budd chairi

1. By: Srijan Pradhan
PGR, WMW

2.  Name: Sharafat Ali
 Age: 18 years
 Sex: Male
 Marital status: Unmarried
 Address: Multan
 History obtained from patient himself
 Admitted on 7th July, 2014 via Emergency

3.  Blood in vomitus X 1 Day
 Black Tarry Stool X 1 Day

4.  My patient was in usual state of health 1 day prior to
presentation to our emergency. He then developed 3
episodes of vomiting which contained blood.
Vomiting was sudden onset, non projectile, each
vomitus contained 1-2 cups of blood (fresh with
clots).
 There is history of 1 episode of black tarry stool
which was 3-4 cups in amount, semi solid in
consistency, was foul smelling and contained no
fresh blood.

5.  There is no history of recurrent pain abdomen
and discomfort, dyspepsia, belching, persistent
vomiting, constipation, diarrhoea, anorexia,
weight loss or bleeding from any other site of
the body.
 There is no history of abdominal distention,
jaundice, history of blood transfusion during
childhood, or history of altered state of
consciousness

6.  There is no history of use of alcohol or any other
drugs including steroids or NSAIDs.
 No history of sexual contact or iv drug abuse.

7.  No history of chronic headache, fits, visual
disturbances, blackouts, abnormal behavior,
psychosis, numbness or tingling sensations.
 No history of exertional dyspnoea, chest pain,
body swelling, orthopnea, PND, peripheral
vascular disease.
 No history of cough ,dyspnoea, orthodexia,
pleurisy, wheezing or nasal discharge
 There is no history of dysuria, urgency,
hesitancy and no history of polyuria or
hematuria or renal colic or genitourinary ulcers.

8.  There is history of similar episode of blood
vomiting 6 years back. Was admitted at
Nishtar Hospital, Multan and was managed.
Upper GI endoscopy was done at that time.

9.  He was discharged from Nishtar Hospital on
Carvedilol, Lactulose and PPI which he took
for about 6 months and left medication on
his own.

10.  No history of similar illness in his family
 No history of congenital disorders
 No history of DM, HTN, IHD, Asthma or
tuberculosis.

11.  Low socioeconomic status
 Unmarried
 No formal education
 no history of use of any illicit substance

12.  No known allergy to any drugs

13.  Patient is working at brick kiln factory for last
7-8 years.

14.  Non cirrhotic portal hypertension
 Acid peptic disease
 Budd-Chiari Syndrome
 Gastric carcinoma
 Zollinger Ellison syndrome
 Decompensated Chronic Liver Disease
 Dieulafoy’s Lesions

15. General physical examination
 A young gentleman of good built sitting on
bed comfortably having cannula on left arm
and well oriented to TPP with following vitals:
 Pulse-78/minute regular
 BP-110/80
 T-Afebrile
 RR-14/min

16.  Pallor++
 Icterus-
 Edema-
 Lymhadenopathy: -
 Cyanosis-
 Bruises-
 rash-
 Thyroid not enlarged
 Petechiae –
 JVP -
 Spider naevi-
 Palmer erythema-
 Gynecomastia-
 Loss of body hair-
 Testicular atrophy-
 Leuconychia-
 Flapping tremor-
 Dupuytren
contracture-
 Parotid enlargement -

17.  Abdominal Examination-
 Inspecton- Shape of abdomen normal, moving
with repiration, peristalsis is not visible,
umbilicus central, no scar, stria or prominent
veins. Hernial orifices intact.
 Palpation- No rigidity or tenderness. Spleen is
palpable 1 finger below the left costal margin.
Liver not palpable
 Percussion- There is no dullness or fluid thrill.
 Ausultation- Bowel sounds 3 per minute of
normal intensity

18.  CHEST- Abdomino thoracic respiration, equal
expansion, trachea central. Normo vesicular breath
sounds bilaterally with no added sounds heard
 CVS: Apex beat in fifth intercostal space in mid
clavicular line
 1st and 2nd heart sound heard with normal intensity
and character.
 No added sounds or murmur heard
 CNS EXAMINATION:GCS 15/15……..No motor n
sensory abnormality

19.  Blood vomiting 3
episodes with previous
similar history 6 yrs
back.
 Black tarry stool.
 No history of
abdominal pain,abd
distension,weight loss
ASOC,nsaids intake,
steroid or any drug
intake
 PALLOR
 Splenomegaly
 No stigmata of
Decomp CLD
ON HISTORY ON EXAMINATIONS

20.  Non cirrhotic portal hypertension
 Budd-Chiari Syndrome
 Cirrhotic portal hypertension
 Gastric carcinoma

21.  CBC
 Hb -10.2gm%
 Tlc-4.3
 N-71%,L-25%,M-2%,E-
2%
 Platelets-1,76,000
 Anti HCV by ELISA –ve
 HBsAG -ve

22.  Bilirubin-0.9mg/dl
 ALT-214
 AST-103
 ALP-300
 ALB-4.0
 T.Protein -7
 UREA-24
 CR-0.7
 NA-137
 K-3.5
 RBS-95
LFTS RFTS

23.  PT-14 SEC
 PT CONTROL-14 SEC
 APTT-25SEC
 APTT CONTROL-25
SEC
 CHEST X-RAY-NORMAL
 ECG-NORMAL

24.  liver has slightly irregular margins with
normal parenchymal texture and measures
16 cm
 portal vein measures 11 cm
 spleen-16 cm enlarged
 ascites-minimal pelvic ascites

25.  three column of grade 3 esophageal varices
seen
 rest of examination wasn’t done due to
presence of food in stomach

26.  no flow in right and left hepatic veins with
absence of flow noted in intrahepatic part of
ivc
 flow is normal in suprahepatic and infra
hepatic part of ivc
 portal vein is 11mm in diameter with normal
centripetal flow and normal flow velocity.,no
collateral formations
 finding suggestive of budd-chiari syndrome

27.  BUDD- CHIARI SYNDROME

28.  Peptic ulcer(35-50%)
 Gastric erosion(10-20%)
 Esophagitis(10%)
 Variceal bleeding(2-9%)
 Mallory Weiss(5%)
 Vascular malformation (5%)
 Cancer of stomach and esophagus(2%)

30.  The Budd–Chiari syndrome is a
heterogeneous group of disorders
characterized by hepatic venous outflow
obstruction at the level of the hepatic
venules, the large hepatic veins, the inferior
vena cava, or the right atrium.

32.  Physical examination may reveal the
following:
 Jaundice
 Ascites
 Hepatomegaly
 Splenomegaly
 Ankle edema
 Stasis ulcerations
 Prominence of collateral veins

33.  Acute and subacute forms: Characterized by rapid
development of abdominal pain, ascites (which can cause
abdominal distention), hepatomegaly, jaundice, and renal
failure.
 Chronic form: Most common presentation; patients present
with progressive ascites; jaundice is absent; approximately
50% of patients also have renal impairment.splenomegaly and
esophago-gastric varices are seen
 Fulminant form: Uncommon presentation; fulminant or
subfulminant hepatic failure is present, along with ascites,
tender hepatomegaly, jaundice, and renal failure.

34.  Serum aspartate and alanine aminotransferase levels
may be more than five times the upper limit of the
normal range in the fulminant and acute forms of the
Budd–Chiari syndrome, whereas increases are smaller
in the subacute form.
 Serum alkaline phosphatase and bilirubin levels also
increase to a varying extent, along with a decrease in
serum albumin.
 The serum–ascitic fluid albumin gradient is high, with
the total protein level in the ascitic fluid usually more
than 2.5 g per deciliter, which is similar to the
composition of ascites in patients with cardiac
disease.

35.  Doppler ultrasonography of the liver has a
sensitivity and specificity of 85 percent or more,
is the technique of choice for initial investigation
when the Budd–Chiari syndrome is suspected
 Necrotic areas of the liver are better seen on
contrast-enhanced computed tomographic (CT)
scanning, which is used to delineate the venous
anatomy and the configuration of the liver when
a transjugular intrahepatic portosystemic shunt is
being considered

36.  However, MRI is better for visualizing the
whole length of the inferior vena cava and
may permit differentiation of the acute form
of the Budd–Chiari syndrome from the
subacute and chronic forms.
 MRA
 Liver Biopsy
 Echocardiography may be needed to rule out
tricuspid regurgitation, constrictive
pericarditis, or atrial myxoma

37.  Work up for all the acquired and congenital
disorders of hypercoagualtion.
◦ Protein C & S level
◦ Factor V leiden level
◦ Anti thrombin III level
◦ Prothrombin level
◦ Homocystene Level
◦ Flow Cytometry for PNH
◦ Bone Marrow for Myeloproliferative Disorders
◦ JAK2 Mutation
◦ ANA, Anti phospholipid antibody
◦ RBC mass and erythropoitin level (PRV)

38.  Therapy for patients with the Budd–Chiari
syndrome includes medical management and
the relief of hepatic venous outflow tract
obstruction in order to prevent necrosis, with
liver transplantation in selected patients,
especially those with fulminant hepatic failure

39.  Medical management of the Budd–Chiari
syndrome consists of efforts to control the
further development of ascites, the use of
anticoagulation therapy to prevent further
extension of the venous thrombosis, and the
treatment of detectable underlying causes

40.  Ascites
◦ Managed by restricting the patient's sodium intake
to 90 mmol per day and administering
spironolactone and furosemide to achieve a
negative sodium balance. Large-volume
paracentesis and intravenous infusions of albumin
are necessary when ascites is tense or refractory to
diuretic therapy.

41.  Anticoagulation
◦ The majority of studies continue to suggest lifelong
anticoagulation with the aim of preventing thrombus
extension and encouraging recanalization. In the setting
of IVC thrombus, spontaneous fibrinolysis occurs within
1 year in the majority of the patients treated with
warfarin. This practice is echoed by the 2009 AASLD
guidelines which recommend commencing
anticoagulation at diagnosis and continuing indefinitely
unless there is a contraindication. This is generally done
with the use of low-molecular-weight heparin followed
by warfarin when the patient is clinically stable. The
platelet count should be followed closely on
anticoagulation.

42.  Thrombolysis
◦ There are case reports of successful systemic
thrombolytic therapy for BCS, catheter directed and
a combination of both. Recombinant tissue
plasminogen activator, urokinase and streptokinase
have all been used.

43.  Radiological Intervention
◦ Balloon Dilatation
◦ Percutaneous transhepatic balloon angioplasty
(PTBA)

44.  Portosystemic Shunting
◦ TIPSS
◦ Surgical portocaval Shunts
 Liver Transplantation

46.  The overall 5 year survival rate is 50-90%
with treatment but less than 10% without
intervention.

47.  Older Age
 High Child Pug Score
 Encephalopathy
 Ascites
 High Bilirubin
 Prolonged PT
 Increased Creatinine
 Concomitant portal vein thrombosis
 Histologic features of acute liver disease
superimposed on chroic liver injury

48. THANK YOU