the lower common bile duct, raising a suspicion of worm in the biliary tree. In view of failed attempts at extraction on endoscopy, patient underwent surgery. At exploration, biliary casts and stones were extracted from the proximal and the second order bile ducts, with the help of intraoperative choledochoscopy and a bilio-enteric anastomosis was accomplished. Although endoscopic retrieval of the biliary cast can be employed as first-line management, surgery should be considered in case it fails.
Keywords: Biliary cast syndrome, intraoperative choledochoscopy, worm infestation
|How to cite this article:|
Jain P A, Gandhi V V, Desai P, Doctor N H. Recurrent cholangitis in the tropics: Worm or cast?. J Postgrad Med 2010;56:287-9
| :: Introduction|| |
Biliary cast syndrome (BCS) is reported after liver transplantation, but is rare in a non-liver transplant setting. Though it is associated with ischemic type injury, it may also occur with a normal arterial inflow. BCS has a multifactorial etiology and its management is difficult due to late detection. Here, we report a middle-aged diabetic patient who developed BCS and was managed surgically.
| :: Case Report|| |
A 54-year-old male presented with recurrent cholangitis with jaundice and pruritis of four months duration. He had lost significant weight. He was a diabetic and on oral hypoglycemics. He had undergone angioplasty two years back for ischemic heart disease (IHD). His biochemical profile revealed deranged liver function tests (Serum bilirubin - 8.3 mg/dl, direct bilirubin - 5.6 mg/dl, SGOT - 64 U/l, SGPT - 72 U/l, alkaline phosphatase - 355 U/l). Ultrasonography of abdomen (USG) showed mildly dilated intrahepatic biliary radicals (IHBR), distended gall bladder and sludge in the proximal common bile duct (CBD). Magnetic resonance cholangiopancreaticography (MRCP) showed T1 hyperintense and T2 hypointense linear filling defects in the right and left hepatic ducts extending into the common hepatic duct (CHD), and a calculus in the lower CBD [Figure 1] and [Figure 2]. In view of the MRCP findings suggestive of a worm in the CBD as the cause of cholangitis, endoscopic retrograde cholangiopancreaticography (ERCP) was attempted with a view to extraction. However, it failed on two occasions due to failure of cannulation. Thus, surgical drainage was planned for relief of cholangitis. At exploration, the CBD appeared dilated (8 mm) with a distended gallbladder. Choledochotomy revealed bile sludge and a biliary cast lying in the proximal CHD, branching into the right and left hepatic ducts with involvement of second-order ducts. The cast was extracted in Toto and intraoperative choledochoscopy was done to confirm complete bile duct clearance [Figure 3]. The biliary epithelium appeared normal. Cholecystectomy with Roux-en-y choledocho-jejunostomy was done. The postoperative period was uneventful. On follow-up for 12 months, patient remains asymptomatic.
|Figure 1 :Non-contrast T1 VIBE MRI images showing hyperintense cast-like filling defects in (a) left hepatic duct, (b) at the confluence (at hilum)|
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|Figure 2 :T2 HASTE AXIAL MRI images showing hypointense filling defects in (a) right hepatic duct (RHD) and left hepatic duct (LHD), (b) distal common bile duct (CBD) (c) RARE image showing filling defects in RHD and LHD (thin arrow) and CBD (thick arrow)|
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|Figure 3 :Specimen of biliary cast extending from CBD to the second-order ducts. RAD: Right anterior hepatic duct, RPD: Right posterior hepatic duct, LHD: Left hepatic duct, CHD: Common hepatic duct|
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| :: Discussion|| |
In patients with recurrent cholangitis, in the tropics, linear filling defects, seen on MRCP, are usually due to parasitic infestation of the biliary tree.  BCS is the presence of casts anywhere within the biliary system (intra- or extra-hepatic), causing obstruction with resultant biliary infection, stasis and cholangiopathy.  Though it is known to occur in liver transplant patients, it is seldom seen in the non-transplant setting.
The incidence, after liver transplantation, ranges from 3-18%.  The exact etiopathogenesis remains uncertain but it has been observed that anything which increases the viscosity and/or decreases the flow of bile, may precipitate cast formation. Some of the proposed mechanisms include acute cellular rejection, ischemic injury to the bile duct, biliary infection, fasting, prolonged ICU stay, parenteral nutrition, abdominal surgery, head injury, drugs (cyclosporine), foreign body (T tube, stents) and any cause of super saturation of bile with cholesterol.  In a transplant setting, prolonged cold  and warm  ischemia time have been described as predictive factors of BCS. Our patient had episodes of fasting (religious reasons) and probable ischemia due to atherosclerosis. The differences of all reported non-liver transplant patients with BCS underscore its multifactorial pathogenesis.
The sludge in BCS can be in the form of a cast, as in our case, hard like a calculus or it can be thick inspissated debris. Biochemical analysis of biliary casts in liver transplant patients has shown either unconjugated bilirubin (10-15%) in cases of delayed BCS (six months to 5 years post-operative.) or solid casts in the bile duct consisting of connective tissue and biliary duct epithelium.  However, the chemical composition of biliary casts in non-liver transplant patients is unknown. Unfortunately, in our case as well, biochemical analysis of the cast was not performed.
Because of its rarity, there is not much data on the value of the imaging modalities. Biliary sludge in the intrahepatic bile ducts can be easily missed on USG, if it is isoechoic. MRCP is the investigation of choice for biliary complications after liver transplantation and could similarly be used for patients with recurrent cholangitis in the non-transplant setting. In a series of 63 patients by Carlos Valls et al., the accuracy of MRCP for biliary complications was 88.9%. 
Due to considerable increase in the success rate of endoscopic procedures, ERCP is now considered as the treatment of choice in patients with infestation, where it has a high success rate.  However, if it fails, as in our patient, BCS should be considered due to the intrahepatic branching of the cast. Surgery is indicated when endoscopic management fails. Surgical repair involves bilioenteric anastomosis with extraction of biliary sludge from the larger ducts. Intraoperative choledochoscopy should be used to ensure complete removal of the casts.  Our patient had significant branching of the cast from the proximal CBD into intrahepatic second-order ducts.
There are few published reports on BCS in the non-liver transplant setting. D'Haens et al., described BCS in an elderly lady after emergency cholecystectomy.  Byrne et al., describe a patient with head injury, who developed BCS and was managed by liver transplant after failure of endoscopic and surgical interventions.  In another report by Gleeson et al., where they described two patients with BCS, one with hepatic infarction probably induced by antiphospholipid syndrome and the other patient had multifactorial etiology, similar to our case.  In 2008, Katsinelos et al., reported a case of BCS developing after cholecystectomy for gangrenous cholecystitis and managed with combined endoscopic treatment and ursodeoxycholic acid.  Although endoscopy has been described as a modality of treatment for BCS, there are reports of stent occlusion by a cast  and failure of endoscopy to retrieve the cast, probably due to intrahepatic branching of the cast, into secondary ducts. 
In conclusion, biliary cast formation should be suspected in patients with recurrent cholangitis, when linear densities are seen on MRCP. This is especially so in the tropics, where worm infestation is well described. However, endoscopic intervention, which would be the procedure of choice for worms, may not work for patients with BCS who are likely to need surgical intervention.
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[Figure 1], [Figure 2], [Figure 3]