Vascular Malformations

About vascular malformations

Vascular malformations may refer to a malformation of any vessel type (arterial, venous, lymphatic, capillary), but in dermatology it usually refers to a capillary malformation (a malformation of the smallest blood vessels).  A capillary malformation is often also called a port-wine stain or a nevus flammeus.  A capillary malformation is present at birth, often on the head or neck,  and grows proportionately with the child.  This is in distinction to a hemangioma, which has a rapid growth phase followed by involution.  Capillary malformations do not involute.  Capillary malformations tend to progress from a flat pink in infancy to red in childhood and purple in adulthood, often attaining a cobblestoned thickened texture.

An isolated capillary malformation typically does not result in any morbidity or mortality, but it can be disfiguring, especially on the face.  It is estimated to occur in 0.3-0.5% of newborns in the United States. Capillary malformation may be associated with other disorders.   Ocular (especially glaucoma) and/or CNS involvement may occur, notably for lesions that involve the V1 or V2 nerve distributions in the face.

There are a number of syndromes associated with capillary malformation, notably Sturge-Weber Syndrome (capillary malformations involving the upper face and the ipsilateral leptomeninges and  cerebral cortex), Klippel-Trenaunay Syndrome (capillary malformation, varicose veins, and hypertrophy of underlying tissues), and Cobb Syndrome (cutaneous vascular lesion overlying the spine associated with vascular malformations in the underlying spinal meninges), among several other associated syndromes.

Additional dermatological abnormalities may be present, a finding termed Phakomatosis pigmentovascularis. There are several varieties of associations, including various combinations of epidermal nevus, dermal melanocytosis, nevus anemicus, and nevus spilus.

With what can a vascular malformation be confused?

A capillary malformation may be misdiagnosed as an infantile hemangioma.

How is a vascular malformation diagnosed?

A capillary malformation is typically diagnosed by clinical exam and history.  If Sturge-Weber Syndrome or Cobb Syndrome is suspected, imaging is usually performed.

How is a vascular malformation treated?

The pulsed-dye laser is the treatment of choice for isolated capillary malformations.

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