8/8/2019 jact03i4p282

1/4

Anasarca in a Non-Cirrhotic Portal Fibrosis Patient

after Spleno-Renal Shunt Procedure

Anupam Prakash*, Samir Kubba*, NP Singh**, SK Agarwal***

* Senior Resident, ** Professor, *** Director-Professor and Head,Department of Medicine, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi-110 002, India.

P O S T G R A D U A T E C L I N I C

An 18-year old girl presented with history of two episodes

of haematemesis at the age of 6 years and 15 years. In

between the two episodes, she complained of progressive

abdominal distension, but was not certain whether it was

restricted to left upper abdomen or was diffuse. She was

diagnosed as a case of non-cirrhotic portal fibrosis (on

liver biopsy), and doppler examination of abdomenshowed evidence of portal hypertension. She underwent

spleno-renal shunt surgery. Four months after surgery, she

presented with progressively increasing abdominal

distension and swelling of feet and face over the last two

months. On specific probing, the patient did reveal a

history of reduced urine output for the last 2 days.

However, there was no history of fever, haematuria, pyuria,

dysuria, nocturia, cough, expectoration, chest pain, loose

motions, vomiting, haematemesis, or malena. Clinical

examination revealed a conscious, oriented patient, BP

160/90 mm Hg, no icterus; but pallor, gross ascites, pedal

and facial swelling were present. Systemic examination

revealed decreased air entry at the right lung base with

stony dull percussion note suggestive of right pleural

effusion; however, the liver span was maintained.

Investigations revealed : haemoglobin 8.9 g/dl, TLC 10,000/

mm3, P75 L25, platelets 372,000/mm3, ESR 40 mm at the end

of first hour, normocytic normochromic peripheral blood

picture, blood urea 180 mg/dl, serum creatinine 6.4 mg/

dl, serum sodium 140 meq/l, serum potassium 3.8 meq/l,

random blood sugar 68 mg/dl, total serum bilirubin 0.8

mg/dl, serum alanine transferase 26 units/l, serum alkaline

phosphatase 13 KA units, total serum proteins 6 g/l, serum

albumin 3.2 g/l. Chest radiograph revealed right pleural

effusion. ECG was normal. Ultrasound doppler

examination of the abdomen revealed normal liver span

with slightly coarsened liver echotexture with multiple

channels seen at the porta replacing middle portal vein,

suggestive of portal cavernoma formation with gross

ascites. Both the kidneys were reduced in size right

kidney measuring 7 x 3 cm with cortical thickness of 5

mm and left kidney measuring 8 x 4 cm with cortical

thickness of 7 mm; both showed increased cortical

echogenicity with partial loss of corticomedullary

differentiation. The intrarenal arterial and venous flow

patterns were preserved; bilateral renal veins were patent;and a tributary was seen opening into mid-portion of left

renal vein.

Urine output was 300 ml/24 hours and examination of

urine revealed: 2+ albuminuria, 4-8 RBCs/HPF, fine and

coarse granular casts. Prothrombin time was 18 seconds

as against a control of 14 seconds. The patient was

negative for hepatitis B surface and hepatitis B envelope

antigens, and for antibodies against hepatitis C virus,

hepatitis B core antigen, and HIV 1 and 2. HCV RNA was

not done due to financial constraints. Ascitic tap and right

pleural tap were transudative; serum to ascitic albumin

gradient (SAAG) was 1.6. Blood, urine, and ascitic fluid

cultures were sterile.

Repeated abdominal paracentesis against albumin

infusions were done since the patient appeared in obvious

distress. Kidney biopsy was withheld in view of slight

derangement of prothrombin time and uraemia. It was

planned to be performed after a few dialysis sessions

which would improve uraemia, and after vitamin K

injections which would improve prothrombin time.

Central venous pressure monitoring was done and the

pressure varied from 8-10 cm of water, indicating there

was no obvious intravascular volume contraction.

However, renal functions continued to worsen and the

ascites was refractory. Blood urea increased to 272 mg/dl

and serum creatinine to 10 mg/dl over 2 weeks, with a

urine output of 150 ml/24 hours. The patient was initiated

on intensive haemodialysis and blood transfusion support.

She continued to be oliguric, and expired due to uraemic

JIACM 2003; 4(4): 282-5

8/8/2019 jact03i4p282

2/4

complications (refractory fluid overload, severe metabolic

acidosis, hyperkalaemia). Unfortunately, the relatives

refused a post-mortem kidney biopsy.

Question 1 : What were the problems in thispatient?

Answer 1 : This is a young female who was diagnosed as

a case of non-cirrhotic portal fibrosis (NCPF) and was

negative for viral markers of hepatitis A, B, C, and E. In view

of recurrent haematemesis and recalcitrant ascites, porto-

systemic shunting procedure was performed, namely, the

spleno-renal shunt. The patient, however, developed

anasarca 4 months after surgery, associated with oliguria

and serial derangement of renal parameters including

reduction of renal size.

Question 2 : What are the common causes of

anasarca?

Answer 2 : The common causes of anasarca are :

1. Anaemia and hypoproteinaemia

2. Congestive cardiac failure

3. Chronic liver disease

4. Acute or chronic renal insufficiency, nephrotic

syndrome

5. Protein-losing enteropathies/malabsorptionsyndrome

6. Wet Beri-beri

7. Epidemic dropsy (Argemone mexicanaingestion)

8. Myxoedema, may also simulate anasarca

9. Angioneurotic oedema at times may also simulate

anasarca.

Question 3 : What are the clinical conditions

in which simultaneous renal and hepatic

involvement may be found?Answer 3 : Table I lists the various conditions in which

both liver and kidneys are affected, but the liver disease

does not play an aetiological role in the pathogenesis of

renal failure. These conditions constitute the so-called

pseudo-hepatorenal syndrome. The functional renal

failure encountered in patients with severe liver disease

is called hepatorenal syndrome (HRS) and liver disease is

considered to be at the centre-point of its aetiology.

Table I : Causes of pseudo-hepatorenal syndrome.

1. Primary hepatic disease with secondary renal

involvement :

Obstructive jaundice Renal tubular acidosis,

acute tubular necrosis

Alcoholic liver disease IgA nephropathy, distal

tubular acidosis

HBV and HCV liver disease Glomerulonephritis,

cryoglobulinaemia

Autoimmune liver disease Distal tubular

acidosis

Cirrhosis of varied aetiology Impaired sodium

and potassium excretion, pre-renal azotaemia,

renal tubular acidosis, glomerulonephritis

2. Primary renal diseases with secondary hepatic

involvement :

Hypernephroma Acute renal failure

3. Disorders with simultaneous liver and kidney

involvement :

Congenital Polycystic disease, sickle cell

anaemia

Toxic agents Drugs, viz., tetracycline, halothane,

acetaminophen, sulphonamides, rifampicin,

allopurinol, and carbon tetrachloride poisoning

in industrial workers

Infections Miliary tuberculosis, hepatitis B,

septicaemia, yellow fever, leptospirosis

Connective tissue disorders

Circulatory alterations Shock, heart failure

Unknown aetiology Amyloidosis, sarcoidosis,

Reyes syndrome.

Question 4 : How would you diagnose

hepatorenal syndrome and what are the

points in favour of, or against it in this case?

Answer 4 : Hepatorenal syndrome (HRS) is the final stage

of complex haemodynamic derangements associated

with portal hypertension, namely, peripheral arterial

vasodilatation and reduced effective arterial blood

volume which ultimately leads to intense renal

vasoconstriction. It is characterised by worsening

azotaemia with avid sodium retention and oliguria due

to marked reduction in renal perfusion and glomerular

filtration rate in the absence of any characteristic or

significant histological abnormalities of kidneys. It is a

common complication of patients with chronic liver

Journal, Indian Academy of Clinical Medicine Vol. 4, No. 4 October-December 2003 283

8/8/2019 jact03i4p282

3/4

284 Journal, Indian Academy of Clinical Medicine Vol. 4, No. 4 October-December 2003

disease and ascites. HRS may need to be differentiated

from pre-renal azotaemia and acute tubular necrosis;

which is outlined in Table II1,2. At times, HRS may be

precipitated by severe gastrointestinal bleeding, sepsis,

or overly vigorous attempts at diuresis or paracentesis.HRS may mimic pre-renal azotaemia very closely, which

at times may be superimposed on HRS. However, pre-renal

azotaemia is often accompanied by increased

haematocrit, orthostatic rise in pulse rate with a fall in

blood pressure. If plasma expansion leads to an increase

in central venous pressure upto 10 cm of water without

any improvement in urine output, pre-renal azotaemia is

unlikely.

although a kidney biopsy could not be performed in this

case, since the patient presented to us with markedly

deranged renal functions. In a pioneering study3 on NCPF

patients undergoing spleno-renal shunt surgery, it was

reported that the incidence of proteinuria increased from7% during the pre-operative period to 32% during the

post-operative follow-up. Clinical presentation as

nephrotic syndrome was seen in 28% over a 5-year follow-

up. Microhaematuria worsened from 6% at one month

post-operatively to 25% after 4 years. In the

glomerulonephritis patients, serum creatinine worsened

from a pre-operative value of 1 mg% to 3.3 mg% at 5 years.

Similar findings were not seen in patients of extrahepatic

Table II : Differentiation of HRS from pre-renal azotaemia and acute tubular necrosis (ATN).

Differentiating characteristic HRS Pre-renal azotaemia ATNHistory of precipitating event May or may Invariably present; Present with fluid loss, shock,

not be present loss of fluids evident sepsis, or nephrotoxic drugs

Urine output Oliguria Oliguria Oliguria, anuria

Urinary sediment Absent Hyaline casts Granular casts, cellular debris

Urinary sodium (meq/l) < 5 < 10 > 20

Urine to plasma osmolality ratio > 1.5 > 1.5 1

Urine to plasma creatinine ratio > 40 > 40 < 20

Response to plasma expansion Absent Good Absent

This patient was subjected to abdominal paracentesis due

to recalcitrant ascites, although they were done against

albumin infusions. She was also on diuretics although she

continued to have oliguria. No evidence of sepsis was

evident, although inadvertent transient bacteraemia may

have occurred since patient had peripheral intravenous

access, central venous line, haemodialysis access, and

abdominal paracentesis was also repeatedly performed,

though with aseptic precautions. All these factors are

known to precipitate HRS at times. Also, her central venous

pressure was normal. The presence of a mild active urinarysediment and shrunken kidneys are strong points against

the diagnosis of HRS.

Question 5 What is the likely precipitating

factor in this case?

Answer 5 : Spleno-renal shunt operation is the probable

precipitating factor for renal deterioration in this case. The

active urinary sediment suggests glomerulonephritis,

portal obstruction who underwent similar surgery.

The exact pathogenesis of an increased occurrence of

glomerulonephritis in NCPF patients following spleno-

renal shunt surgery is unclear. It is known that the hepatic

reticulo-endothelial system (RES) function in NCPF is poor,

while it is intact in extrahepatic portal obstruction.

Although the shunt is performed to reduce portal

hypertension and thus prevent gastrointestinal

haemorrhage, it enhances direct porto-systemic

circulation leading to bypass of hepatic RES. This

eliminates the first pass mechanism which is important

for clearance of immune complexes emanating from the

gastrointestinal tract, thereby leading to excessive delivery

to the systemic circulation and resulting in immune-

complex glomerulonephritis. A similar mechanism is

believed to exist in cirrhosis also, where

mesangioproliferative glomerulonephritis has been

reported to occur after a spleno-renal shunt procedure4.

However, similar procedure does not lead to renal

8/8/2019 jact03i4p282

4/4

Journal, Indian Academy of Clinical Medicine Vol. 4, No. 4 October-December 2003 285

involvement in extrahepatic portal obstruction where the

hepatic RES is normal-functioning3. Therefore, it is agreed

that spleno-renal shunting procedure alone is not the

predisposing factor but it can be in the presence of poor

hepatic clearance as in NCPF and cirrhotics.

References

1. Brady HR, Brenner BM. Acute renal failure. In: Braunwald E,

Fauci AS, Kasper DL et al(eds.) Harrisons principles of Internal

Medicine. New York:McGraw Hill, 15th edn, 2001; pp 1541-51.

2. Chung RT, Podolsky DK. Cirrhosis and its complications. In:Braunwald E, Fauci AS, Kasper DL et al (eds.) Harrisons

principles of Internal Medicine. New York:McGraw Hill, 15th

edn, 2001; pp 1754-66.

3. Dash SC, Bhuyan UN, Dinda AKet al. Increased incidence of

glomerulonephritis following spleno-renal shunt surgery

in non-cirrhotic portal fibrosis. Kidney Int1997; 52: 482-5.

4. Rana SS, Das K, Mukhopadhyay S et al. Mesangial

proliferative glomerulonephritis triggered by spleno-renal

shunt in a cirrhotic patient. J Assoc Physicians India2002:

50; 266-8.