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KOR J CEREBROVASCULAR SURGERY

September 2010 Vol. 12 No 3, page 159-164

Non-Traumatic Primary Intraventricular Hemorrhage


Department of Neurosurgery, College of Medicine, Ewha Womans University, Seoul, Korea

Sang Wook Ahn, MDSung-Kyun Hwang, MD


ABSTRACT
Objective : Non-traumatic primary intraventricular hemorrhage (PIVH) in adults has rarely been reported. This study aimed to identify
clinical features, risk factors, and neurological outcomes of PIVH in adults. Methods : We retrospectively reviewed the clinical data,
complementary examinations, outcomes, and computed tomography (CT) scans for non-traumatic PIVH occurring between 2002 and 2008
at our institutions. We defined PIVH as a hematoma caused by non-traumatic factors, either confined completely within the ventricular
system or arising within 15 mm of the ventricular wall. Result : Among the 19 PIVH patients treated in our institutions, the mean age was
55.5 years (range 19-81), and the male to female ratio was 0.72. Symptom onset was abrupt in 18 patients and progressive in 1. The most
frequent complaint was headache (47%) followed by nausea and vomiting (38%). Seven patients (38%) had mental status impairment,
ranging from stupor to semicoma. The causes of PIVH were unknown, although arteriography showed a suspicious vascular malformation in
1 patient. Seven (37%) patients died or entered a vegetative state. All survivors became functionally independent. No patients received
ventriculoperitoneal shunts. Two of the surviving patients had limb weakness. Overall, 7 patients (37%) had GOS scores 1 to 2 and 12 (63%)
had GOS scores 3 to 5. Conclusion : Although present technology allows clinicians to reach a diagnosis in many PIVH patients, the
condition's etiology remains unknown, and its clinical manifestations vary, due to the rarity of these cases. Overall results and prognoses
seem relatively better than in secondary intraventricular hemorrhage. However, evaluating and clarifying the nature of PIVH requires
experience with more cases. (Kor J Cerebrovascular Surgery 12(3):159-164, 2010)
KEY WORDS : Intraventricular hemorrhageRisk factorOutcomeStroke

Introduction
One uncommon type of intracerebral hemorrhage (ICH),
the primary intraventricular hemorrhage (PIVH), is a nontraumatic hemorrhage confined to the ventricular system,
without any bleeding into the cerebral parenchyma, and its
incidence is very rare.1)3)13)19) Sanders described PIVH first,
more than a century ago, but few researchers have studied it
since then. Prior to the introduction of the computed
tomography (CT) scan, knowledge concerning PIVH came

: 2009 12 27
: 2010 06 21
: Sung-Kyun Hwang, Department of Neurosurgery,
Mokdong Hospital, 911-1 Yangcheon-gu, Seoul, 158-710, South Korea
: (02) 2650-2872 : (02) 2650-0948
E-mail : nshsg@ewha.ac.kr

from autopsy series, which usually included patients with


massive intraventricular hemorrhages who had had poor
prognoses, or, more rarely, from a few patients undergoing
surgery.
The definition of PIVH is a hematoma either confined
completely within the ventricular system or arising within 15
mm of the ventricular wall. 1)13)19) The presence of
intraventricular blood is a source of general alarm to
neurosurgeons due to the possibility of hydrocephalus and
ensuing brain damage. Although small amounts of blood
resolve quickly, with no sequelae, the neurosurgeon often
encounters a patient who has moderate or severe IVH
involving all the ventricles. Relatively little is known about
IVH's clinical and imaging features, and even less about its
prognosis and mortality predictors. The present study aimed
to analyze the clinical and imaging features, etiology,
prognosis, and predictors of outcome in PIVH.

159

Non-Traumatic Primary Intraventricular Hemorrhage

Patients and Method


We retrospectively reviewed the clinical data,
complementary examinations, outcomes, and computed
tomography scans for non-traumatic PIVH occurring
between 2002 and 2008 at our institutions. We defined PIVH
as a hematoma caused by non-traumatic factors, either
confined completely within the ventricular system or arising
within 15 mm of the ventricular wall. Evaluations of the
clinical features included type of onset, presenting type and
symptoms, risk factors, and treatment methods. We
evaluated all CT scans in detail for the location of blood in
the ventricle and for hydrocephalus and reviewed other
radiological examinations for any abnormalities.
We evaluated patient outcomes as death directly related to
PIVH, survival with a significant neurologic deficit, or
survival with minimal neurologic deficits and functional
independence. The patients' follow-up periods ranged from 3
month to 2 years.

Results
Patient age ranged from 19-81 years (mean age 55.5
years), with the majority (79%) between the ages of 38 and
71 years. There were 19 patients: 8 females and 11 males.
Hematoma sizes ranged between 2 and 42 cc, with 7 under
10 cc, 6 between 10 and 30 cc, and 6 between 30 and 50 cc.
Symptom onset was abrupt in 18 patients and progressive
in 1 patient. The most frequent complaint was headache
(47%), followed by nausea and vomiting (38%), and 7
patients (38%) suffered from impaired mental statuses,
ranging from stupor to semicoma. One patient had cranial
nerve abnormalities, consisting of extraocular movement
abnormalities (dysconjugate gaze) and facial droop. Motor
deficits and abnormal reflexes (hyperreflexia, pathologic
reflexes) were present in 2 patients.
Ten patients had elevated blood pressure, with systolic
pressure ranging from 150-230 and diastolic pressure from
80-120 mm Hg. Intraventricular hemorrhage was present in
the lateral ventricles in 9 scans (7 bilateral, 2 unilateral),
while 10 scans showed blood in all ventricles. Of the 9
patients presenting with or developing, hydrocephalus, 8
underwent extraventricular drainage. The remaining 1 was
treated medically (Table 1).

160 Kor J Cerebrovascular Surgery 12(3):159-164, 2010

The associated risk factors were hypertension in 9


patients, diabetes mellitus in 3 patients, and end stage renal
disease, angina, and valvular heart disease in 1 patient each.
Angiography was performed on 8 patients. These revealed
no aneurysms, definite arteriovenous malformations, or other
vascular anomalies except for a suspicious vascular
malformation in 1 patient.
Regarding outcomes, 7 (37%) patients died or entered a
vegetative state, of whom 4 (58%) had hematomas less than
30 cc and 3 (42%) had hematomas greater than 30 cc. Of the
remaining 12, all became functionally independent; 2 (16%)
had hematomas greater than 30 cc and 10 (84%) has
hematomas less than 30 cc. These results suggest that
hematoma volume could correlate with prognosis, but
demonstrating this would require a large-scale study needed.
Of the functionally independent patients, 2 had limb
weaknesses. Overall, 7 patients (37%) had Glasgow
Outcome Scale (GOS) scores of 1 to 2 and 12 (63%) scored
3 to 5. No permanent CSF diversions were performed.

Discussion
1. Clinical Features
Excluding premature infants, PIVH is very rare and
accounts for only 3.1% of all non-traumatic central nervous
system hemorrhages. Considering only the adult population,
PIVH is still relatively rare, comprising only 3.1% of all
intracranial hemorrhages, and clinicians have reported only a
small number of cases. 1)9)13)19) Although PIVH is rarely
encountered clinically, the real frequency is unknown, as
PIVH may in fact be the cause of any sudden death, in
particular, any death occurring before the patient reaches the
hospital or receives a CT scan. Hospital series may thus be
biased toward more benign cases, as current diagnostic
imaging capabilities allow the detection of less severe cases.
CT can now confirm a diagnosis of PIVH easily and rapidly,
depicting blood when it occurs in the ventricular system
only, even when it is a small clot in a circumscribed portion
of the ventricular system (usually a lateral ventricle).
PIVH affects all age groups, with a mean onset age of 60
years, similar to that reported in other series.13)19) Earlier
PIVH diagnoses relied on a sudden onset, with coma or
decreased consciousness level, nausea and/or vomiting,
nuchal rigidity, severe headache, and bilateral extensor

Sang Wook AhnSung-Kyun Hwang

plantar responses, followed by a severe and often fatal


outcome.
Nearly all our patients complained of headache. Nausea
and/or vomiting, nuchal rigidity, and unilateral or bilateral
extensor plantar responses were frequent, but focal motor
symptoms and signs were absent or mild. This probably
correlated with midline hemorrhage without parenchymal
damage, although hematoma's asymmetric features could
explain the focal signs. As noted in other studies,
hydrocephalus was also a frequent complication in our
patients but only occurred when the third or fourth ventricle
was involved.
PIVH without a parenchymal component is unusual,
carrying an average mortality rate of about 40%. When it
occurs in association with aneurysm and a large blood

volume causing hydrocephalus, the mortality rate


approaches 90%.12)13)19) Unlike intraparenchymal hemorrhage
patients, PIVH survivors often left the hospital with no
physical disability, which presumably indicates the absence
of parenchymal damage. On the other hand, residual
cognitive dysfunction is relatively frequent in such patients.
2. Etiology
The etiology of PIVH is varied, and, in some patients,
unknown. Some researchers have argued, by analogy with
subarachnoid hemorrhage, that vascular malformations
adjacent to the ependymal lining or very small
malformations, which either self-destroy because of the
hemorrhage or do not opacify on angiography, may cause
these cases. In some reports, patients whose angiographic

Table 1. Clinical data


Pt

Age

No.

/Sex

1
2

Tx.

CT(IVH)

vol.

Onset

HC

DSA

Symptom & Sign

Risk factor

Outcome

70/m

all

30

semicoma

none

EVD

death

49/m

all

32

semicoma

mannitol

death

62/f

LV

16

headache

none
vavular heart
disease

mannitol

no impairment

44/m

LV

23

nl

stupor

none

EVD

memory deficit

45/m

LV

19

nl

headache

none

mannitol

no impairment

19/m

LV

headache

none

mannitol

no impairment

76/f

all

35

headache

HT

mannitol

no impairment

54/m

LV

nl

headache

HT

mannitol

no impairment

63/m

all

32

stupor

HT/DM

EVD

death

10

81/f

LV

24

headache

HT

mannitol

no impairment

11

76/f

all

semicoma

HT/DM

EVD

death

12

74/f

all

semicoma

ESRD/DM

EVD

death

13

66/f

all

39

semicoma

none

EVD

vegetative state

14

45/m

all

nl

headache

none

mannitol

no impairment

15

50/f

all

20

abnormality, facial

HT

EVD

death

EOM
droop
16

38/m

LV

17

40/f

LV

18

32/m

all

45

19

71/m

LV

10

nl

hemiplegia

HT

mannitol

hemiplegia

nl

headache

HT

mannitol

no impairment

hemiplegia

none

EVD

hemiplegia

headache

HT/angina

mannitol

no impairment

susp
AVM
nl

Tx.=treatment, HC=hydrocephalus, m=male, f=female, IVH=intraventricular hemorrhage, nl = normal,


LV=Lateral ventricle, 3V=third ventricle, 4V=fourth ventricle, A=acute, P=progressive, HT=hypertension, DM=diabetes mellitus, ESRD=end stage renal
disease, AVM=arteriovenous malformation, EOM=extraocular movement

Kor J Cerebrovascular Surgery 12(3):159-164, 2010 161

Non-Traumatic Primary Intraventricular Hemorrhage

findings were normal while they lived showed a vascular


malformation at autopsy.13)22) PIVH is frequently caused by
vascular malformations, including cavernous angiomas and
aneurysms. Some researchers have suggested that
malformations are more probable in young patients.13)22)
PIVH was attributed to hypertension in 9 of our patients.
However, we prefer to speak in terms of association rather
than causality, because we cannot prove that hypertension in
fact caused the bleeding. Hypertension was the only
identified risk factor in 50%-70% of patients in the
series,1)11)13) and it was present in all patients reported by
Gates et al.6)
In literature review, the most frequent cause of PIVH was
a very small, parenchymatous, hypertensive hemorrhage,
originating in tissues very close to the ventricular system
(caudate and thalamus), of a type that can go undetected by
CT or arise in the choroid plexus. We therefore believe that
hypertension can induce hemorrhage in choroidal arteries.
This study agrees with previous work regarding the high
probability that thalamic ICH will rupture into the ventricles.
This may be due to the thalamuss anatomic proximity to the
third ventricle, as well as to blood's tendency to spread
medially. Finally, one of our patients had a suspected
intraventricular tumor, and previous studies have also
reported such. A wide range of other possible causes of
PIVH has been reported in the literature. Recently, CT,
computed tomography angiography (CTA), magnetic
resonance image (MRI), and magnetic resonance
angiography (MRA) have come into use as noninvasive
screening procedures for intracranial vascular abnormalities.
Hence, these noninvasive imaging modalities are useful for
screening for PIVH, particularly in older, hypertensive
patients. MRI is useful for investigating possible vascular
malformations, and MRA and CTA are likewise useful for
aneurysm investigation. However, a negative result cannot
completely exclude a vascular lesion, and a definitive
diagnosis and treatment planning may require conventional
cerebral angiography.22)23)
3. Prognosis & Risk factors
Hydrocephalus is a frequent PIVH complication and is
probably due to the obstruction of cerebrospinal fluid (CSF)
circulation or impairment in meningeal absorption.

162 Kor J Cerebrovascular Surgery 12(3):159-164, 2010

Intuitively, it makes sense that IVH impacts clinical


outcomes through several possible mechanisms. 20) The
ventricular system can provide an outlet for IVH expansion
with perhaps less resistance than does brain parenchyma,
and IVH volume can independently exert a mass effect on
the surrounding brain tissue. In addition, obstruction of the
cerebrospinal fluid can cause obstructive hydrocephalus,
which can raise intracranial pressure, resulting in global
impairment.7)12)19) Finally, the presence of blood in the CSF
may represent a global injury to the brain, whereas the
original IVH may only result in a focal deficit. It was not
surprising that hematoma volume and degree of midline shift
correlated positively with mortality. If the IVH was close to
the foramen of Monro or within the third or fourth
ventricles, the likelihood of obstructive hydrocephalus
increased, which increased the chance of a lethal result.
Acute hydrocephalus contributed significantly to death and
deterioration in the acute stages. Brott et al.2) speculated that
hemorrhages originating in the periventricular regions are
more likely to expand toward the nearby ventricle because
the fluid spaces are more compressible than the surrounding
brain parenchyma is, as the CSF redistributes extracranially.
The results of Young et al.21) support this. They found that
thalamic hemorrhages, with their proximity to the ventricles,
correlated with larger IVH volumes and worse outcomes8)10)19)
and showed a close correlation between baseline
intraventricular blood volume and outcome. Similarly, they
found deep hemorrhages in association with increased
incidence of obstructive hydrocephalus, which results in
raised intracranial pressure, beyond the direct effects due to
the hemorrhage volume. Prognosis would logically seem
related to the amount of blood; the more severe the
hemorrhage, the worse the prognosis, and our study shows
possibility of this relationship. However, some authors have
not found a lethal volume of intraventricular hematoma or a
correlation between hematoma size and outcome.1)3)13)
The correlation between IVH and increased morbidity is
not clearly understood. Certainly, the CSF outflow blockage
with obstructive hydrocephalus and subsequent increase of
intracranial pressure (and, therefore, the reduction in
cerebral perfusion pressure) plays an important prognostic
role.20) Hydrocephalus is an independent outcome predictor
in spontaneous IVH. The observation that IVH volume may

Sang Wook AhnSung-Kyun Hwang

be associated with a commensurate decrease in global


cerebral blood flow further supports this concept.
Interestingly, in other studies, outcomes did not differ in
those patients treated by ventriculostomy. This may support
the hypothesis that the enhanced morbidity associated with
IVH is due, at least in part, to the pressure the clot exerts on
periventricular structures. This emphasizes the possible
impact of any treatment that prevents IVH or limits further
IVH expansion. Others have correlated total IVH volume
with outcome via a lethal volume of IVH, which is greater
than 20 mL.8)21) Proposed mechanisms for this IVH-induced
morbidity include development of hydrocephalus, decreased
consciousness, and IVH-induced inflammation. The greater
the age and the further the blood pressure is out of the
normal range (whether higher or lower), the worse the
prognosis. 12) Research has also confirmed that clinical
condition and level of consciousness on admission
significantly influence prognosis.2)13) A good prognosis in
such circumstances seems due to the absence of
parenchymatous damage.13)
Some authors have noted memory difficulties, a common
consequence in such patients.5)13)19) Some have suggested that
these memory problems are due to a lesion of the fornix or
dorsomedian thalamic nuclei. In addition, in patients whose
initial clinical condition is not serious, early treatment
strategies aimed to prevent increases in ventricular
enlargement may improve their prognoses.
4. Treatment
The appropriate treatment for PIVH is not clear, and the
prognosis is variable, with mortality rates previously
reported as 40-83%.1)9)13)19)20) The need of extraventricular
drainage in IVH patients is seven times greater than in other
types of hemorrhage; however, the poor outcome rate for
EVD patients is alarmingly high.8)14)20) While this is likely due
to the larger volume of IVH and hydrocephalus, EVD related
morbidity such as rebleeding and infection may contribute to
the observed poor outcome in these patients, although this
has been recently debated. IVH patients' poor outcomes,
with or without EVD, should encourage the development
and testing of new treatments for IVH, such as
intraventricular instillation of thrombolytic agents.4)15-18)
Clinicians have used for IVH due to a variety of causes,

with good results and no bleeding complications, and,


consequently, can make good use of it. However,
establishing that it improves outcomes or reduces the need
for ventriculoperitoneal shunting would be a complicated
analysis, due to both IVH's variable causes and the many
prognostic factors involved. Perhaps the aggressive
management of hydrocephalus and the use of thrombolytic
agents in the future will improve the outcomes of patients
with primary intraventricular hemorrhage.

Conclusion
Although present technology allows us to reach a
diagnosis of PIVH in many patients, its etiology and clinical
manifestations are varied and remain largely unknown, due
to rarity of such cases. Our study shows a possible
relationship between hemorrhage volume and prognosis,
with overall results and prognoses relatively better than for
secondary IVH. However, evaluating and clarifying the
nature of PIVH requires experience with more cases.

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