Abnormal elastin and collagen deposition is present in extracranial arteriovenous malformations: A comparison to intracranial disease.
2019
Background. Vascular malformations are
characterized by anomalous vascular channels with
fragile walls and a propensity to bleed. Arteriovenous
malformations (AVMs) in particular have disorganized
vascular spaces with intervening fibrosis.
Characterization of the structural abnormalities of these
vessels has not been comprehensively evaluated. We
hypothesize that AVMs are likely to demonstrate altered
elastic and collagen fiber organization and distribution,
reflecting their fragility, vascular instability, and
abnormal development.
Methods. Fifteen AVMs were histologically
evaluated by H&E, elastin and trichrome staining. To
identify potential differences between extracranial and
intracranial AVMs, 5 AVMs were harvested from the
brain (n=5) and 10 from extracranial sites involving the
skin and deep soft tissue (n=10).
Results. The elastin staining demonstrated
reduplication, fragmentation and disruption of internal
elastic lamina as well as irregular thickness, and
inconsistent vascular density of all AVM specimens.
Trichrome staining revealed thickening of the intimal
layers of AVM arteries and demonstrated an irregular
thickness of venous walls within the malformation and
some areas of medial degeneration. Intracranial AVMs
are characterized by more intramural inflammation with
predominant neutrophil and lymphocyte infiltration. In
contrast, extracranial AVMs display more extravascular
inflammation with mast cell and neutrophil infiltration.
Microvascular proliferations intervening between larger
blood vessels were also noted in both types of AVMs,
but more obvious in extracranial AVMs.
Conclusion. These observed histologic anomalies of
AVMs demonstrate disorganized deposition of elastin
and collagen that point to the clinically observed
vascular instability and fragility of these lesions.
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