AM REPORT. The Budd Chiari Syndrome and Polycythemia Vera Ryan Sanford 12.8.2009.
Budd chiari syndrome
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Transcript of Budd chiari syndrome
A Case Of Per Abdomen Examination
BY:Dr. Tikal Kansara
R2 Medicine D Unit
Bio Data
• Sajjan Devibhai Balai• 50 / F• Hindu• Illitrate• Married• Housewife• Low socio economic status• From Borakheda Village, Ujjain, Madhya Pradesh
CHIEF COMPLAINS
• Abdominal Distention since 19 years• Abdominal Pain since 19 years
Origin, Duration & Progress
• Abdominal Distention since 19 years• Abdominal Pain since 19 years• Yellowish discoloration of urine & sclera 19
years ago• Swelling of both lower limbs since 19 years• Dilated Veins over the abdomen since 15 years• Early Satiety & anorexia since 19 years
• No History s/o:– Malena, Hemetmesis, Bleeding PR, Diarrhoea,
Vomitting– Burning Micturation, Reduced UOP, Hematuria– Cough, Hemoptysis, Fever– Dyspnoea, Chest Pain, Palpitations, Orthopnea,
PND.
• PAST HISTORY:– Hypo pigmented patches over the skin of cheek,
back & soles since childhood• FAMILY HISTORY:– Nothing Significant
• PERSONAL HISTORY:– Nothing Significant
• MENSTRUAL HISTORY:– Menopausal since last 10 years
• OBSTRETIC HISTORY: G4P3A1L3
– G1 – IUFD @ 7 months– G2 – M/24/Home/FTND/Vaccinated– G3 – M/19/Home/FTND/Vaccinated– G4 – M/15/Home/FTND/Vaccinated
HISTORY CONCLUSIONSo, at the end of history we have a 50 y/o F with long standing abdominal distention, discomfort; which have waxing and waning course being partly relieved by medications, & dilated veins over the abdomen most likely we are dealing with a case of ‘Ascites From Portal Hypertension’. The differentials (According to Anatomical Location of Abnormality) would be as follows:
1. Cirrhosis Of Liver1. Hepatitis B & C Infection2. Autoimmune Hepatitis (Late Stage)
2. Portal / Splenic Vein Thrombosis3. Post-Hepatic Obstruction
1. Membranous Webs2. Myeloproliferative Diseases3. Anti-Phospholipid Antibody
Syndrome4. Recurrent Pregnancies5. Hereditary Thrombophilias
GENERAL EXAMINATION
• Patient is conscious, co-operative and well oriented to time, place & person.– VITALS• TPR- N/84/Regular• BP- 104/62 mmHg
– Hypopigmented patches are present in skin of chin, back, foot & distal phalanyx of dorsum of fingers of hands s/o Vitiligo
• No pallor, cyanosis, clubbing, icterus, pedal edema, lymphadenopathy
• No KF Ring visible• Back & Spine Normal• No Signs Of Liver Failure present– No hair loss, parotid enlargement, spider naevi,
dupuytren contracture, palmer erythema.
PER ABDOMEN EXAMINATION
• INSPECTION– Shape of abdomen –
globular– Umbilicus – Shifted
downwards & inverted– Dilated & tortuous
veins present over upper part of the abdomen and upper part of the back.
• ABDOMINAL MOVEMENTS:– Bulges during inspiration
• No Abnormal pulsations are visible• No peristaltic waves visualised• Skin over the abdomen is lax.
• PALPATION:– Superficial Palpation• Temperature: Normal• Non tender abdomen
– Deep Palpation:• Liver – Not Palpable• Spleen – Not Palpable
– Dilated Veins over upper abdomen – Flow from below upwards on milking veins
• PERCUSSION:– Shifting Dullness is present
• AUSCULTATION:– Bowel Sounds audible– No Bruits, No venous hum
OTHER SYSTEM EXAMINATIONS• RESPIRATORY SYSTEM:
– AEBE – No Crepts / Rhonchi
• CARDIOVASCULAR EXAMINATION:– S1;S2 Normal– No Murmur
• CENTRAL NERVOUS SYSTEM EXAMINATION:– HF/CN Normal– Conscious, Cooperative– No Focal Neurological Deficits– Tone Normal; Power 5/5 in all four limbs– PR ↓ / ↓
EXAMINATION CONCLUSIONSo, from history & physical examination we have a 50 y/o F; with chronic abdominal distention & discomfort with dilated veins over front and back of abdomen, which fills from below upwards, without florid signs of liver cell failure. This is most likely a case of ‘Ascites From Portal Hypertension from Obstruction likely at the level of Hepatic Veins &/or Inferior Vena Cava’. At this point, cirrhosis & other differentials seems less likely & post-hepatic obstruction seems probable enough to label it as Budd-Chiari Syndrome.
Etiology for Budd-Chiari Syndrome at this stage would include:
1. Membranous Webs2. Anti-Phospholipid Antibody Syndrome3. Hereditory Thrombophilias
1. Protein C Deficiency2. Protein S Deficiency3. Factor V Laden Deficiency4. Anti Thrombin III Deficiency
4. Myeloproliferative Diseases5. Recurrent Pregnancies
INVESTIGATIONS• COMPLETE BLOOD COUNT
PARAMETER VALUE NORMAL VALUE
Haemoglobin 12.00 12.0 – 16.0 gm%
Total Counts 6000 4,000 – 11,000 / cumm
Differencials 70 / 28 / 01 / 01
Platelet Count 1.31 lac/ cumm 1.5 – 4.1 lac / cumm
ESR 22 MM 00 – 15 MM
RETICULOCYTE COUNT 0.5 % 0.5 – 2.0 %
PARAMETER VALUE NORMAL VALUE
PCV 32.60 36 – 46 %
MCV 79.00 82 – 92 Fl
MCH 29.20 27 – 32 pg
MCHC 36.80 32 – 35 %
SMEAR STUDY
NORMOCYTIC NORMOCHROMIC RBCs
• Biochemical InvestigationsPARAMETER VALUE NORMAL VALUE
Blood Urea 50 14 – 40 mg/dl
Serum creatinine 0.8 0.1 – 1.2 mg/dl
Bilirubin
Total 1.4 0.1 – 1.2 mg/dl
Direct 0.7 0 – 0.4 mg/dl
Indirect 0.7 0.1 – 0.8 mg/dl
SGPT 20 < 40 U/L
SGOT 42 <37 U/L
ALP 91 28 – 111 IU/L(Adults)
Serum Sodium 136 135 – 145 mmol/L
Serum Potassium 4.5 3.5 – 5.1 mmol/L
Total Protein 8.0 6.0 – 8.0 gm/dl
Serum Albumin 4.8 3.2 – 5.0 gm/dl
PARAMETER VALUE
Chest X-Ray Normal
RBS 122 mg%
ECG Grossly WNL
ASCITIC FLUID ANALYSIS
PARAMETER VALUE NORMAL VALUE
TOTAL CELLS 320 00 – 05 /cumm
DIFFERENCIALS 60 % / 40 %
PROTEIN 3.6
SUGAR 94
• PROTHROMBIN TIME:
• ACTIVATED PARTIAL THROMBOPLASTIN TIME:
PARAMETER VALUE
Prothrombin Time 14.80
Control Time 14.00
INR 1.06
PARAMETER VALUE
PATIENT 30.00
CONTROL 34.00
Ultrasound Abdomen
• LIVER: – Span: 126 mm – Altered with surface irregularity– s/o Cirrhosis
• Spleen: 122 mm Spleenomegaly• Free Fluid: Moderate free fluid in abdomen
ULTRASOUND LIVER SCREENING• Occlusion of terminal IVC• Entire IVC shows reversal of flow• Both iliac veins show flow reversal
• Right hepatic vein patent, dilated & sole outflow channel of the liver
• Left hepatic Vein patent but its ostium is occluded. Flow drains into right hepatic vein through a prominent collateral running over liver surface.
• Spleen is not enlarged. Portal and splenic vein shows normal hepatopetal flow.
CECT Abdomen• Enlarged Caudate lobe and left lobe of liver with surface
nodularity represents Cirrhotic changes.• Multiple homogenously enhancing nodules of varying sizes
in both lobes represents Regenerating Nodules.• Marked narrowing of intrahepatic IVC seen. Middle hepatic
vein not visualised. Right and left hepatic veins visualized. • Intra-hepatic veno-venous collaterals seen. Multiple
abdominal wall, paraspinal and perioesophageal collaterals seen.
• Above findings represent Budd-Chiari Syndrome.
UGI Scopy
1. GRADE II OESOPHAGEAL VARICES PRESENT.
2. CONGESTIVE GASTROPATHY
Viral MarkersPARAMETER VALUE
HIV Non-Reactive
HBsAg Negative
HCV Negative
Other InvestigationsPARAMETER VALUE
SERUM ANA PROFILE NEGATIVE
PARAMETER VALUE NORMAL VALUE
Serum TSH 4.61 0.35 – 5.50
PARAMETER VALUE NORMAL VALUE
Protein C Level 0.66 units/ml 0.55 – 1.11 Units/ml
Protein S Level 0.92 units/ml 0.60 – 1.13 units/ml
Anti Thrombin III Level 0.23 g/L 0.19 – 0.31 g/L
Factor V Laden 4.6 Units/L 2.0 – 10.0 Units/L
THROMBOPHILIC PROFILE