1. Budd chiari syndrome
Dr. Daya Kalsariya ( PG scholar 2nd yr)
Under the Guidance of Dr. Surendra A. Soni
(HOD & Professor)
2. Budd chiari syndrome name from the
British Physician – George budd &
Australian Pathologist – Hans chiari
3. Introduction
Defintion
It is a very rare condition.
Budd chiari syndrome is caused by obstruction
of the Hepatic venous blood flow that drain the
Liver can occur in hepatic vein, some times in
inferior vena cava.
5. Causes:-
A) Primary Budd chiari syndrome -
Thrombosis of hepatic vein.
B) Secondary Budd chiari syndrome –
Compression of hepatic vein by outside
structure.
(eg. Tumor)
6. A) Primary causes
1) Polycythemia Vera :
Bone Marrow Neoplasm in which bone marrow
make too many RBC. It may also result in over
production of Platelets and WBC. It is the main
cause of BCS.
Increase viscosity of blood → stasis of blood →
hyper - coagulability → thrombosis.
Polycythemia Vera is classified as a
Myeloproliferative disease. (Group of disease of
bone marrow in which excess cells are produce.)
7. Conti. Causes…
2) Pregnancy :
Pregnancy induced hypercoagulability →
physiologically adaptive mechanism to prevent
postpartum bleeding.
Pregnancy itself is a factor of
hypercoagulability.
During pregnancy changes occurring in clotting
factors,
Fibrinogen ↑ up to 3 or 4 times.
Thrombin ↑, Protein S ↓, Protein C ↓,
Anticoagulant ↓.
8. Conti. Causes…
3) Use of oral contraceptive:
Oral contraceptive pills contain – estrogen and
progestin.
High dose of estrogen can increase a risk for
blood clot.
Oral contraceptive pills are contraindicated in
hepatic tumor, hepatic adenoma, cirrhosis.
9. Conti. Causes…
4) Paroxysmal nocturnal hemoglobinuria (PNH):
It is a rare life threatening disease of blood
characterized by destruction of RBC by
autoimmune system.
High incidence of blood clot formation are
incompletely understood.
5) Hepatocellular carcinoma :
Blood clotting abnormalities – coagulopathy is a
condition either prolonged, excessive bleeding or
hyper coagulation.
Autoimmune disease.
10. Conti. Causes…
6) Lupus anticoagulant* :
Lupus anticoagulants (LAs) antibody in living
system cause an increase in inappropriate blood
clotting.
In vivo, this antibody interact with platelet
membrane phospholipid, increasing adhesion &
aggregation of platelets.
The term ‘Anticoagulant’ accurately describe its
function in vitro.
Anticoagulants are a type of antibody produced by your
body’s immune system. While most antibodies attack
diseasein body, Las attack healthy cells and cell protein.
11. Conti. Causes…
Aspergillosis
It is a caused by fungal infection in compromised
immune system.
eg. Those undergoing bone marrow transplantation.
Bachet’s disease
It is Autoimmune disease.
Inflammation of blood vessels leads to narrowing or
blockage of vessel.
12. Conti. Causes…
Budd chiari syndrome is also seen in hepatic TB,
congenital venous web and occasionally in inferior vena
cava stenosis.
Genetic tendency - include protein C, protein S
deficiency, hereditary anti thrombin deficiency.
Non genetic – hormonal contraception, pregnancy,
trauma, bachet’s disease, Anti-phospholipid antibody
(APA).
APA – Autoimmune, hyper coagulable state caused by
anti phopholipid antibody. It has formation of clot and
develop pregnancy related complication eg.
Miscarriage.
13. Pathophysiology
Thrombosis in hepatic vein
↓
Obstruction in blood flow from liver
↓
Backward flow of blood in liver
↓
Increase pressure in liver
(Portal hypertension)
↓
Centrilobular necrosis and peripheral fatty changes
(increase central pressure lead to compress
peripheral part)
14. Conti. Pathophysiology...
Portal hypertension
↓ ↓
Splenomegaly due to congestion Extravasation of fluid and
because of portal hypertension. accumulation of fluid in
peritoneal cavity.
(Ascites)
15. Sign & Symptoms
Acute Syndrome present with
Severe upper Abd. Pain
Yellow discoloration of skin and eye
Liver enlargement
Caudate lobe hypertrophy
Splenomegaly,
Ascites
Caput medusae
Esophageal varices
Umbilical varices
Rectal varices
Elevated liver enzyme level
Severe Lactoacidosis
16. Kidney failure
Majority of patient have a slower onset of Budd-
chiari syndrome and this can be painless.
Complication of BCS:
Hepatic encephalopathy
Variceal hemorrhage
Hepatorenal syndrome
17. Investigation
USG of Abdomen – Stenosis, Collateral, Thrombosis
Angiography
CT scan and MRI
Liver biopsy – non specific but some times necessary
to differentiate between Bud chiari syndrome &
other cause of hepatomegaly, Ascites.
Liver enzyme test – to know the hepatic tissue
necrosis.
Endoscopy – to detect varices
Hematology - BT,CT,PT
18. Prognosis
Survival depend on cause.
Incase of Myeloproliferative disorder
progress to acute leukemia.
Bad prognosis in – Ascites,
encephalopathy, elevated prothrombin
time.
19. Treatment
Sodium restrict diet,
Diuretic,
Anticoagulant – Heparin, warfarin.
Urokinase (enzyme) – Thrombolytic. Isolated
from human urine and also present in blood
and tissue.
Symptomatic management
Milder form of Budd chiari treated by surgical
shunt to divert blood flow around the
obstruction.
20. Conti. Treatment…
Angioplasty to wide narrow and obstructed
vein.
Liver transplantation – effective treatment,
failure of shunt & progression of cirrhosis that
reduce the life expectancy.
Long term survival after transplantation range
about 69 – 87%
21. Complication of Liver transplantation
Arterial or venous thrombosis
Bleeding
Up to 10% of patients may have
recurrence of Budd chiari syndrome.
24. Causes of Udara Roga
Ati Ushna, Vedavidharana,
Ati Lavana, Srotodushti,
Ati Kshara, Sankshobha,
Ati Vidahi Anna Sevana, Atipurana,
Ati Amla Rasa sevana, Arsha, Bal, Shakruta Avarodha,
Mithya Sansarjana Krama, Antra Sphutana & Bhedana,
Ruksha, Viruddha, Ashuchi bhojana, Atisanchita Dosha,
Karshana due to Pleeha, Arsha,- Papa Karma,
-Grahani Roga, Mandagni is main cause of-
Panchkarma Vibhrama, Udara Roga.
Klishta roga Apratikara,
25. Purvarupa of Udar roga
राजीजन्म वलीनाश इति तलङ्गं भतवष्यिाम्||१९||
Normal sign of absence of normal muscles wrinkles and
presence of engorged veins on abdominal wall.
Samprapti of Pleehodar
शोतििं वा रसातिभ्यो तववृद्धं िं तववर्धयेि्||३६||(Ch.Chi13)
Increase Rakta from Rasa Dhatu leads to Pleeha
Vrudhhi. In BCS Srotorodha in hepatic veins
(Thrombosis) leads to above
phenomenon.
27. Lakshana of Pleehodara / Yakritudara
Daurbalya, Angamarda, Mrudujwara,
Arochaka, Chhardi, Anaha,
Avipaka, Murchha, Agninasha,
Varcha, Mutra nigraha, Angasada, Karshya,
Tamah Pravesha, Kasa, Asya Vairasya,
Pipasa, Shwasa, Parvabheda,
Udara Shoola,
Udara pradeshe Aruna, Vivarna, Nila, Harita, Haridra Raji Udbhav.
But, if Yakrita Vrudhhi which is located at right side of the
abdomen with above sign and symptoms is known as Yakritudara.
Pleehodara and Yakritudara both have same Hetu, lakshana and
Aushadha.
28. Interpretation
Ancient Ayu.description of Udara Roga mentioned
by Acharya Charaka covers variety of G.I.
pathologies viz all Hepatitis, Pancreatitis, Tumors,
Cirrhosis etc.
BCS is also to be understood differentiating
the sign & symptoms and investigations outcome.
29. Chikitsa of Pleehodar / Yakritudara
स्नेहं स्वेिं तवरेकं च तनरूहमनुवासनम्|
समीक्ष्य कारयेद्बाहौ वामे वा व्यर्येि् तसराम्||७७||
षट्पलं पाययेि् सतपधिः तपप्पलीवाध प्रयोजयेि्|
सगुडामभयां वाऽतप क्षाराररष्टगिांस्िथा||७८|| (Ch.chi.13)
30. Chikitsa of Pleehodara /Yakritudara
Snehana, Shatapala Ghrita,
Swedana, Pippali,
Virechana, Gudaharitaki,
Niruha Basti, Kshara,
Anuvasana Basti, Arishta Prayoga.
Vama Hasta Siravedha,
Niruha Basti, Kshara and Arishta prayoga is most
suitable in BCS as per basic pathology.
32. Samprapti of Jalodara
Snehapana Paschhata,
Mandagni,
Durbala Purusha,
Ati krusha purusha
Atyambupana Agninasha
Srotorodha
Vata, Kapha
Dosha
vrudhhi
Ambu
Vrudhhi in
Udara
Jalodara
33. Lakshana of Jalodar
िस्य रूपाति-
अनन्नकाङ्क्षातपपासागुिस्रावशूलश्वासकासिौबधल्यातन, अतप चोिरंना
नाविधरातजतसरासन्ििमुिकपूिधदृतिक्षोभसंस्पशशं
भवति, एििुिकोिरतमति तवद्याि्||४७|| (Ch.chi.13)
Bhojne Ashradhha, Shwasa,
Pipasa, Kasa,
Gudastrava, Daurbalyanubhuti,
Udarashoola,
Udara pradeshe Vividh Varna Rajiudbhava,
Sparshne Udaka Purna Druti Sparsha and Kshobha.
In BCS ascites developed because of thrombosis & portal
hypertension.
34. Chikitsa of Jalodar
अपां िोषहराण्यािौ प्रिद्यािुिकोिरे||९३||
मूत्रयुक्तातन िीक्ष्िातन तवतवर्क्षारवतन्ि च|
िीपनीयिः कफघ्नश्च िमाहाररुपाचरेि्||९४||
द्रवेभ्यश्चोिकातिभ्यो तनय्छेिनुपूवधशिः|९५| (Ch.chi.13)
Mutra Yukta Vividha Kshara & Aushadha Yoga,
Dipaniya Aushadha,
Kaphaghna Ahara Sevana,
Restriction of Drava Sevana.
These all therapeutic measure applicable to BCS.
35. In Grathita Rakta Paravata Vit is used with the Madhu for Lehana.
( Ch. Chi. 4/72)
Properties of Kshara : Tridoshghna, Shukla, Saumya,
Ropana, Shoshana, Stambhana, Lekhana,
Shodhana,
Dahana, Pachana, Darana, Vilayana,
Agneyaguna, Ushna virya, Tikshna, Katu Rasa,
Krumi, Kapha, Ama, Visha, Medavrushhi –
Nashaka. ( Su.Su. 11/5)
Katu Rasa is used for Bhedana of Shonita Sanghata.