Roy G Geronemus, MD

Port-wine stains (PWSs) are a type of congenital vascular malformation estimated to occur in 0.3% to 0.5% of newborns.

 These birthmarks, which represent dilated capillaries and postcapillary venules in the dermis, appear as pink or erythematous flat patches that reflect the increased hemoglobin content in the skin.

These patches vary in size and can occur anywhere on the body, but approximately 80% of PWS lesions are present on the face and neck.

 The vast majority of PWSs are isolated findings. A facial PWS involving the distribution of the V1 (ophthalmic) branch of the trigeminal nerve, however, is a hallmark feature of Sturge-Weber syndrome, and babies with this presentation should be referred to an ophthalmologist and neurologist to screen for glaucoma and brain involvement.

Other than Sturge-Weber syndrome, which is very rare, the most significant concern for children with a PWS is the potential that they will experience stigmatization and psychosocial morbidity because of the disfiguring birthmark. Published studies document the negative emotional and quality-of-life consequences that a PWS has on affected children and how these problems are lessened by treatment that improves the appearance of the lesion.

 Although spontaneous lightening of PWSs has been reported, the typical natural history of these lesions is characterized by an increase in surface area proportional with growth of the child along with darkening and thickening with the potential for spontaneous bleeding.

When a baby is born with a disfiguring PWS, parents may be anxious for options to eliminate the lesion. Pediatricians can help these families by referring them to a dermatologist who has expertise treating PWSs in infants.

The vascular-selective pulsed dye laser (PDL) represents the gold standard treatment of PWS. Performed with proper parameters and with repeated sessions until a plateau in response is reached, the procedure has excellent safety and the potential to provide complete to near-complete clearance. With early initiation of treatment in infants, the procedure can be done as an atraumatic in-office treatment without general anesthesia.

The safety and efficacy of PDL treatment of PWS is explained by the principle of selective photothermolysis. The 595-nm wavelength of the PDL targets hemoglobin in the affected cutaneous blood vessels. Heat that is produced upon hemoglobin absorption of the laser energy results in photocoagulation and aggregation of red blood cells and subsequently necrosis of the vascular endothelial cells. Risk of collateral damage to surrounding cutaneous structures is limited by selection of a proper pulse duration that confines thermal injury to the targeted PWS vasculature.

Optimal treatment of PWS with the PDL laser involves multiple sessions that are performed until there is no further improvement. Early initiation of PDL treatment has several benefits. Importantly, available evidence indicates that earlier treatment allows for optimal outcomes, including a greater response and lower risk of recurrence.

Several factors may explain the better results that are achieved with earlier PDL treatment for PWS. Because skin thickness increases with age, penetration of the laser energy to its target- the hemoglobin in the PWS capillaries-is better in younger children. The blood in neonates also contains a greater proportion of erythrocytes, and young infants have relatively less melanin in the skin that can be a competing chromophore for absorbing the laser energy.

 In addition, changes that occur in PWSs with time (thickening, darkening, and increase in size) make achieving clearance more challenging.

Treatment in infancy also enables the laser procedure to be performed in office without general anesthesia. Because the treatment can be mildly uncomfortable-each laser pulse produces a sensation that has been likened to the feeling of a light rubber band snap or less-controversy exists over the best clinical setting for performing the procedure.

Citing concerns over comfort and the need for the child to remain still, some laser surgeons advocate performing the treatment in an operating room with the child placed under general anesthesia. Treatment with this approach, however, is expensive and time consuming, and access to an operating room staffed by an anesthesiologist with specialized training in the care of pediatric patients may be limited.

Perhaps most importantly, the safety risks associated with the use of general anesthesia in children must be considered. As noted in a warning issued by the US Food and Drug Administration (FDA) in 2016, repeated or lengthy use of general anesthetic and sedation drugs in children aged younger than 3 years may affect brain development.

 The warning was based on evidence from animal studies showing that exposure to general anesthetic and sedation drugs for more than 3 hours can cause widespread loss of nerve cells in the developing brain with resultant long-term negative effects on behavior or learning. In the warning, the FDA advised that consideration be given to delaying potentially elective surgery in young children where medically appropriate. Treatment of PWSs typically requires multiple treatments.

Because of the relative ease of holding babies immobile, infants can undergo PDL treatment for PWS without the need for general anesthesia. Use of a dynamic cooling device that emits a cryogen spray before each laser pulse significantly diminishes pain during treatment.

 Optimization of laser parameters decreases treatment time. Other strategies that have been described for enabling tolerability of PDL treatment include use of topical anesthesia, oral sucrose administration, or allowing the infant to suck on a pacifier.

Many have reported on the success of PDL treatment for PWS in infants as an in-office procedure without general anesthesia (Figure).

 A paper published in the peer-reviewed literature described the positive findings from a retrospective cohort study of in-office PDL PWS treatment without general or topical anesthesia in children aged 1 year or younger.

 Jeon and colleagues’ medical record review identified 197 infants who were treated between 2000 and 2017. The study population was comprised of 73 (37%) boys and 124 (63%) girls. The mean age of first treatment was 3.4 months, and the earliest treatment was performed in a child who was aged just 5 days.

Approximately 75% of the children were treated for a facial lesion, and 91% of the children had light skin (Fitzpatrick phototypes I-III).

. On average, the children underwent 10 treatments (range, 2 to 23). The mean treatment interval was 37 days, although the study authors recommended that patients return for treatment every 2 to 3 weeks because the more frequent interval seemed to accelerate the response and minimize the total number of treatments needed and the time until treatment was completed. Therefore, it enabled the opportunity to perform the sessions without general anesthesia and the likelihood that maximal clearing was achieved before the child entered school and risked becoming a target for teasing and bullying.

Improvement in PWS was rated by 4 independent physicians who used a 5-point visual analog scale to compare before and after photographs. The Table summarizes the results that show near-complete to complete clearing was achieved in the majority of children.

Consistent with reports favoring earlier treatment, Jeon and colleagues found that lesions that cleared completely had a smaller average size and required fewer treatments than those achieving less improvement.

 Although analyses of outcomes based on anatomic location showed that the best results were obtained when treating lesions on the first branch of the trigeminal nerve, the mean visual analog scale (VAS) grade was 3.65 in analyses of all lesions as well as for all facial and all nonfacial PWSs. Despite the ability to achieve good results regardless of PWS location, guarantees about the outcome are never given, and when the PWS is below the elbow or knees, the family is informed about the potential for less improvement when treating birthmarks here.

Transient purpura and mild swelling are the most common adverse effects of PDL treatment for PWS. These reactions generally persist for 4 to 7 days. Skin pigmentary changes are possible, but the risk is limited by selection of appropriate laser settings, use of photoprotection, and, in patients with darker skin, extending the interval between treatment sessions. The researchers encountered no cases of scarring or permanent pigmentary changes in their study of 197 children who were aged 1 year or younger when starting PDL treatment for PWS.

Treatment of PWSs with the PDL can be initiated within the first few days after birth as an in-office procedure. This approach to early intervention may relieve parental anxiety about the baby’s appearance, avoids exposing the child to the risks of general anesthesia, and enables the best cosmetic outcome with fewer sessions. Therefore, it reduces the likelihood that a child born with a PWS will experience the psychosocial morbidity that can be the most significant complication of these lesions.

Help with referrals to a specialist with experience treating PWSs in infants and children can be obtained through the Vascular Birthmarks Foundation (

 and the American Society for Dermatologic Surgery (

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. Published March 3, 2018. Accessed August 12, 2019.

15. Waldorf HA, Alster TS, McMillan K, Kauvar AN, Geronemus RG, Nelson JS. Effect of dynamic cooling on 585-nm pulsed dye laser treatment of port-wine stain birthmarks. 

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Articles

When a baby is born with a disfiguring port wine stain, parents may be anxious for options to eliminate the lesion. Laser treatment is an effective option, and it is best begun early.

Roy G Geronemus, MD

Articles

Port wine stains in infants: Optimizing outcomes with early treatment