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Cutaneous arteriovenous malformation in Infant/Neonate
Other Resources UpToDate PubMed

Cutaneous arteriovenous malformation in Infant/Neonate

Contributors: Rajini Murthy MD, Craig N. Burkhart MD, Dean Morrell MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Arteriovenous malformations (AVMs) are vascular anomalies where arterial and venous systems connect directly to each other, bypassing venules, arterioles, and capillaries. They are considered fast-flow anomalies with a direct connection between arteries and veins, compared to venous malformations, which are slow-flow anomalies.

AVMs most frequently occur in the intracranial circulation, in the pelvis, and extremities. Cutaneous AVMs are rarer. They typically present on the head and neck area and are often present at birth (40%), although they may not be clinically evident until later in childhood, or progressive growth may occur during puberty or pregnancy.

The majority of AVMs are sporadic. They are sometimes associated with a somatic mutation in the KRAS gene. AVMs may also be a manifestation of various syndromes, including Parkes-Weber syndrome, Cobb syndrome, capillary malformation (CM)-AVM, syndrome, and hereditary hemorrhagic telangiectasia syndrome (pulmonary, gastrointestinal, and intracranial AVMs). Underlying AVMs may also exist in the port-wine stains of adults that have developed a hypertrophic or nodular component.

AVMs may continue to enlarge over time, causing disfigurement or pain. Ulceration, infection, and hemorrhage are local complications. High-output cardiac failure is a systemic complication that may occur with extensive AVMs.

Codes

ICD10CM:
Q27.30 – Arteriovenous malformation, site unspecified

SNOMEDCT:
254780006 – Arteriovenous malformation of skin

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Port-wine stain
  • Infantile hemangioma

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Therapy

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References

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Last Reviewed:09/10/2023
Last Updated:09/19/2023
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Cutaneous arteriovenous malformation in Infant/Neonate
A medical illustration showing key findings of Cutaneous arteriovenous malformation (Progressing AVM)
Clinical image of Cutaneous arteriovenous malformation - imageId=1735267. Click to open in gallery.  caption: 'A close-up of a violaceous plaque on the chest.'
A close-up of a violaceous plaque on the chest.
Copyright © 2024 VisualDx®. All rights reserved.