A model for the assessment of cardiac problems

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murmurs by office based (general practice) paediatricians. Arch Dis Child
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IMAGES IN PAEDIATRICS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
doi: 10.1136/adc.2006.103481

Giant cerebral arteriovenous malformation

A

3-year-old girl presented with a
12 hour history of left sided
weakness. The parents reported
that she had experienced episodic partial amnesia in the preceding weeks,
being unable to recognise her favourite
cartoon characters and family friends.
The examination revealed reduced
power in the left upper and lower limb
(4/5) and unsteady gait, without other
neurological abnormalities. Prominent
dilated, pulsating veins in the right
parietal region and a audible bruit in
the same area were noted on examination (fig 1). A cranial MRI (fig 2) scan
showed a large, dysplastic arteriovenous
malformation with moyamoya-like
appearance extending throughout most
of the right hemisphere and crossing the
midline. An angiogram (fig 3) confirmed shunting between this malformation and the extracranial circulation.
The patient recovered fully from this
episode but continues to experience
transient ischaemic attacks with varying
symptoms including amnesia, mood
swings, motor deficits, and visual
impairment. The treatment options are
limited by her young age and the extent
of the malformation. Since interventional endovascular closure is deemed
to be technically unfeasible, alternatives
including microsurgery and radiosurgery are currently being considered.
Arteriovenous malformations are congenital disorders with an estimated prevalence of 0.68 per 100 000.1 Data from
postmortem examinations suggest that as
few as 12% become symptomatic during
life.2 Although the majority of cases
present before 40 years of life, symptomatic manifestation this young is
extremely rare. Intracranial haemorrhage

Figure 1 Clinical photograph showing
prominent scalp veins. Consent was obtained
for publication of this figure.

is the most common presenting feature,
followed by seizures, recurrent headaches,
and progressive neurological deficit.3
Heart failure, macrocephaly, and prominent scalp veins are less commonly seen
in this group of patients.

Figure 2 Initial MRI scan showing an extensive
vascular malformation in the right hemisphere.

M O Tebruegge, A Shrivastava
Department of Paediatrics, Southend University
Hospital NHS Trust, Westcliff-on-Sea, UK
Correspondence to: Dr M O Tebruegge,
Department of Paediatrics, Southend University
Hospital NHS Trust, Prittlewell Chase,
Westcliff-on-Sea, SS0 0RY, UK;
marc.tebruegge@southend.nhs.uk
Competing interests: None declared.

References
1 Stapf C, Mast H, Sciacca RR, et al. The New York
Islands AVM Study: design, study progress, and
initial results. Stroke 2003;34:e29–33.
2 McCormick WF. Classification, pathology, and
natural history of angiomas of the central nervous
system. Wkly Update Neurol Neurosurg
1978;14:2–7.

Figure 3 Angiogram showing the dysplastic
character of the arteriovenous malformation.
Further sequences showed shunting between
intra- and extracerebral circulation.
3 Nataf F, Schlienger M, Lefkopoulos D, et al.
Radiosurgery of cerebral arteriovenous
malformations in children: a series of 57 cases.
Int J Radiat Oncol Biol Phys 2003;57:184–95.

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