Keywords
Extra-hepatic portal venous obstruction
-
paracholedochal plexus
-
portal biliopathy
-
stent
INTRODUCTION
Extrahepatic portal venous obstruction (EHPVO) is characterized by a chronic blockage
of portal vein, usually presented in childhood with signs of portal hypertension (PHT)
and its complications.[1] The diagnosis of EHPVO is mainly clinical – features of PHT without any evidence
of liver dysfunction and presence of portal cavernoma on Doppler ultrasound (US).
Portal biliopathy is seen in 80%–100% of patients with EHPVO, however, remains asymptomatic
in 62%–95% patients.[2] The patient usually present with features of jaundice with cholestatic features,
biliary colic, and recurrent cholangitis, presence of bile duct stones and abnormal
liver function test.
Endoscopic retrograde cholangiopancreatography (ERCP) with stenting is usually required
in case with obstructive jaundice and cholangitis.[2] ERCP and stent-induced bleeding from venous plexus in portal hypertensive biliopathy
(PHB) is extremely rare.[3],[4] Here, we are discussing a case of EHPVO with symptomatic PHB who underwent biliary
stenting which led to torrential bleeding from paracholedochal collateral and its
management.
CASE REPORT
A 31-year-old male patient presented with painful progressive jaundice from the last
1 month associated with intermittent episodes of high-grade fever. He was icteric
with moderate splenomegaly and tenderness in the right hypochondrium on abdominal
examination. He was diagnosed as a case of EHPVO ~15 years back and on secondary prophylaxis
with beta blockers for variceal bleeding. He also underwent open cholecystectomy 4
months back for symptomatic cholelithiasis. With the background of EHPVO and current
clinical scenario; he was worked up for portal biliopathy.
Biochemical investigations were suggestive of raised leukocyte count (19,700/mm3) and deranged liver function test: bilirubin (27.5 mg/dl) with direct component (13.57
mg/dl); alkaline phosphatase (311 IU/L). Ultrasound abdomen was suggestive of portal
cavernoma and dilated common bile duct (CBD) with echogenic sludge within. Magnetic
resonance imaging abdomen with magnetic resonance cholangiopancreatography reported
stricture at lower end CBD with proximal dilation of CBD (21 mm) and intrahepatic
biliary radicals (IHBR) with few soft calculi (2–4 mm). Upper gastrointestinal (GI)
endoscopy revealed eradicated esophageal varices and no gastric varices. With these
findings, he underwent ERCP under sedation. Selective CBD cannulation done and cholangiogram
revealed dilated IHBR with grossly dilated CBD except narrowed distal segment. Endoscopic
papillotomy was done using pull type papillotome, and minor bleed was seen which resolved
spontaneously. Stones were extracted with the help of extraction balloon and over
the wire and 10 fr × 10 cm straight plastic stent was placed. Immediately, a gush
of blood was noted from ampulla into the duodenum with stent in situ. The procedure was abandoned as patient developed hypotension.
The patient was shifted to the Intensive Care Unit (ICU) for resuscitation, and nasogastric
tube was placed and intravenous fluids, inotropes, and terlipressin were started.
Injectable antibiotics with injection tranexamic acid were also supplemented. Two
units of packed red blood cells were transfused over 6 h.
After maintaining hemodynamic stability, contrast-enhanced computed tomography (CT)
abdomen with angiogram and portal venography was performed. Noncontrast CT abdomen
[Figure 1a] showed contrast within dilated intrahepatic biliary radicles which was injected
during ERCP. Biliary stent was away from hepatic artery branches with no active extravasation
or pseudoaneurysm seen [Figure 1b].
Figure 1: (a)Noncontrast computed tomography abdomen in coronal plane showing contrast filled
dilated intrahepatic biliary radicles (small black arrow). The lower end of common
bile duct stent (large black arrow) is also seen; (b)Coronal maximum intensity projection
in arterial phase showing biliary stent (white arrow) away from hepatic artery branches
with no active extravasation or pseudoaneurysm seen
Portal venous phase [Figure 2a and b] showing multiple paracholedochal collaterals with dilated CBD. Tip of the biliary
stent was misplaced and seen within one of the large paracholedochal collateral. Interventional
radiologist and surgical gastroenterologist opinion sought. Meanwhile, inotropic support
was gradually tapered and terlipressin was continued for 72 h.
Figure 2: (a and b) contrast-enhanced computed tomography abdomen during portal venous phase
in coronal plane showing multiple paracholedochal collaterals (white arrow) with dilated
common bile duct. Tip of biliary stent (black arrow) is misplaced and seen within
one of the larger paracholedochal collateral
Percutaneous transhepatic biliary drainage (PTBD) was done to decompress biliary system
and with the fear of displacing hematoma around collateral puncture site; external
catheter was placed at hilum [Figure 3]. He responded to treatment well with the stabilization of hemoglobin and decrement
in bilirubin and improvement in liver function test.
Figure 3: Cholangiogram taken through an external biliary catheter (black arrow) placed from
the left side biliary system into the right side showing mild dilatation of intrahepatic
biliary radicals and common bile duct. Biliary stent seen within common bile duct
in lower part and misplaced out of common bile duct (white arrow) in the upper part
After 7 days, ERCP was repeated, and in situ stent was removed, and 8 fr × 10 cm double pigtail stent was placed into the right
ductal system. The procedure was uneventful. Later, PTBD was clamped and with continued
decreasing level of bilirubin; it was removed. The patient was subsequently discharged
with an advice of follow-up after 14 days. After a follow-up of 3 months, the patient
was symptomatically improved with no further episodes of GI bleed and decrement in
the level of bilirubin.
DISCUSSION
Portal biliopathy is reported in 80%–100% cases of EHPVO; however, it manifests with
symptoms of biliary obstruction in only 5%–14% of cases.[2] The mechanisms postulated to cause peribiliary plexus are dilated epicholedochal
and paracholedochal venous plexuses causing compression on the bile ducts; the formation
of new vessels and connective tissue resulting in fibrosis around the ducts and ischemic
changes in bile duct.[5] These processes may lead to stasis, stricture, cholangitis, and choledocholithiasis.
Symptomatic biliopathy is a definite indication for intervention–endoscopic or surgical.
Endoscopic interventions include sphincterotomy, stone extraction, biliary stenting,
stricture dilatation, and mechanical lithotripsy.[6] Portosystemic shunt is done primarily to release pressure on CBD and if symptoms
persist, endotherapy is utilized. However, surgical biliary drainage either choledocho-duodenostomy
or hepaticojejunostomy may be needed for persistent stricture.[7] In our case, apart from EHPVO, the patient also had splenic vein and superior mesenteric
vein thrombosis with no shuntable varix and in view of cholangitis, we had to rely
on endoscopic management primarily.
The overall success rate of endotherapy in portal biliopathy was 70%–100%.[2],[8] Various complications such as hemobilia and pancreatitis have been reported with
ERCP and stenting in portal biliopathy and managed conservatively.[8] The bleeding has been encountered in portal biliopathy while performing ERCP either
during sphincterotomy, balloon dilatation, or while removing stent.[3],[4] We encountered bleeding after stent insertion which has not been reported before.
Usually, ERCP- and stent-induced bleeding do not cause significant hemodynamic changes
and stop spontaneously with conservative management. In this case, the patient developed
hemorrhagic shock, managed with inotropes, splanchnic vasoconstrictors, and blood
transfusion. In view of cholestatic liver with associated coagulopathy, risk of massive
bleeding due to the formation of bilio-portal collateral fistula on CT scan and also
risk of bilhemia due to associated obstructive jaundice with high level of jaundice,
stent was not removed initially. PTBD was performed and that helped in following ways:
decrease the biliary pressure, decrease the level of bilirubin, and improve cholestasis
and coagulopathy. Similar approach by endoscopic nasobiliary drainage (ENBD) has been
utilized in the past for bridging period of acute crisis.[9] Later, the culprit stent was removed and replaced with another plastic stent.
To the best of our knowledge, this is the first incidence of stent-induced bilioportal
collateral fistula leading to massive hemobilia. The mode of injury was different
in our case although the culprit lesion was same, i.e., paracholedochal varices. This
case highlights various important aspects in managing such patients: (a) utmost care
while performing ERC stenting in symptomatic PHB without portosystemic shunt; (b)
importance of CT portal venography and endoscopic ultrasound to delineate the biliary
plexus collaterals in such patients; (c) biliary drainage with either PTBD or ENBD
to improve cholestasis if hemobilia occurs; (d) managing such patients in ICU with
multidisciplinary approach.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form the patient(s) has/have given his/her/their consent for his/her/their
images and other clinical information to be reported in the journal. The patients
understand that their names and initials will not be published and due efforts will
be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
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