P O S T G R A D U AT E C L I N I C
Anasarca in a Non-Cirrhotic Portal Fibrosis Patient
after Spleno-Renal Shunt Procedure
Anupam Prakash*, Samir Kubba*, NP Singh**, SK Agarwal***
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 abdomen
showed 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
* Senior Resident, ** Professor, *** Director-Professor and Head,
Department of Medicine, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi-110 002, India.
JIACM 2003; 4(4): 282-5
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
complications (refractory fluid overload, severe metabolic
acidosis, hyperkalaemia). Unfortunately, the relatives
refused a post-mortem kidney biopsy.
Question 1 : What were the problems in this
patient?
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/malabsorption
syndrome
6. Wet Beri-beri
7. Epidemic dropsy (Argemone mexicana ingestion)
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.
Journal, Indian Academy of Clinical Medicine
Vol. 4, No. 4
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,
Reye’s 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

October-December 2003 283
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.
Table II : Differentiation of HRS from pre-renal azotaemia and acute tubular necrosis (ATN).
Differentiating characteristic HRS
History of precipitating event May or may
not be present
Urine output Oliguria
Urinary sediment Absent
Urinary sodium (meq/l) <5
Urine to plasma osmolality ratio > 1.5
Urine to plasma creatinine ratio > 40
Response to plasma expansion 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 urinary
sediment 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,
284 Journal, Indian Academy of Clinical Medicine
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 from
7% 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
Pre-renal azotaemia ATN
Invariably present; Present with fluid loss, shock,
loss of fluids evident sepsis, or nephrotoxic drugs
Oliguria Oliguria, anuria
Hyaline casts Granular casts, cellular debris
< 10 > 20
> 1.5 1
> 40 < 20
Good Absent
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

Vol. 4, No. 4
October-December 2003
involvement in extrahepatic portal obstruction where the 2.
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 3.
hepatic clearance as in NCPF and cirrhotics.
4.
References
1. Brady HR, Brenner BM. Acute renal failure. In: Braunwald E,
Fauci AS, Kasper DL et al (eds.) Harrison’s principles of Internal
Medicine. New York: McGraw Hill, 15th edn, 2001; pp 1541-51.
Journal, Indian Academy of Clinical Medicine
Vol. 4, No. 4
Chung RT, Podolsky DK. Cirrhosis and its complications. In:
Braunwald E, Fauci AS, Kasper DL et al (eds.) Harrison’s
principles of Internal Medicine. New York: McGraw Hill, 15th
edn, 2001; pp 1754-66.
Dash SC, Bhuyan UN, Dinda AK et al. Increased incidence of
glomerulonephritis following spleno-renal shunt surgery
in non-cirrhotic portal fibrosis. Kidney Int 1997; 52: 482-5.
Rana SS, Das K, Mukhopadhyay S et al . Mesangial
proliferative glomerulonephritis triggered by spleno-renal
shunt in a cirrhotic patient. J Assoc Physicians India 2002:
50; 266-8.

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