Diagnostic approach in a patient with thrombosis of the portal ......2017/04/22  · Diagnostic...

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Pierre-Emmanuel RAUTOUInserm U970, PARCC@HEGP, Paris

Hepatology, Hôpital Beaujon, Clichy, Francepierre-emmanuel.rautou@inserm.fr

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