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| Journal of the New Zealand Medical Association,
21-November-2003, Vol 116 No 1186
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Thalamic haemorrhage: a rare presentation of vein of Galen
aneurysmal malformation in infancy
Urmila Chauhan, Milind Tullu, Mamta Muranjan and Keya
Lahiri
Vein of Galen aneurysmal malformation (VGAM) is a unique,
well-circumscribed group of malformations developing at the end of the embryonic
period. It is the most common arteriovenous malformation originating within the
subarachnoid space.1 The natural history of
patients with VGAM is characterised by cardiac failure in neonates, cerebral
hydrodynamic disorders in the fetal, neonatal and infantile age groups, and
cerebral thrombosis in late childhood.1–5
The neurological symptoms of epilepsy and focal neurological deficit occur late
in childhood.1,2 Intracerebral haemorrhage in
infancy is a rare event as cerebral haemorrhage usually develops in older
children.1 VGAM presenting in early infancy
with cerebral haemorrhage leading to convulsions and focal neurological deficit
is an extremely rare occurrence.6–8 We
report such a case of VGAM with thalamic haemorrhage.
Case reportA three-month-old, male child born
of non-consanguineous marriage presented with three episodes of generalised
tonic–clonic convulsions, fever and paucity of movements on the right side
of the body. He had been referred with a diagnosis of complication-associated
meningitis from a peripheral healthcare centre. The birth and perinatal history
were normal.
Physical examination revealed normal vital parameters,
cardiovascular and respiratory systems. Central nervous system examination
revealed right upper motor-neuron-type facial palsy with right-sided hypotonia,
hyperreflexia and muscle power of 2/5 in right upper limb and lower limb.
Investigations including haemoglobin, complete blood count, renal and liver
function tests, and coagulation profile were normal.
Cerebrospinal fluid (CSF) examination revealed presence of
3680 erythrocytes, 3 polymorphs, 3 lymphocytes, normal proteins (40 mg%) and low
sugar (52 mg% for corresponding blood sugar of 120 mg%). The CSF culture did not
grow any organism. Left thalamic haemorrhage and mild compensated hydrocephalus
were detected on the CT scan of the brain. Contrast enhancement disclosed the
presence of a vein of Galen aneurysmal malformation (VGAM) (Figure 1). The
digital subtraction angiogram (DSA) confirmed the diagnosis of a mural type of
VGAM (Figure 2).
The child was treated conservatively with intravenous
antibiotics, anticonvulsants, short course of steroids (for five days) and
cerebral decongestants. He showed significant neurological improvement within
six days of admission. Seizures were well controlled but the hemiparesis
recovered partially and the facial paresis persisted. The child was discharged
after 15 days of hospital stay. Elective endovascular treatment was planned
after the age of five months.
Figure 1. Axial contrast enhanced CT scan of brain.
Arrow denotes the aneurysmally dilated vein of Galen malformation. The thalamic
bleed is seen adjoining the malformation (arrow head) with perilesional
oedema.
 Figure 2. Digital subtraction angiography, arterial (a)
and venous (b) phases showing a slow-flow, single-hole, mural-type VGAM (arrow)
fed by anterior (single arrow head) and posterior (double arrow heads)
pericallosal arteries.
DiscussionThe vein of Galen develops from the
embryologic precursor, which is the median vein of the
prosencephalon.1,2 The arteries feeding the
VGAM are the choroidal arteries, subependymal network of the posterior circle of
Willis, thalamoperforating arteries and the limbic arterial
arch.1,2 Based on the angioarchitecture, two
forms of VGAM are recognised: the ‘choroidal’ type (very primitive
condition with contribution of all choroidal arteries and an interposed network
before opening into the large venous pouch seen in neonates with low clinical
scores), and the ‘mural’ type (direct arteriovenous fistulae within
the wall of the median vein of the prosencephalon seen often in infants with
better disease tolerance and higher clinical
scores).1,2 Our case had the mural type of
malformation. The venous drainage of the malformation occurs through the
straight sinus or through an abnormal, persistent falcine sinus when the
straight sinus is thrombosed, hypoplastic or
absent.1,2 Associated congenital heart diseases
reported include patent ductus arteriosus, patent foramen ovale, sinus venosus
atrial septal defect, partial anomalous pulmonary venous drainage to the
superior vena cava, and aortic
coarctation.3–5
Johnston (1987) reported the following clinical features in
82 infants: CSF disorders (70%), neurological deficits (31%) and neurocognitive
delay (12%); in those aged 1–5 years the same features were seen with a
different frequency, ie, 61%, 33% and 5%
respectively.1 In the 109 neonates and infants
studied by Lasjaunias, over 50% had neurocognitive
delay.1 It is preferable to delay endovascular
treatment till the child is about five months old and management is dictated by
clinical scores in infancy.1
Macrocrania and hydrodynamic disorders are seen in infants.
They may be caused by increased intracerebral (intrinsic) water retention or
increased CSF volume (extrinsic), mechanical compression of mesencephalic
aqueduct, failure of medullary venous resorption of intracerebral water,
etc.1,2 Hydrocephalus and intracranial
hypertension may occur if the sutures stop growing, if medullary venous pressure
decreases, or if the compliance of the venous system
fails.1 Ventriculoperitoneal shunt should be
avoided as it may increase intracranial complications (as the original cause is
not dealt with and shunting creates a centripetal gradient opposite to the
normal centrifugal gradient).1 In patients
presenting late with clinical manifestations of raised intracranial pressure and
ventricular haemorrhage, emergency embolisation leads to insufficient clinical
improvement even if the hydrodynamic result is good and surgical ventricular
drainage may be necessary (with additional embolisation, the ventricular drain
can be removed).1 The worst outcome is in cases
where embolisation is performed after ventricular
shunt.1 In those with dural venous sinus
(sigmoid and jugular bulb) thrombosis with dural venous congestion and
supratentorial pial reflux with bone hypertrophy, development of facial venous
collaterals and epistaxis (due to nasal vein congestion) may
occur.1,9
In the chronic phase (beyond infancy), bilateral cerebral
calcifications, subependymal atrophy, pseudoventriculomegaly, and
cerebro-meningeal haemorrhage may be seen and patients manifest with mental
retardation, neurocognitive delay, seizures and neurological
deficits.1–3 Spontaneous thrombosis of
the malformation is very rare, late and
unpredictable.1
The treatment should aim at achieving normal development
without neurological sequelae.1 Before the
advent of endovascular treatment, VGAMs were fatal in 90% of patients under one
month of age and half of those between one month and one
year.2,5 Transarterial femoral embolisation
with glue (N-butylcyanoacrylate) is the endovascular modality of
choice.1,2 In neonates, adequate management of
cardiac failure is necessary to gain time, as emergency embolisation in the
neonate with severe multi-organ failure is
disastrous.1 Successful transcatheter
embolisation has been performed in neonates as
well.3,4 Some authors feel the need to perform
endovascular treatment as early as possible after aggressive medical treatment
of the cardiac failure in neonates.4 They feel
that severe neonatal cardiac failure secondary to VGAM is not an absolute
contraindication to interventional neuroradiology, provided expert neonatal
intensive care, anaesthesia and interventional neuroradiology are
available.4 However, rapid progression to
multi-organ failure despite aggressive medical treatment remains a
contraindication to intervention because of poor neurological
outcome.4
The first diagnostic and therapeutic angiogram can be
performed at five months of age.1 Repeat
sessions of embolisation may be necessary. Neurocognitive examination (yearly),
magnetic resonance imaging (MRI) (every two years), and post-embolisation
angiography or magnetic resonance angiography (yearly) are necessary for follow
up.1 Mortality is about 9% post-procedure and
the overall mortality in VGAM is 26%.1 Surgical
ligation of the fistula and radiotherapy have limited success with higher
morbidity and mortality as compared with endovascular treatment, and are not
commonly used.1 Antenatal diagnosis of VGAM is
possible by ultrasonography with colour Doppler and antenatal
MRI.2
Thrombosis in the dural venous sinuses occurs usually by the
age of five years in untreated VGAM.1
Thrombosis leads to congestion in the venous system, which may lead to
intracranial haemorrhage as a manifestation in late childhood, usually after the
age of five years.1 In the present case, the
child presented with thalamic haemorrhage at the age of three months – an
extremely rare event to occur so early in the natural evolution of a VGAM. The
possible reason for this early presentation could be that the outflow channels
of this VGAM were very narrow, which led to obstruction to the venous outflow,
thus causing venous congestion and haemorrhage. In spite of this complication,
in view of the neurological improvement, emergency endovascular treatment was
deferred. The decision to perform endovascular treatment in the therapeutic
window (at five months of age) was taken. On follow-up visits, any deterioration
in the neurological status of the child would be an indication for emergency
embolisation.
Author information:
Urmila M Chauhan, Registrar; Milind S Tullu, Lecturer; Mamta N Muranjan,
Associate Professor; Keya R Lahiri, Professor and Head of Department, Department
of Paediatrics, Seth GS Medical College and KEM Hospital, Parel, Mumbai –
400012, Maharashtra, India
Acknowledgements: We
thank Dr NA Kshirsagar, Dean of Seth GS Medical College and KEM Hospital,
Mumbai, for granting permission to submit this manuscript for
publication.
Correspondence:
Dr Milind S Tullu, ‘Sankalp Siddhi’, Block No 1, Ground
Floor, Service Road, KherNagar, Bandra (East), Mumbai – 400 051,
Maharashtra, India. Email: milindtullu@vsnl.net
References:
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