A Case of Sudden
Severe Headache and Unresponsiveness
in a 10 Year-old Boy
Case Presentation
A 10 year-old young man
was having breakfast with his family on Sunday morning. He complained
of sudden onset of headache, walked to his mother, and became unresponsive.
His father (a physician) said that he was unresponsive except for
muttering incomprehensible words when stimulated. No abnormal movements
were noticed.
Father carried the child
to the car and drove him to the emergency department. The child arrived
in the emergency department with diminished level of consciousness
but with eyes open. He would not follow commands or speak but would
look briefly at the examiner when questioned. Pulse was 50; BP 100/62;
temperature 36.3; oxygen saturation 99%. Pupils were equal, mid-position,
and responsive to light. Gaze tended to be downward. The boy would
withdraw extremities to painful stimulation; muscle tone seemed diminished.
What is your differential
diagnosis and how would you proceed?
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A Case of Sudden
Severe Headache and Unresponsiveness
in a 10 Year-old Boy
Intracranial hemorrhage in a young man secondary to
arteriovenous malformation rupture.
Intoduction
Background,
Risk Factors, and Epidemiology
Subarachnoid hemorrhages and intracranial hemorrhages
do occur in children but have different causes and treatments than
in adults.
Arteriovenous malformations are complex tangles
of abnormal arteries and veins interlinked by fistulas. The fistulas
allow high flow arterio-venous shunting causing
arterial hypotension in the surrounding areas of the brain.[1]
Many AVM’s have associated saccular or berry aneurysms.
Arteriovenous malformations occur in 1 per 100,000
children per year and are four times more common than aneurysm as
a cause of subarachnoid hemorrhage in children younger than the age
of 15 years.[2] They typically present before age 45 and only about
20% of symptomatic AVM’s manifest before age 15.[3] Their course cannot
be easily predicted and they may remain stable in size, grow, or even
regress; autopsy data suggests that as few as 12% are symptomatic
during life.[1]
Clinical Presentation
Intracranial hemorrhage is the most common clinical
presentation of an AVM.[1] Symptoms range from headache to coma depending
on the anatomic location of the arteriovenous malformation and the
size and location of the hemorrhage. The headache is typically that
of subarachnoid hemorrhage with sudden onset of a severe headache.
If intraparenchymal hemorrhage occurs, stroke-like deficit may be
present. It is thought that AVM’s account for an estimated 2% of acute
strokes.
Again, clinical manifestations of the intracranial
hemorrhage reflect the anatomic location of the mass and rate of expansion.
For cerebellar hemorrhages as in the case discussed above, the classic
clinical manifestations are inability to walk, ataxia, and headache
though there are many exceptions to this imperfect triad. Repeated
vomiting, cranial nerve abnormalities, and progressive decline in
consciousness are other findings described with cerebellar hemorrhage.
Patients may abruptly become comatose and apneic if the hemorrhage
expands in the small posterior fossa and compresses the brainstem.
Some generalities hold for cerebellar hemorrhage; generally lateral
hemorrhages are better tolerated than medial hemorrhages. Salvage
with good clinical outcome may occur even in comatose patients with
prompt neurosurgical evacuation. Neurosurgical decompression may be
life-saving in some cases. Smaller hemorrhages may be managed non-operatively.[4]
Seizures are another common initial symptom of
patients with arteriovenous malformations. The majority of seizures
are of partial or partial complex type. Headache is the presenting
symptom in another group.[1]
One series from a tertiary referral center reported
congestive heart failure from rapid arterial-venous shunting as the
presentation of the AVM in 18% of their pediatric-only population;
this was a common presentation in neonates.[3]
Finally, progressive focal neurologic deficits
(without hemorrhage) may be
another presentation. The deficits will again vary depending
on the location of the arteriovenous malformation.
Emergency department care primarily involves patient
stabilization and then identification, proper consultation, and admission.
Imaging Studies, Consultations, and Procedures
Noncontrast cranial CT is the initial imaging modality
of choice since it will identify all intraparenchymal hemorrhages
and the majority of subarachnoid hemorrhages. If the history suggests
subarachnoid hemorrhage and initial CT is unremarkable, lumbar puncture
is recommended.
Further neuroimaging studies will be obtained in
consultation with other physicians. MRI identifies vascular anomalies
but angiography is still preferred by many for finer anatomic detail
and vascular flow information.
Immediate neurosurgical consultation is recommended
with identification of the intracranial hemorrhage or arteriovenous
malformation.
Treatment issues are complex. While it is agreed
that the rate of recurrent hemorrhage is less than for aneurysmal
hemorrhage, the rate of AVM
rehemorrhage is still not clear. For patients that presented
with hemorrhage, the annual rate of rehemorrhage was found to be 18%
compared to a rate of 2% in patients with arteriovenous malformation
who presented in a manner other than hemorrhage.[1]
Once hemorrhage has occurred, treatment is generally
recommended with options including surgical resection and various
interventional angiography procedures. Some report a rapid decrease
in hemorrhage reoccurrence after the first year. Some studies suggest
that for unruptured AVM’s, surgery does not improve overall
outcomes.[5]
Arteriovenous malformations of the posterior fossa
are uncommon accounting for less than one-quarter of intracranial
AVM’s. The incidence of hemorrhage is reported to be higher with greater
morbidity for AVM’s in this location. [6]
Surgery is generally recommended for AVM’s that
have bled if they are accessible; other treatment options may include
endovascular occlusive techniques or gamma-knife therapy. Again, the
natural history of these malformations remains unclear.
Clinical course and outcome
After examination and establishment of intravenous
access, the child was taken to neuroradiology with physicians in attendance
for airway management if needed. CT confirmed a cerebellar hemorrhage
and he was admitted to the pediatric intensive care unit with neurosurgical
consultation. He was started on dexamethasone and famotidine. An MRI/MRA
later that day showed a cerebellar arteriovenous malformation with
the right superior cerebellar artery being the primary feeding vessel.
Level of consciousness was decreased on the second
hospital day and he was not following commands or opening his eyes
spontaneously; repeat CT showed hydrocephalus. A ventriculostomy was
placed but this was not followed by improvement. A suboccipital craniotomy
with resection of the AVM and clot removal was performed hours later.
Postoperative course was unremarkable; repeated angiography showed
no remnant of the arteriovenous malformation.
He was transferred to a rehabilitation hospital
for therapy of ataxia and speech difficulties; these improved over
two weeks. He currently engages in sports and performs well in school.
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Annotated
Bibliography
References tend to be either reviews or summaries
of cases at one institution. There is nothing in the way of randomized
controlled therapeutic trials for this condition.
1.
The Arteriovenous Malformation Study Group: Arteriovenous malformations
of the brain in adults. N Engl J Med. 1999; 340:1812-8.
Masterful recent summary
of literature on AVM’s from an international study group. Recommended for overview of the topic.
2.
Getzoff MB, Goldstein B: Spontaneous subarachnoid hemorrhage
in children. Pediatr Rev.
1999; 20:141.
Brief synopsis of several
recent publications with listing of caveats for detecting and managing
SAH in children.
3.
Millar CB, Bissonnette B, Humphreys RP: Cerebral arteriovenous
malformations in children. Can J Anaesth; 1994; 41:321-31.
Summary
of experience of a large tertiary referral center with emphasis on
anesthetic techniques.
4.
Huff, JS: Dr. C. Miller Fisher's description of acute cerebellar
hemorrhage. J Emerg Med;
1994; 12:521-4.
A review of Dr. Fisher’s
classic work on cerebellar hemorrhage contrasting diagnosis and treatment
changes over the last forty years.
5.
Aminoff MJ: Treatment of unruptured cerebral arteriovenous
malformations. Neurology.
1987; 37:815-9.
A
neurologist’s analysis of surgical and non-surgical treatments for
arteriovenous malformations. Articulates the argument that for AVM’s
that have not ruptured, surgical treatment offers no proven benefit.
6.
Symon L, Tacconi L, Mendoza N, Nakaji P: Arteriovenous malformations
of the posterior fossa: a report on 28 cases and review of the literature.
Brit J Neurosurg. 1995; 9:721-732.
Summary
of experience from British referral center of their experience with
posterior fossa AVM’s. These are unusual lesions and the natural history
is not clear though the authors feel posterior fossa AVM’s are at
greater risk of hemorrhage and morbidity that AVM’s in other locations.
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Questions
1.
The most common cause of spontaneous intracranial hemorrhage in children
and young adults is:
- Aneurysm
- Arteriovenous
malformation
- Tumor
- Moyamoya
disease
2.
The most common presentation of an arteriovenous malformation is:
- Hemorrhage
- Generalized
seizure
- Focal
neurologic deficit without hemorrhage
- Partial
seizure
3.
Which of the following is true regarding cerebellar hemorrhages?
- Coma
is never a feature of cerebellar hemorrhage since the cortex is
not involved
- Lateral
cerebellar hemorrhages generally do better clinically than medial
hemorrhages
- Most
cerebellar hemorrhages are from rupture of an AVM
- Most
cerebellar hemorrhages are from rupture of an aneurysm
4. If subarachnoid hemorrhage is strongly suspected, what is the diagnostic
strategy of choice?
- Immediate CT
- Immediate CT followed by lumbar puncture if CT is non-diagnostic
- MRI- perfusion-weighted if possible
- Immediate angiography
5. The natural history of AVM’s is most consistent with...
- Steady growth until rupture
- Nonprogressive mass lesion
- Increasing steal phenomena leading to seizures
- Not clearly known
Answers
1.
Answer b.
The most common cause of spontaneous intracranial
hemorrhage in children and young adults are ruptures of arteriovenous
malformations. In adults, the most common cause of non-traumatic intracranial
hemorrhage is aneurysmal rupture.
2.
Answer a.
The most common presentation of an arteriovenous
malformation is hemorrhage; seizures are the next most common presentation.
3.
Answer b.
Lateral cerebellar hemorrhages generally do better
clinically than medial hemorrhages; sometimes patients have only minimal
residual neurologic deficits after evacuation of lateral hemispheric
cerebellar hemorrhages.
4.
Answer b.
Immediate CT followed by lumbar puncture if CT
is non-diagnostic is the most common diagnostic strategy. CT is needed
to exclude intraparenchymal hemorrhage and is thought to be 90-95%
sensitive for subarachnoid hemorrhage; it is generally recommended
that LP be employed to increase diagnostic sensitivity further.
5.
Answer d.
The nature history of AVM’s is not known.
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