Pierre-Emmanuel RAUTOUInserm U970, PARCC@HEGP, Paris
Hepatology, Hôpital Beaujon, Clichy, [email protected]
Diagnostic approach in a patient with
thrombosis of the portal venous axis
Nothing to disclose
Diagnostic approach in a patient with thrombosis
of the portal venous axis (PVT)
• Be sure of the diagnosis
• Is there a complication?
• Is there a risk factor for PVT?
Diagnostic approach in a patient with thrombosis
of the portal venous axis (PVT)
• Be sure of the diagnosis
• Is there a complication?
• Is there a risk factor for PVT?
Uncomplicated Acute PVT
Abdominal PainSystem. Inflam.
Intestinal Ischemia
PVT: symptoms
Pain, AscitesOrgan failure Plessier, Hepatology 2009
Chronic PVT
BleedingEncephalopathy
Cholangiopathy
PVT: symptoms
PVT: diagnosis
• Doppler-ultrasonography:
• Contrast enhanced CT-scan ++
Berzigotti A, Nat Rev Gastroenterol Hepatol. 2014
- Absence of flow within the portal vein
- Hyperechoic thrombus in the portal lumen
- Diagnosis
- Extension
- Complication
- Local factor
No enhancement
Acute PVT diagnosisPortal phase
No collaterals Plessier, J Hepatol 2012
Plessier, Hepatology 2009
Chronic PVT diagnosis
Diagnostic approach in a patient with thrombosis
of the portal venous axis (PVT)
• Be sure of the diagnosis
• Is there a complication?
• Is there a risk factor for PVT?
Intestinal ischemia
Portal cholangiopathy
Intestinal necrosis
Intestinal ischemiaUncomplicated
Acute PVT
Abdominal PainSIRS
Intestinal Ischemia
Risk factors for intestinal necrosis
Elkrief, Liver Int 2013; Nuzzo, Am J Gastro 2017
No resection
N=46
Resection
N=11P =
General prothrombotic factor 44% 44% 0.91
Local factor 20% 30% 0.08
Diabetes 9% 27% 0.02
Superior Mes. Vein thrombosis
Identification of intestinal necrosis
Acute intestinal ischemia (n=67)
Transmural necrosis
(n=23)No transmural necrosis
(n=44)
Nuzzo, Am J Gastro 2017
arterial 2/3
venous 1/3
Identification of intestinal necrosis
Nuzzo, Am J Gastro 2017
• Organ failure
• Serum lactate > 2 mmol/L
• Bowel loop dilation
Necro
sis
fre
e s
urv
iva
l(%
) 0 factor
1 factor
2 factors
3 factors
Follow-up (days )
Elkrief, Liver Int 2013
Intestinal necrosis
Specificities of venous intestinal necrosis
Thrombosis of second order radicles
Ascites
Thickened bowel wall
Portal cholangiopathy in chronic PVT
Llop E, Gut. 2011
Strictures w/o
dilationStrictures + dilationNormal or
parietal
irregularities Risk of complication
particularly if ALK or
GGT
26% 15% 58%
Diagnostic approach in a patient with thrombosis
of the portal venous axis (PVT)
• Be sure of the diagnosis
• Is there a complication?
• Is there a risk factor for PVT?
Is there a liver disease?
Transient elastography
Baveno VI
Seijo, Dig Liv Dis 2012
PSD CirrhosisPrimary
PVT
Fib
roscan
(kP
a)
Is there a risk factor for PVT?
10
PSD, portosinusoidal disease
Is there a liver disease?
Transient elastography
< 10 kPa ≥ 10 kPa
Consider
liver biopsy
Normal liver
blood tests
Abnormal liver
blood tests
Turon
2014
(n=140)
Bureau
2016
(n=79)
Poisson
2017
(n=213)
Local factor 35% 16% 8%
General factor 37% 34% 63%
Idiopathic 37% 49% 34%
Turon, J Hepatol 2014; Bureau, J Hepatol 2016; Poisson, J Hepatol 2017
• Abdominal surgery
• Abdominal infection
• Abdominal trauma
• Pancreatitis / IBD
Extrahepatic risk factors for PVT
Turon
2014
(n=140)
Bureau
2016
(n=79)
Poisson
2017
(n=213)
Local factor 35% 16% 8%
General factor 37% 34% 63%
Idiopathic 37% 49% 34%
Turon, J Hepatol 2014; Bureau, J Hepatol 2016; Poisson, J Hepatol 2017
Extrahepatic risk factors for PVT
Barcelona + Toulouse +
Clichy (n=432)
Myeloproliferative neoplasms % 21%
Factor II Leiden 6%
Factor V Leiden 3%
Low prot. C level 5%
Low prot. S level 6%
Antiphospholipid syndrome % 6%
Others (PNH, Behcet, IBD, …) % 5%
Turon, J Hepatol 2014; Bureau, J Hepatol 2016; Poisson, J Hepatol 2017
Extrahepatic risk factors for PVT
20-30%
55-65%
3%
Absent
CALR
2%
Spleen height 16 cm
and Platelets > 200x109/L
No mutation
Bone marrow biopsy
Spleen height < 16 cm
or Platelets ≤ 200x109/L
Consider bone
marrow biopsy
Present
MPN
No
MPN
Patients with splanchnic vein thrombosis
mutated
Poisson, J Hepatol 2017
Collab: F Turon & JC Garcia-Pagan
JAK2V617F
Turon
2014
(n=140)
Bureau
2016
(n=79)
Poisson
2017
(n=213)
Local factor 35% 16% 8%
General factor 37% 34% 63%
Idiopathic 37% 49% 34%
Turon, J Hepatol 2014; Bureau, J Hepatol 2016; Poisson, J Hepatol 2017
Risk factors for thrombosis in PVT
Ove
rwe
ight(%
)
0
10
20
30
40
50
60
70
80
90
Waist circumference was the strongest parameter
independently associated with idiopathic PVT
Secondary
PVT
General
population
“Idiopathic”
PVT
Overweight: a risk factor for PVT**
**
Bureau, J Hepatol 2016
Conclusion
• Contrast enhanced CT-scan:
• Rule out cirrhosis: Fibroscan (± biopsy)
• Work-up for causes:
Diagnosis
Extension
Complication
Local factor
Local factor
General factors (MPN)
Obesity
Juan-Carlos Garcia-Pagan
Andréa de Gottardi
French network for
vascular liver diseases
Dominique Valla
Aurélie Plessier
Audrey Payancé
Odile Goria
Lille
AmiensRouen
Caen
Reims
Paris Nancy
Strasbourg
BesançonDijonOrléansTours
Angers
Rennes
Brest
Poitiers
LimogesClermont-Ferrand
Lyon
Grenoble
Nice
Marseille
Montpellier
Toulouse
Bordeaux
Pr Eric Nguyen-Khac
Pr Dominique Larrey
Pr Paul Cales
Pr Jean-Pierre Bronowicki
et Dr. Hélène Barraud
Pr Victor de LédinghenPr Albert Tran et
Dr Rodolphe Anty
Dr Xavier Causse
Pr Armand AbergelPr Christine Silvain
Dr Anne Minello et Dr
Marianne Latournerie
Dr Alexandra Heurgue
Pr Jean Pierre Zarski
Pr Dominique Guyader
Pr Philippe Mathurin
Dr Odile Goria
Dr Marilyn Debette Gratien
Pr Michel Doffoel
Pr Danielle Botta-FridlundPr Christophe Bureau
Pr. Vincent di Martino
Pr T. Dao & Dr I Ollivier
Dr Louis D’Alteroche
Thank you
MPN treatment and BCS/PVT outcome
Budd-Chiari syndrome
(n=46)
Portal vein thrombosis
(n=63)
Major events: vascular events (new thrombosis, hemorrhage)
or liver-related events (refractory ascites, hepato renal
syndrome, encephalopathy and transplantation)
Major events
(N=33 )
Major events
(N=26 )
Independent prognostic factor: absence of
cytoreductive therapy after BCS/PVT diagnosis
Chagneau-Derrode, AASLD 2013
