case presentation…

Sudden Headache Sudden Headache and Unresponsivenessand Unresponsiveness

in a 10 year-old boyin a 10 year-old boy

J. Stephen Huff, MD, FACEPEmergency Medicine and NeurologyUniversity of Virginia Health System

Charlottesville, Virginia

J. Stephen Huff, MD

Case presentation…

A 10 year old young man was having breakfast with his family. 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.

J. Stephen Huff, MD


Father carried the child to his 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….

J. Stephen Huff, MD


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 the extremities to painful stimulation; muscle tone seemed diminished. He did not speak but would briefly look at people in the room….

J. Stephen Huff, MD

What is your differential diagnosis?What is your differential diagnosis?

Anatomic?

Pathophysiologic?

Etiologic?

J. Stephen Huff, MD


Pathophysiologic / Etiologic?

Subarachnoid hemorrhage

Intracranial hemorrhage

J. Stephen Huff, MD


What is causing the lesion?

Aneurysm?

Arteriovenous malformation?

Leukemia / blood dyscrasia?

Medications?

Tumor?

J. Stephen Huff, MD

What is your management plan?What is your management plan?

Stabilization

A, B, C’s

Diagnostic plan?

J. Stephen Huff, MD


Stabilization

A, B, C’s

Diagnostic plan?

Physician accompanies patient to CT with proper equipment…

J. Stephen Huff, MD

J. Stephen Huff, MD

What is your CT diagnosis?

J. Stephen Huff, MD

Cerebellar hemorrhage

J. Stephen Huff, MD


Stabilization

A, B, C’s….

What now?

J. Stephen Huff, MD

CT: Cerebellar hemorrhageCT: Cerebellar hemorrhage

Clinical--

Risk of sudden deterioration

brainstem compression

“Neurosurgical emergency”…

J. Stephen Huff, MD

Cerebellar hemorrhageCerebellar hemorrhage

May be life threatening…

Expanding mass posterior fossa…

Brainstem compression…

Sudden coma and apnea…

Salvage possible…

Lateral (hemispheric) better…

J. Stephen Huff, MD


Stabilization

A, B, C’s….

Neurosurgical consultation…

PICU….

J. Stephen Huff, MD

J. Stephen Huff, MD

Arteriovenous malformationsArteriovenous malformations

Posterior fossa AVM

Origin- R superior cerebellar artery

Drain-hemispheric veins

Options ?

J. Stephen Huff, MD


Inpatient course…

Diminished level of consciousness

Repeat CT-hydrocephalus

J. Stephen Huff, MD

Inpatient courseInpatient course

Ventriculostomy….

Resection of AVM…

Good clinical outcome….

J. Stephen Huff, MD

DDX: Pediatric intracranial DDX: Pediatric intracranial hemorrhagehemorrhage

Aneurysms

Blood dyscrasias

Tumors

Medications

Arteriovenous malformations

J. Stephen Huff, MD

Brain slice showing cross-Brain slice showing cross-section of large AVMsection of large AVM

J. Stephen Huff, MD

Brain slice showing intraparenchymal Brain slice showing intraparenchymal hemorrhage from AVMhemorrhage from AVM

J. Stephen Huff, MD


Tangle of abnormal arteries and veins

Linked by fistulas

High-flow AV shunting

Lacks capillary bed

Congenital

Course not predictable….

J. Stephen Huff, MD


Variety of architectures

Some small, close to surface

Others large, wedge-shaped

straddle vascular border zones

extend to ventricular wall

Associated aneurysms - about half

J. Stephen Huff, MD


Present before age 40

Only 12% symptomatic

2% of strokes“Advances in…therapy…have come at a faster

pace than information on the natural history…associated morbidity…and risks of invasive therapies.”

The Arteriovenous Malformation Study Group: Arteriovenous malformations of the brain in adults. N Engl J Med. 1999;340:1812-8.

J. Stephen Huff, MD


Presentations

Hemorrhage most common

Seizures

Partial

Generalized

Headache - nonspecific

Focal neurologic deficitsThe Arteriovenous Malformation Study Group: Arteriovenous malformations of

the brain in adults. N Engl J Med. 1999;340:1812-8.

J. Stephen Huff, MD


Re-hemorrhage

-18% risk (vs. 2%) annual risk

-may decline with time

Posterior fossa AVM’s

-about 7-18% of all AVM’s

higher hemorrhage risk?

higher morbidity?Symon L, et al: Arteriovenous malformations of the posterior fossa: a report on

28 cases and review of the literature. Brit J Neurosurg. 1995; 9:721

J. Stephen Huff, MD


Treatment - (or not?)

Surgery

if superficial, non-eloquent

Interventional radiology

coils, glues, balloons

Radiation therapy, gamma-knife

Aminoff MJ: Treatment of unruptured cerebral arteriovenous malformations. Neurology. 1987; 37:815

J. Stephen Huff, MD


Controlled trials of therapy lacking…

Natural history unclear…

Goal: complete removal AVM

For symptomatic AVM’s

-hemorrhage=>surgery if small

-other therapies if larger

If asymptomatic?

J. Stephen Huff, MD

SAH and ICH can occur in children and young adults

Different causes and treatments than in adults…

AVM likely cause, not aneurysm

Take home messages:Take home messages:

J. Stephen Huff, MD

Questions?Questions?

case presentation…

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