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Port-wine stains are capillary malformations. They are the most common vascular malformation present at birth. Port-wine stains are pink or red patches that arise at birth and grow in proportion to the child's growth. They persist throughout the child's life if left untreated. The most common areas affected by port-wine stains tend to be eyelids, occipital and nuchal regions in fair-skinned newborns. The lesion is called a port-wine stain because as the patient ages the light pink macules change to a crimson red or deep purple hue (port-wine). In addition, the lesions thicken, develop blebbing and nodularity and leads to the overgrowth of the underlying soft tissue and bone. A port-wine stain can be a harbinger of Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Sturge-Weber syndrome is a neurocutaneous disorder with angiomas involving the leptomeninges (leptomeningeal angiomas) and skin of the face, typically in the ophthalmic (V1) and maxillary (V2) distributions of the trigeminal nerve. The overall risk of having Sturge-Weber syndrome if the child has a port-wine stain is 8%, however the risk is higher if the port-wine stain in larger and dependent on its location (V1, V2 distribution). Klippel-Trenaunay syndrome is characterized by a triad of port-wine stain, varicose veins, and bony and soft tissue hypertrophy as well as having an atriovenous malformation involving an extremity.

Differential Diagnosis

At birth, port-wine stains may be confused with early macular and telangiectatic presentations of hemangiomas. In the older child, port-wine stains are very characteristic and seldom confused with other entities.

Pathophysiology

All vascular malformations are considered developmental errors in vascular formation.

Treatment

The vascular-specific pulsed dye laser is the treatment of choice for port-wine stains. Response to treatment is dependent on the size of the lesion and the age of the child. The younger the child and the smaller the lesion at the beginning of treatment, the more likely it is that the port-wine stain will improve. Treatment may be started as early as 2 weeks of age, and starting treatment as early as possible is better because of the increasing size of the stain over time due to skin growth and thickening. With time untreated port-wine stains tend to become darker and more nodular. This also explains why some port-wine stains slowly recur after treatment.