Portal hypertensive biliopathy is a very rare and deadly situation if not managed properly. A team of Gastrosurgeon, Gastro physicians, and Interventional radiologists should be involved before making any decision in this kind of cases.

1. Dr. Keyur Bhatt
MS, FAIS, MRCS (UK), FACS (USA)
ASIA BOOK & INDIA BOOK Record holder for “Grand Master of laparoscopic cholecystectomy”
LIMCA BOOK & INDIA BOOK Record holder “longest foreign body removal from stomach”

2. An Interesting case

3. Case history
• A 37 years old female known case of ?EHPVO
with portal biliopathy

4. Significant Past History
• Upper GI scopy was done multiple times for
hematemesis,
• Finding showed grade 3 varices hence PHT-EVL
and glue were done multiple times.
– On 09/12/14
– On 17/01/15
– On 06/04/15.
– On 17/04/2017
– On 19/03/2018
– On 19/05/2018
No deterioration
No ascites
No jaundice
No encephalopathy
low CBC – All the lines

5. CT scan abdomen - 19/05/2018
• EHPVO with portal hypertension
• Chronic complete occlusion of splenoportal confluence
and entire portal vein with resultant multiple varices
and marked splenomegaly,
• Atrophic left lateral segment and prominent caudate
lobe of liver with mild nodular outline of rest of the
liver.
• Compression effect over LHD, RHD & their confluence
by varices with resultant moderate bilobar IHBR.
• Multiple small calculi within segment V-VI IHBR & left
hepatic duct, small gallbladder calculi.

6. Diagnosis
• Medical gastroenterologist & Surgical Gastroenterologist
consultation was done and diagnosed as
• ? NCPF ? EHPVO.
• Plan
– CBC/LFT and liver biopsy (to rule out CLD)
– If liver biopsy normal  then shunt surgery.
• LFT report - Total bilirubin-0.8, SGPT-44, ALP-158.
• Surprisingly - after this LFT report patient was advised for
ERCP, stenting followed by liver biopsy (? in view of CBD /
CHD Stones!!)

7. ERCP and stenting was done on
31/5/2018
– 7 fr stenting was not possible probably due to
stricture, so 5 fr stent was kept as a bail out.
(practically inferior for biliary drainage)
– Significant bleeding was encountered during ERCP

8. Liver biopsy
• Showed mild widening and fibrosis in few
portal tract, no cirrhosis.

9. What happen next?

10. Patient worsened – as expected
• Patient developed
– Jaundice
– High grade fever
– Mild ascites
– Abdominal pain
– Abdominal distension
– Hypotension
– Sepsis
• Cholangitis with sepsis
– Nothing was there before procedure

11. Now
• ERCP failed  patient had cholangitis
(bilirubin level increased to 7.9, SGPT-11,
Persistent fever, not responding to antibiotics)
• What next ? – what's the only option?
• PTBD was done on 11/06/2018.

12. Post PTBD
– Persistent abdominal pain
– Continues High grade fever
– Decreased oral intake
– Abdominal distension
– Rising Icterus to 11.5mg/dl

13. Now what’s the Plan?
• What could be the possibility?
• What next could be done?
• What is the issue?
• Is the source controlled?

14. MRI

15. MRCP

16. MRCP – was done on 13/06/18
– Showed – Seg. VIII  Cholangiolytic abscess. 2x 3cm
– Multiple small and discrete rounded fillings defects in
right anterior > posterior ducts  sludge / soft
calculi.
– Left hepatic duct - terminal segment could not be
visualized,
– Left IHBR, mildly dilated segment IV duct.
– Moderately dilated right anterior IHBR, severe
stenosis of terminal segment V duct
– Common right anterior duct, Right posterior duct -
terminal segment could not be visualized.

17. Patient was received at SIDS Hospital

18. TIPS

19. If we look back what happened?

20. One must not disturb the snake when
he is sleeping!!

21. When your first step is wrong then
entire game is changed
• In this case first wrong step was unindicted
ERCP
– Which almost always leads to complications.

22. When we received the patient
• Patient had
– Gross abdominal distension, with mild ascitis
– Jaundice,
– Hypotension,
– High grade fever,
– PTBD in situ – not draining,
– Stent in situ without pneumobilia – not draining
• On
– Inj. Meropenum, Tigicyclin.

23. First step
• Antibiotics were changed according to blood
culture which showed Psudomonas growth
• Colistin & Amikacin were started.

24. What next for undrained biliary system
• Repeat PTBD in other lobes?
• How many?
• Almost all ducts were separate on MRI /MRCP
Interventional radiologist was consulted
• Denied for so many multiple PTBD Especially in
presence of Ascities and worsening sepsis
(Patient on inotropic supports)

25. Out of the box plan
• Streptokinase was given through PTBD
• Strikingly  PTBD started draining and daily
300 to 400 ml bile started coming.
• S. Bilirubin decreased to 6.1mg/dl

26. High grade fever was still persistent
– Investigation showed No pneumobilia (left as well as
right duct
( Bilirubin decreased to 5.6 mg/dl, Higher antibiotic
were on, PTBD was draining 300 to 400 ml greenish
(infected) bile daily)
– Probably the left ducts were still not drained.
Repeat ERCP and stenting was done on 19/06/2018
* A 10 Fr Cotton and Leung stent was placed into the left
duct and a 7 Fr in the right duct for drainage after
removal of 5 Fr stent, without any bleed.

27. Finally we could Discharge the patient
on POD 6 of ERCP
Afebrile
Bilirubin level
decreased (5.3)
With draining PTBD

28. What next ? What is the definite plan ?
• Bilirubin-slowly decreased to 5.3 2.3
• 2.5 month patient was asymptomatic
• PTBD Got pulled out accidently.
• Planned for shunt surgery.
• CT scan showed
– Splenic vein-18 mm, left renal vein-13 mm
– Both stents were patent-pneumobilia in both duct,
left as well as right.
– Additionally she had multiple peri hilar flow related
splenic artery aneurisms.

29. CECT

30. Surgery
• PSRS was done on 21/09/2018.
• Bilirubin level was 0.91 at time of surgery
• Post op on Tab Dabigatran 150 mg od and Tab
UDCA 300mg 1 BD Was given

31. PSRS

32. Following surgery
• She had fever again after 1 month (bilirubin-
2.3)
• ERCP and stenting was done on 13/11/2018.
– Additional 10 fr DPT was kept
• She was discharged after couple of days
without fever or sepsis.

33. Again she was admitted after 1 month
• On 20/12/2018
– Fever, bilious vomiting, altered sensorium, hypotension
– Total bilirubin-4.83, TC-33800.
– Severe sepsis with septic shock (Nor adrenalin, vasopressin support)
– Severe Septic Myocarditis (EF dropped to -25-30 %)
• ERCP + Stent Exchange was done.
– Partially clogged stents, The hilum was patent and right anterior duct
opened low with ?Right posterior opening from left
– The duct was swept with a Balloon extractor and lot of sludge, Pus and
stone were removed.
• Bilirubin decreased to 2.29 on 5/01/2019.
• Patient gradually improved

34. After 10 days (16.1.2019)
• Again got admitted with high grade fever
• Deranged LFT (1.4/27/323)
• Next day another stent exchange was done,
Mild bleeding happened so no cholangiogram
was done, two stents placed
• Discharged on next day in Afebrile status.

36. 1 month later re-admitted
• Fever with chills and rigors again
• LFT (0.89/15/110)
• PS showed P. Falci.
• ERCP Again was done on 22.2.2019
• Multiple stones could be retrieved
• Final cholangiogram was done which did not
showed any stones in situ
• Two DPT Kept (10Fr).
• Discharged on 26.02.2019

38. Cholangiogram

39. Next ??
• Asymptomatic for 3 months
• CECT was done for final Surgery - HJ
• Showed patent shunt, B/L Pneumobilia with
stents in situ, No residual abscesses.

40. CT SCAN

41. HJ

42. Final picture

44. Portal biliopathy
• Portal biliopathy is relatively rare.
• The development of symptomatic portal
biliopathy is a difficult problem to treat.
• There is no standard treatment
recommendation available for portal
biliopathy in literature.
• There are very little reports on long term
outcome analysis following treatment for
portal biliopathy.

45. Pathogenesis
• In most of the pts, the acute inciting event
leading to EHPVO often goes unrecognized and
thrombus gradually becomes organized.
Multiple tortuous hepatopetal collaterals, which
develop around and inside the thrombus within
the PV, known as cavernoma, appear within a
span of 6-20 days following PV flow interruption.
•

46. Pathophysiology
• The portal cavernoma bypasses the obstructed
portal vein and thus a thrombosed portal vein
turns into a fibrotic cord. The network is seen
around structures near the obstructed portal vein
such as the bile duct, gall bladder, pancreas,
gastric antrum, and duodenum.
• The bile duct may be difficult to locate within the
network of collaterals on abdominal
ultrasonography.

47. Three theories have been described
• The first one suggests that the pliable CBD is compressed
by the large collateral veins running along the wall of the
CBD in patients with portal hypertension which represents
external compression. This theory explains the reversal of
biliary obstruction following portal decompressive surgery
as demonstrated in various studies.
• The second theory is that long standing portal venous
thrombosis can cause sclerosis of the veins draining the bile
duct. This can affect and damage the capillaries and
arterioles. This in turn can lead on to ischaemic strictures of
the bile duct.
• Infection or cholangitis is speculated as the third reason for
biliary strictures. Cholangitis can lead on to inflammation
and fibrosis leading to biliary strictures.

48. Types

49. Types

50. Other studies

52. THANKS