Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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August 24, 2017
Cerebrofacial Arteriovenous Metameric Syndrome (CAMS) Type II
- Background:
- CVMS is a term that encompasses craniofacial AVM syndromes and classifies them according to the location of AVMs. CAMS is classified into 3 subgroups:
- CAMS I: medial prosencephalic group with involvement of nose and hypothalamus
- CAMS II: lateral prosencephalic group with involvement of occipital lobe, optic chiasma, optic tract, thalamus, retina, and maxilla
- CAMS III: rhombencephalic group with involvement of cerebellum, pons, and mandible
- Our case has a rare variant of CAMS II, which involves the temporal lobe as well.
- CVMS is a term that encompasses craniofacial AVM syndromes and classifies them according to the location of AVMs. CAMS is classified into 3 subgroups:
- Clinical Presentation:
- Most cases present in childhood and adolescence, even though the brain and facial AVMs may be present since birth.
- The most common presenting symptoms are progressive visual loss, progressive neurologic deficits, and bleeding from gums/teeth.
- Cosmetic complaints like facial asymmetry and proptosis are also common.
- Seizures have also been reported as presenting symptoms.
- Key Diagnostic Features:
- Diagnosis involves thorough clinical evaluation, review of detailed patient history, and identification of characteristic findings, especially ocular findings.
- CT with contrast and/or brain MRI show dilated vessels or flow voids.
- The AVM nidus is usually diffuse and extends along the optic nerve into the orbit.
- Differential Diagnosis:
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Cerebral proliferative angiopathy: characterized by the presence of normal brain parenchyma interspersed throughout the tangle of vessels that corresponds to the nidus; no facial involvement
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Treatment:
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As the nidus is diffuse, a complete cure is usually not possible.
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Treatment strategies include palliative treatment with targeted embolization of the higher flow compartments or the angioarchitectural weak points.
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