A 45 years old female with rapidly filling ascites .

DR.NAVYA(INTERN)

DR.CHETANA(INTERN)

DR.ABDUL RAHEEM (INTERN)

DR.ASHFAQ(INTERN)

DR.SRAVYA(INTERN)

DR.GNANADA(INTERN)

DR.CHARAN(PG1)

DR.VAMSI(PG1)

DR.SUSMITHA(PG2)

DR.ADITHYA (PG3)

DR.PRANEETH(PG3) 

DR.PRAVEEN NAIK (ASS.PROF)

DR.RAKESH BISWAS(HOD)

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Here is a case i have seen:

A 45y/o female, from West Bengal, presented to casualty with complaints of abdominal distention since 2 years & pedal edema since 2 months.

Patient was apparently asymptomatic 2 years back. Then developed abdominal distension , insidious in onset and has been rapidly progressing since last one year.

With the increased distention she complains of SOB, difficulty to eat and drink and extreme low back ache. She has desire to eat varieties but is unable to .On doing paracentesis there is improved urine output and decreased SOB ,increased appetite.

Pedal edema since 2 months which is more in the mornings.

She’s k/c/o DM since one year. Took medicines for 6 months and stopped.

Not a k/c/o HTN , TB, Thyroid or Cardiac diseases.

No significant Family history. Has 7 children ( 4 sons , 3 daughters)

Menopause -4.5 years ago.

Bowel movements irregular, sleep inadequate , No addictions. 

No h/o. Drug Allergies 

General Examination-

Pt is conscious, coherent and cooperative.

Malnourished.

Head to toe examination :-
    Hair quality is poor with thin and sparsely greyish hair

Severe temporal wasting present, loss of buccal pad of fat consistent with hippocratic facies

Skin fold thiclness <4 mm with severe generalised proximal wasting

Clubbing +

B/L Pedala edema +

Terry nails +

No Pallor, Icterus, Cyanosis , Koilonychia, Palmar erythema.

No generalised lymphadenopathy

Vitals:-

Temperature- 98.5 F

Bp - 110/90 mm Hg

PR - 64 bpm , regular

RR -24 cycles / min

P/A:

Inspection- Ovoid distension of abdomen , slit like umbilicus, absence of scars and sinuses , engorged veins+

Abdominal distension greater in lower abdomen  with UA : 18cm & LA : 21 cm

Palpation- ? Lymph nodes palpated around umbilicus -multiple, smooth, regular , adherent, non-tender and immobile.

Hepatojugular reflux sign -nt.

Percussion- Fluid Thrill +nt

Auscultation: Bowel sound +

CVS: S1 S2 + , no murmurs

RS: Complete restriction of movement of right hemithorax with strong dull note and decreased breath sounds likely suggesting right pleural effusion.
No Rib crowding was noted

CNS: NFND

INVESTIGATIONS:

PT 15

INR 1.1

APTT 30sec

Ascitic tap:

                                                  

Ascitic Fluid Cell Count 

TC 192

N 60%

L 40

RBC plenty

Pleural tap:

Serology: Negative

X-ray chest:PA view

Triple phase CECT 

PROVISIONAL DIAGNOSIS:

ASCITES with PORTAL HTN

RIGHT PLEURAL EFFUSION

Treatment:

Day 1 to Day 8

Fluid restriction less than 1litre per day

Salt restriction less than 2 gms per day

Tab.Lasilactone  

BP/PR/TEMPERATURE MONITORING 4TH HOURLY

Ascitic tap done on day 1 ,5litres tapped

On analysis it is high SAAG low protien,it is probably due to portal HTN ,

QUESTION:

what is the cause of her portal hypertension?

Pleural tap was done on day 2

On analysis according to lights criteria it transudate.

?.Hepatic hydrothorax

On day 7 

Ascitic tap and pleural tap was done at same time sent for analysis:

Right sided:Pleural fliud

Left sided: Ascitic fluid

                           ASCITIC FLUID.                 PLEURAL FLUID

Sugars.                  109mg/dl.                            75mg/dl

Protein.                 1.5 gm/dl.                            2.1 gm /dl

LDH.                      214 IU /L.                            234 IU/L

SAAG.                    1.5

CELL COUNTS

TC.                         21 cells.                              25 cells

DC.         

 lymphocytes.       80 percent.               40 percent

  neutrophils         20 percent.               60percent

RBC.                      present.                  Plenty

DAY8 _Therapuetic ascitic tap done 2 litres tapped 

DAY 9 &DAY 10

Fluid restriction less than 1 litre per day

Salt restriction less than 2 gms per day

TAB.LASIX 80 mg BD

TAB.ALDACTONE 50 mg BD

ON DAY 10 :

24 HRS URINE VOLUME:1,100ML

24 HRS URINARY CREATININE:0.42GM/DAY

24 HRS URINARY SODIUM:278mmol/day

DAY 11 to DAY 14

Fluid restriction less than 1 litre per day

Salt restriction less than 2 gms per day

TAB.LASIX 20 mg BD

TAB.ALDACTONE 50 mg BD

SERUM CREATININE_0.9MG/DL

Day 11: Ascitic tap done 2 litres tapped

Day 14:

MR venogram done:

Shrunken right lobe of liver with hypertrophy of left lobe

Gross ascitis 

Gross right sided pleural effusion with pleural thickening and collapse of entire lung

Multiple vertebral bodies and rib lesions in the visualised dorsal and lumbar spine

Severe narrowing of the intrahepatic portion of inferior vena cava with cranial most aspect of 

showing  very faint intraluminal opacification on contrast administration suggestive of chronic budd chiari syndrome

Rule out sarcoidosis and metastasis.

DAY 15:

Fluid restriction less than 1 litre per day

Salt restriction less than 2 gms per day

TAB.LASIX 20 mg BD

TAB.ALDACTONE 50 mg BD

TAB.WARFARIN 5MG OD

INJ.ENOXAPARIN 40 MG OD

Ascitic tap done:2 litres tapped

DAY 16:

Fluid restriction less than 1 litre per day

Salt restriction less than 2 gms per day

TAB.LASIX 40 mg BD

TAB.ALDACTONE 50 mg BD

TAB.WARFARIN 5MG OD

Heparin was withheld

Started prednisolone 30 mg OD

APRAXIA CHARTING WAS DONE:

Day 17-19: 

Fluid restriction less than 1 litre per day

Salt restriction less than 2 gms per day

TAB.LASIX 40 mg BD

TAB.ALDACTONE 50 mg BD
Planned for iliac crest biopsy.
Anticoagulants and steroids are withheld until the iliac crest biopsy

Day 20 :

CT guided Bone Biopsy: From posterior side of iliac crest under LA to evaluate the multiple hyperdense lesions in the bones (spine, ribs , pelvis)

Fluid restriction less than 1 litre per day

Salt restriction less than 2 gms per day

TAB.LASIX 40 mg BD

TAB.ALDACTONE 50 mg BD

Day 21:
Orthopaedic referral done for restricted left shoulder movement since 4 months.
Advised - X Ray Left Shoulder: F/s/o aesthetic changes 
TAB DOLONEXT DT BD x 1 week 
TAB PAN 40 mg x 1 month

TAB TENDOFIT OD x 1 month
Physiotherapy IFT/tens left shoulder with shoulder room exercises.

Day 22-23:

Fluid restriction less than 1 litre per day

Salt restriction less than 2 gms per day

TAB.LASIX 40 mg BD
TAB.ALDACTONE 50 mg BD
TAB DOLONEXT DT BD x 1 week
TAB PAN 40 mg x 1 month
TAB TENDOFIT OD x 1 month
Physiotherapy IFT/tens left shoulder with shoulder room exercises.

DIAGNOSIS : Chronic Budd Chiari Syndrome , likely secondary to systemic sarcoidosis with multiple osteosclerotic bone lesions