How Are Port-Wine Stains Treated?

Uncategorized

Patient Presentation
A 2-week-old female came to clinic for her health supervision visit. She had been noted to have some congenital dermal melanocytosis lesions over her lower back, and also had a different lesion on her right upper extremity. Neither had been bothering the newborn and she had been breastfeeding well.

The pertinent physical exam showed an interactive female who was only 3% decreased from her birth weight. Her growth parameters were in the 10-50%. Her physical examination was normal except for some slate-grey, blush macules on her lower spine and hips. She also had a flat lesion approximately 3 cm wide and linear down the back of her right arm that skipped the elbow. It was relatively discrete but somewhat streaky in appearance and was a dark red color. She had no other obvious dermal lesions.

The diagnosis of a healthy female with congenital dermal melanocytosis and a presumed port-wine stain was made. The infant had already been referred to dermatology for lesion confirmation and potential treatment and her parents were happy with that plan.

Discussion
Port-wine stains (PWS) or birthmarks are capillary and post-capillary venule malformations which are usually congenital but can be acquired. In newborns they occur in 0.3-0.5% of births. They are pink to dark-red to purple in color, usually flat and solid across the tissue. They are persistent lesions and can develop to have hypertrophy and nodular characteristics. They can occur in any location but tend to involve the head and neck. Isolated PWS are associated with GNAQ, GNA11 and PiK3CA hot spot genetic mutations. PWS are also associated with some syndromes such as Sturge-Weber, Klippel-Trenaunay, other phakomatosis and some overgrowth syndromes. Patients with lesions that are higher risk or have other stigmata for a potential syndrome will need intradisciplinary evaluation and treatment.

Over time, many lesions in the limb and trunk distribution are stable or lighten and facial lesions may become darker, and can develop to have hypertrophy and nodular characteristics. Long-term patients can experience cosmetic and functional impairments and also intralesional pyogenic granulomas, and squamous or basal cell carcinomas.

Learning Point
Selective photothermolysis is the theory that underlies the treatment of vascular lesions using lasers. Light absorbing targets (chromophores) are heated and damaged with surrounding tissues having minimal injuries. The targets are oxyhemoglobin, deoxyhemoglobin and methemoglobin. Laser treatments carefully choose and weigh the wavelength to try to match the target, the pulse duration to the target vessels, and the fluence or energy emitted which is converted to heat to damage the vessels. Other factors that influence treatment planning and outcomes include cooling strategies, skin thickness (infants are 40-50% thinner than adults), melanin, hair follicles, lesion thickness, lesion location and extent, treatment intervals, and patient age.

Some PWS are monitored over time. Pulsed dye laser (585-595 nm wavelength) is the gold standard for PWS active treatment since the 1980s. It can be safe and effective. Treatment discomfort is often described as “snapping a rubber band against the skin” which may or may not be tolerated by infants and children. Post-treatment often there is swelling which is treated with ice. Other problems can be irritation or even mild blistering or crusting which is usually managed with bland moisturizers. Sunscreen is always recommended. Other problems after treatment include potential scarring, hyperplasia or depressions, and pigmentary changes. Pulsed dye laser penetrates about 1 mm, but PWS may be 3-5 mm which may be why some can be resistant to pulsed dye laser treatment.

Questions for Further Discussion
1. What other common vascular stains or abnormalities can you name? A review can be found here
2. What other types of physical examination signs of Sturge-Weber or Klippel-Trenaunay syndromes?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Birthmarks and Plastic and Cosmetic Surgery.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Zallmann M, Leventer RJ, Mackay MT, Ditchfield M, Bekhor PS, Su JC. Screening for Sturge-Weber syndrome: A state-of-the-art review. Pediatric Dermatology. 2018;35(1):30-42. doi:10.1111/pde.13304

Tran JM, Kelly KM, Drolet BA, Krakowski AC, Arkin LM. Light-based treatment of pediatric port-wine birthmarks. Pediatric Dermatology. 2021;38(2):351-358. doi:10.1111/pde.14503

Buch J, Karagaiah P, Raviprakash P, et al. Noninvasive diagnostic techniques of port wine stain. Journal of Cosmetic Dermatology. 2021;20(7):2006-2014. doi:10.1111/jocd.14087

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

Published