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 Introduction:
Epistaxis, especially if severe, prolonged
and not associated with hypertension or
bleeding diathesis, can be a manifestation
of an underlying raised, venous pressure,
Superior Venacaval ( SVC) syndrome
or one of its variants. Its detection
is mandatory and its treatment is an emergency.
Case
Presentation:
Mr. M.H. is a 62 year old man. He was
a heavy smoker of about 60 cigarettes
per day for 40 years. He was active
and in a reasonably good physical state,
and able to independently look after his work
affairs.
Seven years ago, he got one cardiac attack
from which he recovered and spontaneously
stopped all the medications that
had been prescribed to him, but he didn't
quit smoking. He was not hypertensive
or alcoholic but he looked at all times
ruddy and plethoric with a constant haemoglobin
level of 18 gm/dl. No data is available
about his previous haematocrit value (PCV).
He was well until 1 0 days prior to seeing
him, when he started to have recurring
attacks of epistaxis mainly from
his left nare. The epistaxis was very
severe and prolonged. He demonstrated
at each time, bleeds of about 300 mls
of blood or more. This had continued for
10 days, and could hardly be controlled
by anterior packing of the nose. The packs
didn't appear to stop the bleeding completely
as the blood passed backward to be spitted
later. At the 9th day of his bleeding
period, the patient's haemoglobin and
PCV went down to 6.9 gm/dl and 25% respectively.
The treating ENT Specialist, because he
couldn't find clearly the cause of bleeding
anteriorly or stop it by anterior
packs, advised to apply a posterior pack,
but the patient refused. In addition,
the patient had been referred to a physician
to exclude any underlying medical cause
for this odd epistaxis.
Other than the low haemoglobin, the required
investigations were found to be normal
except for the erythrocyte sedimentation
rate (ESR) which was 92 mm/hr. Bleeding
time, clotting time, platelet count,
whole blood picture, blood urea, serum
creatinine, and skull X-rays were all normal.
The chest X-ray film showed what was
thought to be a non- homogenous opacity
at the right hilar and right apical areas.
On seeing the patient for the first time,
on the 10th day of his bleeding, his face
was not as pale as the last haemoglobin
level suggested. A blood-soaked pack was
in his left Nare, and he was continously
feeling blood trickling posteriorly down
his pharynx. He was apyrexic, normotensive
and ther~ were no engorged veins in the
neck or upper chest, and no vasculitic
rash, angiomas, organomegaly nor lymphadenopathy.
Cardiac examination was unremarkable.
The lungs were normal to percussion, but
there were low pitched rhonchi allover.
Moderate finger clubbing was the only
other positive physical sign.
Review of the chest X-ray film showed
widening of the superior mediastinum with
an irregular constriction and slight tilting
of the trachea to the right side, that
had been overlooked. The thought that
there may be a mass pressing and occluding
the major veins was considered to be the
cause of this severe epistaxis. Therefore,
an empirical treatment with dexamethasone
was initiated and the patient was told
not to lie down as much as possible in
order to assist draining the veins and
lessening the epistaxis.
MRl of the chest showed at different levels
the presence of a right hilar mass (Fig.
1), a right pulmonary mass at the middle
zone posteriorly, and, as expected, enlarged
paratracheallymph nodes displacing, pressing,
and indenting the two major tributaries
of the superior vena cava- i.e. the right
and left bracheocephalic veins (Fig. 3
& 4. Compare to the diagrammatic anatomic
cross section at Fig. 5).
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Figure 1
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Figure 2
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Figure 3
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Figure 4
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Bronchoscopy couldn't be done because
of the threatening epistaxis, but spuntum
cytology proved to be negative for malignancy.
Unfortunately the patient refused admission
to the hospital, blood transfusion; or
deep X-ray therapy. A few days later,
while the epistaxis was in arrest with
dexatmethasone alone, he suffered a fatal
cardiac attack that appeared to have been
precipitated by the severe anemia and
stress that the patient had sustained
during his last days.
Discussion:
SVC SYNDROME:
Extrinsic compression or intrinsic obstruction
of the thin- walled superior vena cava
may result in superior venacaval syndrome
(SVC); elevated venous pressure in the
upper extremities, head and neck and secondary
increased intracranial pressure, soft
tissue edema, venous distension and venous
collateral formation1
Compression or obstruction occurs in a
large upper mediastinal vein or a low
cervical vein!, which was the state thought
to be present in our patient. The syndrome
may develop rapidly over a period of days
or slowly over a period of many months1.
EPISTAXIS SVC SYNDROME:
Reviewing the available literature in
the MEDLINE, from 1985 through April 2000,
using the keywords: vena cava obstruction,
bracheocephalic obstruction and epistaxis,
didn't show any mention of a relationship
between such venous obstruction and epistaxis.
Rhinology textbooks weren't helpful in
this respect either.
ARTERIAL AND VENOUS EPISTAXIS:
Epistaxis can be arterial or venous in
origin. Arterial bleeding represents most
of the cases (90% y. The vast majority
who suffer from arterial epistaxis bleed
from the nasal septum and chiefly from
the area where anastamosis of the nasopalatine,
greater palatine, anterior ethmoidal and
coronary arteries take place, i.e. the
Little's area2. The confluence of arteries
at this area is called Keisselbach's
plexus3.
It is important to note that the bleeding
from it is arterial in origin and not
venous as some reports suggest2.
The venous bleeding which is common in
young persons, arises from the vein which
lies immediately behind the columella
at the anterior edge of the Little's
area. It runs vertically downwards and
crosses the floor of the nose obliquely
before joining the venous plexus on the
lateral wall of the nose, the Woodruff's
plexus2.
Woodruff's plexus is a collection of large
blood vessels found in many people in
the lateral wall of the inferior meatus posteriorly. These vessels appear to originate
from the posterior pharyngeal wall and
are venous in origin. They are another
well recognized cause of venous epistaxis
which responds to treatment by tamponade2.
The duration of bleeding, as might be
expected is short lived in venous epistaxis
and more prolonged in bleeding of arterial
origin. However, some cases of venous
epistaxis may bleed for more than two
hours.
DOES ELEVATED CRANIAL VENOUS PRESSURE
CAUSE EPISTAXIS?
Raised venous pressure, although uncommon,
had been mentioned as a cause of epistaxis
as may occur in cardiac and pulmonary
disorders including whooping cough and
pneumonia4. It appears that raised venous
pressure doesn't only cause epistaxis,
but may contribute to its severity and
duration, especially if raised venous
pressure is secondary to a state such
as SVC syndrome. The situation is more
or less similar to epistaxis that is associated
with arterial hypertension. The finding
of a high proportion of subjects with
high blood pressure in hospital practice
signifies not that hypertension causes
epistaxis, but rather that patient with
higher blood pressure have more severe
or persistent bleeding and are therefore
eligible for admission to hospital. If
we exclude the arterisclerotic contribution
in the case of arterial epistaxis, one
can apply the same principle on epistaxis
that occur in the context of elevated
venous pressure in the nasal veins.
When occurs in SVC syndrome, epistaxis
looks to be protective against the more
serious neurologic complications of SVC
syndrome. Moreover, it may dampen the
physical signs by reducing the prominence
of the congested neck veins and their
collaterals, in the same way bleeding
oesophageal varices may do with the prehepatic
congestive signs.
DIFFERENTIAL DIAGNOSIS
In our patient's state, a polycythemia
secondary to this heavy chronic smoking
or other sort of polycythemia was not
evident in spite of the marginally increased
haemoglobin level. Moreover, ESR was very
high (92mm/h) during his last illness
and therefore opposes this possibility.
Bleeding time, clotting and platelet count
were all within normal, excluding to a
large extent the possibility of a numerical
or functional thrombocytic aberrancy.
The severity and continuity of bleeding
in this case can not be explained far
away from the impeded venous drainage
of the superior mediastinal veins, and
its consequent nasal venous congestion.
This renews the old fears of hazards that
are associated with the diagnostic procedures
that can be undertaken in such cases.
Treatment:
The control of such sort of epistaxis
necessitates relieving the venous obstruction
first.
In the past, SVC syndrome resulting from
extrinsic compression by cancer was considered
an oncologic emergency that required the
immediate initiation of mediastinal irradiation.
Irradiation was thought to be necessary
because of the need to alleviate increased
intracranial pressure. Lung cancer was
presumed to be the most likely diagnosis,
and because of the erroneous belief that
increased venous pressure would make diagnostic
procedures hazardous! (Is it truly erroneous?)
Recent experiences in adults, however,
suggest that the SVC syndrome is not a
true emergency and that a histological
diagnosis can be quickly established before
treatment begins. Emergency treatment
with mediastinal irradiation is still
warranted in children and occasionally
in adults who have mental state alteration,
other life threatening manifestations
of increased intracranial pressure, cardiovascular
collapse or evidence of upper airway obstruction1.
Blood transfusions, intravenous fluids,
drugs as well anaesthetic agents, should,
if needed, be given through the veins
of the lower limbs rather than through
the upper limbs veins that are congested
and poorly drained.
Acknowledgement:
A lot of thanks to DR. M. F. Kashmoola who provided the most informative MRI films
References:
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