Treating pediatric port-wine stains in aesthetics.

A port-wine stain (PWS) is a vascular birthmark present in 0.3% to 0.5% of newborns. If untreated, this erythematous patch will grow proportionally with the child to thicken and darken with age. PWSs have implications for the child's quality of life for many years, with cosmetic, medical, and psychosocial disability. Controversy exists in many aspects surrounding laser treatment of these birthmarks in the pediatric population. We have reviewed the clinical features as well as the historic and current laser treatment of PWS. We have also examined the current hot topics of debate surrounding the treatment of PWS in the pediatric population. These controversies include the patient age of treatment initiation, the long-term psychologic impact, the use of general anesthesia, the application of eye shields, and alternative treatments for recalcitrant PWS. We have concluded with a discussion on the future directions of management and treatment.

Treatment Update of Port-Wine Stain: A Narrative Review.

BACKGROUND: Port-wine stain (PWS) is a congenital vascular malformation affecting 0.3–0.5% of normal population. These characteristic lesions arise due to the interplay of vascular, neural, and genetic factors. Treatment options include lasers, cosmetic tattooing, electrotherapy, cryosurgery, derma-abrasion, and skin grafting; however, none of these treatment alternatives appears to be satisfactory and is unable to provide consistent, satisfactory responses or even complete cures. Currently, laser is the treatment of choice, as it is comparatively safe and more effective than other procedures. The most commonly used modality is pulsed dye laser (PDL). The literature research includes peer-reviewed articles (clinical trials or scientific reviews). Studies were identified by searching electronic databases (MEDLINE and PubMed) to January 2020 and reference lists of respective articles. Only articles published in English language were included. J Drugs Dermatol. 20(5): doi:10.36849/JDD.5005.

Consensus Statement for the Management and Treatment of Port-Wine Birthmarks in Sturge-Weber Syndrome.

Importance: Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome involving the skin, brain, and eyes. Consensus recommendations for management are lacking. Objective: To consolidate the current literature with expert opinion to make recommendations that will guide treatment and referral for patients with port-wine birthmarks (PWBs). Evidence Review: In this consensus statement, 12 nationally peer-recognized experts in dermatology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included risk stratification, optimum treatment strategies, and recommendations regarding light-based therapies. A systematic PubMed search was performed of English-language articles published between December 1, 2008, and December 1, 2018, as well as other pertinent studies identified by the expert panel. Clinical practice guidelines were recommended. Findings: Treatment of PWBs is indicated to minimize the psychosocial impact and diminish nodularity and potentially tissue hypertrophy. Better outcomes may be attained if treatments are started at an earlier age. In the US, pulsed dye laser is the standard for all PWBs regardless of the lesion size, location, or color. When performed by experienced physicians, laser treatment can be safe for patients of all ages. The choice of using general anesthesia in young patients is a complex decision that must be considered on a case-by-case basis. Conclusions and Relevance: These recommendations are intended to help guide clinical practice and decision-making for patients with SWS and those with isolated PWBs and may improve patient outcomes.

Tratamiento con Pulsed Dye Laser de mancha de vino oporto: seguimiento a 10 años / Treatment with Pulsed Dye Laser of wine stain Porto: 10-year follow-up

Las manchas de vino de oporto son un tipo de malformación capilar que afecta del 0,3 al 0,5% de la población. Están presentes desde el nacimiento como máculas o placas eritematosas en la piel o mucosas. Sin tratamiento, las lesiones tienden a oscurecerse con la edad, tornándose rojizas o color púrpura, y pueden desarrollar engrosamiento nodular o un granuloma piógeno asociado. Los tratamientos con láser proporcionan mejoría mediante la destrucción selectiva de la vasculatura. Se han utilizado una variedad de láseres vasculares selectivos, pero el tratamiento de primera elección es el láser de colorante pulsado. Los mejores resultados se obtienen cuando el tratamiento es instaurado tempranamente. (AU)

Port wine stains are a type of vascular malformation that affects 0.3% to 0.5% of the population. They are present from birth as erythematous macules or plaques on the skin or mucous membranes. Without treatment, these lesions tend to darken with age, becoming reddish or purple and may develop nodular thickening or an associated pyogenic granuloma. Laser treatments might provide an improvement by selective destruction of the vasculature. A variety of selective vascular lásers may be employed, with the pulsed dye laser as the gold standard treatment. Better results are obtained when the treatment is established early. (AU)

Quality of life in adults with facial port-wine stains.

BACKGROUND: Facial port-wine stains (PWS) are considered by some an aesthetic skin problem, yet impact on quality of life (QoL) has not been objectively documented. OBJECTIVE: We sought to (1) characterize the effect of PWS on QoL in adults, (2) to identify the clinical and demographic factors that affect QoL, and (3) to compare our results with QoL studies in other skin conditions. METHODS: In total, 244 adults with facial PWS completed an online QoL survey, which included the Skindex-29 instrument. RESULTS: QoL in adults with facial PWS was diminished, especially from an emotional perspective. Variables associated with reduced QoL in all Skindex-29 subdomains included comorbid depression, limited facial mobility, and presence of other skin conditions. Persons with hypertrophy had more emotional and symptomatic impairment. The composite dermatologic-specific QoL scores were similar to those of cutaneous T-cell lymphoma, rosacea, alopecia, and vitiligo. LIMITATIONS: Selection bias was a potential limitation, as participants were primarily recruited from patient support groups. CONCLUSION: Our analysis demonstrates that the presence of a facial PWS has a significant negative impact on QoL. Dermatologists caring for patients with PWS should inquire about QoL, provide appropriate support and resources, and consider QoL when discussing treatment options and obtaining authorization for these procedures.

Analysis of quality of life and influencing factors in 197 Chinese patients with port-wine stains.

Port-wine stains (PWS) are congenital capillary malformations, usually occurring on the face, neck, and other exposed parts of the skin, that have serious psychological and social impact on the patient. Most researchers focus on the treatment of PWS, but the quality of life (QoL) of PWS patients is seldom researched. The objective of this study is to evaluate the QoL of patients with PWS on exposed parts and explore the factors influencing the QoL of PWS patients. The QoL of 197 cases with PWS on exposed parts were prospectively studied using the Dermatology Life Quality Index questionnaire (DLQI), and the factors influencing the patients' QoL were analyzed by single-factor analysis and multiple-factor logistic regression analysis. The reliability and validity of the QoL of PWS patients were then assessed by DLQI. A total of 197 valid questionnaires were collected. The DLQI scores in PWS cases ranged from 2 to 16, with 2 to 5 in 52.29% (103/197), 6 to 10 in 42.13% (83/197), and 11 to 20 in 5.58% (11/197). The main score elements of the DLQI focused on symptoms and feelings, daily activities, and social entertainment. Single-factor analysis and multiple-factor logistic regression analysis showed that the main influencing factors were female sex, skin hypertrophy, and lesion area >30 cm. The inter-item correlation averaged 47.46% and the Cronbach α was 0.740, indicating high internal consistency. Correlation of the 6 dimensions of the DLQI questionnaires with the total scores showed that the Spearman correlation coefficient r ranged from 0.550 to 0.782 (P < .001), with symptoms and feelings having a correlation coefficient of 0.782 and a high correlation with total scores. This study shows that PWS has mild to moderate influence on the QoL of most patients, mainly on daily activities, social entertainment, and feelings.

Stigmatization predicts psychological adjustment and quality of life in children and adolescents with a facial difference.

OBJECTIVES: This cross-sectional study assessed psychological adjustment and health-related quality of life (HRQOL) in children and adolescents with congenital or acquired facial differences and identified potential predictors of adjustment. METHODS: Data were obtained from 88 children, ages 9 months to 16 years, by means of parent questionnaires (n = 86) and standardized interviews with children ≥7 years old (n = 31). Evaluation measures included the Child Behavior Checklist (CBCL), KIDSCREEN-27, TNO-AZL Preschool Quality of Life Questionnaire (TAPQOL), and Perceived Stigmatization Questionnaire. RESULTS: Psychological adjustment, as measured by the CBCL, was within norms. Parent-reported HRQOL was good in preschool children. Parent- and self-reported HRQOL of participants 7-16 years old was impaired in several dimensions, including psychological well-being. Psychological adjustment (especially internalizing behavior problems) and HRQOL were predicted primarily by perceived stigmatization. CONCLUSIONS: Identification of stigma experiences and appropriate support may be crucial to enhancing psychological adjustment and quality of life in children with facial disfigurement.

Pulsed dye laser vs. intense pulsed light for port-wine stains: a randomized side-by-side trial with blinded response evaluation.

BACKGROUND: Pulsed dye lasers (PDLs) are considered the treatment of choice for port-wine stains (PWS). Studies have suggested broadband intense pulsed light (IPL) to be efficient as well. So far, no studies have directly compared the PDL with IPL in a randomized clinical trial. OBJECTIVES: To compare efficacy and adverse events of PDL and IPL in an intraindividual randomized clinical trial. METHODS: Twenty patients with PWS (face, trunk, extremities; pink, red and purple colours; skin types I-III) received one side-by-side treatment with PDL (V-beam Perfecta, 595 nm, 0.45-1.5 ms; Candela Laser Corporation, Wayland, MA, U.S.A.) and IPL (StarLux, Lux G prototype handpiece, 500-670 and 870-1400 nm, 5-10 ms; Palomar Medical Technologies, Burlington, MA, U.S.A.). Settings depended on the preoperative lesional colour. Treatment outcome was evaluated by blinded, clinical evaluations and by skin reflectance measurements. RESULTS: Both PDL and IPL lightened PWS. Median clinical improvements were significantly better for PDL (65%) than IPL (30%) (P = 0.0004). A higher proportion of patients obtained good or excellent clearance rates with the PDL (75%) compared with IPL (30%) (P = 0.0104). Skin reflectance also documented better results after PDL (33% lightening) than IPL (12% lightening) (P = 0.002). Eighteen of 20 patients preferred to receive continued treatments with PDL (P = 0.0004). No adverse events were observed with PDL or IPL. CONCLUSIONS: Both the specific PDL and IPL types of equipment used in this study lightened PWS and both were safe with no adverse events. However, the PDL conveyed the advantages of better efficacy and higher patient preference.

Autism with facial port-wine stain: a new syndrome?

The hallmark of Sturge-Weber syndrome is leptomeningeal angiomatosis. Over 15 years, four children were identified (2 boys, age 2.9-6 years) with unilateral facial port-wine stain, referred for presumable Sturge-Weber syndrome but who were also autistic. Computed tomography and magnetic resonance imaging scans failed to show evidence of leptomeningeal angioma in all four children. Three of the children had a history of seizures. Detailed neuropsychologic testing of three children revealed a similar presentation, characterized by developmental disturbance, particularly involving delayed onset of language, and early-emerging social atypicality. Positron emission tomography scanning of cerebral glucose metabolism revealed hypometabolism in the bilateral medial temporal regions, anterior cingulate gyrus, frontal cortex, right temporal cortex, and cerebellum. The pattern of glucose hypometabolism differed from that of 12 children with infantile autism (age 2.7-7.9 years) who had mild left medial temporal but more severe right temporal cortical hypometabolism and showed a reversal of normal frontotemporal asymmetry of glucose metabolism. Unilateral facial port-wine stain and autism with no intracranial angioma on conventional imaging may represent a rare clinical entity distinct from both infantile autism and previously described variants of Sturge-Weber syndrome.

Long-term psychological impact and perceived efficacy of pulsed-dye laser therapy for patients with port-wine stains.

BACKGROUND: The rationale for treatment of patients with port-wine stain (PWS) birthmarks has focused on the belief that eventual soft-tissue hypertrophy and negative psychological effects might be diminished or avoided. Pulsed-dye laser (PDL) therapy has become widely accepted as the treatment of choice for PWS, and a variety of studies have been conducted that evaluate its short-term efficacy. Long-term data regarding outcome and patient satisfaction are lacking. OBJECTIVE: Our objective was to evaluate the long-term efficacy of pulsed-dye laser therapy from the patient's perspective. METHODS: A survey was mailed to all patients or parents of minor patients whose last known laser treatment was performed between January 1, 1989, and January 1, 1996. Patients were asked to quantify changes in their PWS as well as their psychological well-being. A formal qualitative analysis was performed on their written comments as well. RESULTS: A total of 164 surveys were mailed to the most recent address available by the patient record. Fifty-two surveys were returned as undeliverable, and 55 evaluable surveys were returned, yielding a 49% overall return rate for patients who could be located. The mean age of respondents was 28.8 years, 96% of whom were white and 62% female. The mean number of years since last treatment was 7.04. The vast majority of patients noted little or no change in texture, height, or dimension of their PWS, whereas 62% noted color improvement. A majority or patients (60%) worried less about their appearance after treatment, whereas a similar number (61%) believed their ability to make friends or meet others was unaffected by treatment. Only 19% thought others looked at or treated them differently because of their PWS. Overall, 48% of patients indicated satisfaction with treatment, 24% dissatisfaction, and 28% neutral. On a 10-point scale indicating their likelihood of recommending treatment to someone similarly affected, the mean score was 7.42. Men were significantly and consistently less satisfied with treatment than women, despite rating the degree of color improvement similarly to women. Qualitative analysis of patient comments helped validate quantitative data and revealed gender differences in satisfaction, as well as correlations with adverse events from treatment, desire for additional treatment, and a trend toward more positive comments with the passage of time. CONCLUSION: The pulsed-dye laser improves the color of PWS over long periods of time in a majority of patients. Patients tended to worry less about their appearance after treatment, although most believed treatment did not substantially affect their relationship with others or others' view of them. Most patients were satisfied or neutral with regard to satisfaction with therapy and would recommend treatment to others. A minority of patients was dissatisfied with treatment, and men were more likely to be dissatisfied. Additional long-term and prospective studies will be helpful in assessing the physical and psychosocial impact of PDL for PWS.

Willingness to pay and time trade-off: useful utility indicators for the assessment of quality of life and patient satisfaction in patients with port wine stains.

BACKGROUND: For cost utility analyses in health economic research it is necessary to assess quality of life for content validation. Previously, both quality of life questionnaires and utility indicators such as willingness to pay and time trade-off have been used successfully in patients with chronic skin diseases, such as psoriasis vulgaris or atopic eczema. OBJECTIVE: For the first time to assess willingness to pay and time trade-off in patients with port wine stains, and to study possible correlations between these indicators, patient income, quality of life questionnaires and subjective or objective outcome measures. METHODS: The results of a survey on 36 patients with facial lesions who had completed laser treatment were used for assessments. The 'Chronic Skin Disease Questionnaire' (CSDQ) and the 'Short Form-36 Health Survey' (SF-36) were used as quality of life questionnaires. Special questions were used as a basis for calculating the utility indicators, willingness to pay and time trade-off. RESULTS: Completed questionnaires were sent back by 25 patients (69%). Willingness to pay and time trade-off were used as instruments for the assessment of quality of life [questions answered by 23 (92% of respondents)]; patients were willing to pay 11.8% of their monthly income and would offer a mean value of 1.2 h per day for an imaginary therapy leading to complete cure of the skin problem. Statistical correlation analyses were not performed because this pilot study included only a small number of patients. Frequency distributions show no apparent correlation between willingness to pay and patients' profession (used as a surrogate for income). Possible correlations were seen between willingness to pay, time trade-off, the scales 'anxiety/avoidance' and 'helplessness' of the CSDQ as well as 'social function' of SF-36 and subjective or objective efficacy. Twenty-nine patients (80%) were willing to pay for the treatment, a surrogate measure for patients' satisfaction with treatment modality. Overall, patients would pay an average of 16.0 euros per single treatment and 192.0 euros for the whole course of treatments. In contrast with patients who evaluated treatment modality as excellent or very good (willingness to pay for single treatment, euro 22.0; for whole treatment, 270.0 euros), patients who judged treatment good (8.0 euros, 145.0 euros), moderate or bad (12.0 euros, 146 .0 euros) were willing to pay markedly less. CONCLUSIONS: (i) The questions for willingness to pay and time trade-off were understood by most patients and produced meaningful answers. (ii) There were apparent correlations between the utility indicators, subjective or objective outcome and the scales of the quality of life questionnaires. (iii) The results show that these indicators have a high potential for use in this dermatological condition, especially as a basis for health economic evaluations.

Undisclosed port-wine stain--anesthetic implications and psychosocial considerations: a case report.

Port-wine stains (nevus flammeus, port-wine nevus) are congenital vascular lesions that have psychological and physiological implications for patient care. The location and size of these lesions can lead to complications related to anesthetic management. Facial lesions have the most profound psychological effect on the patient with respect to behavior alterations and reluctance to disclose the lesion's presence. Covering makeup has become increasingly effective not only in concealing the lesion, but also in being nearly undetectable during routine examination. Issues such as agent choice, surgical position, frequency of positioning evaluation, and choice of intravenous fluid should be considered when caring for a patient with a port-wine stain. In this case study the authors describe the anesthesia implications and outcome in a patient who refused to disclose her port-wine lesion in the preoperative interview and who ignored preoperative instructions to remove all makeup before presenting for surgery.

A prospective study of the impact of laser treatment on vascular lesions.

BACKGROUND: Vascular lesions, especially on exposed sites, can be unsightly and may cause significant psychological distress. Lasers are effective in treating such lesions, but relatively few studies have been performed looking at psychological scoring before and after laser therapy. OBJECTIVES: To assess the change in psychological distress in patients with vascular lesions following laser treatment. PATIENTS AND METHODS: A prospective study was performed with patients recruited over a 3-month period. Psychological distress was measured using subjective scores on a standard questionnaire before treatment and at a 6-month follow-up. Each patient was treated every 4-8 weeks with an appropriate laser by the same dermatologist until discharge. Forty-two patients were recruited with one of four diagnoses: telangiectasia, port wine stain (PWS), vascular spider or cherry angioma, which occurred mainly on facial or exposed sites. RESULTS: Following laser treatment, there was a significant decrease in subjective scores of patients with telangiectasia and vascular spiders. Patients with PWS showed objective improvement but this was not reflected in their subjective scores. Psychological distress had reduced significantly in patients with less severe vascular lesions. CONCLUSIONS: Laser treatment of minor vascular lesions leads to objective improvement, which is paralleled by psychological benefit, but objective benefit in PWS may not be perceived as beneficial by patients.

Patients with port-wine stains and their psychosocial reactions after photothermolytic treatment.

BACKGROUND: Since the pulsed dye laser (PDL) was introduced in the late 1980s the question has been raised whether one should treat congenital capillary malformations, commonly called port-wine stains (PWSs), early or not. OBJECTIVE: There are pros and cons, but studies have mainly emphasized the clinical treatment results. The psychosocial importance of an early treatment needs also to be clarified. METHODS: This retrospective study includes 163 patients with PWS treated with the PDL until there was either total clearing or until there was no further improvement. When a period from a few months to 8 years had elapsed after the treatments, the patients were given a questionnaire, which had been developed in cooperation with a psychiatrist, in order to evaluate the psychosocial implications of the PWS and the consequences of treatment. RESULTS: . Eighty percent of the patients more than 7 years of age had not fully accepted their PWS with increasing age and 80% thought that their life would change for the better if their PWS could be eliminated. Eighty-five percent thought their PWS influenced their life in some negative way. Forty-five percent of the patients considered themselves to have a lower self-esteem than their own age group. The majority of the patients considered themselves negatively influenced by their PWS. Most psychosocial parameters, such as self-esteem, contact with the opposite sex, social relationships, school contacts, the need to cover their PWS with make-up or clothes, and meeting new people, improved significantly after PDL treatment. Older patients had higher negative scores than the younger ones, indicating the advantage of early treatment. Patients who were treated earlier with other methods producing less favorable results scored unfavorably compared to previously untreated patients. CONCLUSION: Patients with PWS treated at our clinic consider their psychosocial status to be improved after treatment. An early treatment seems to be favorable for these patients.

Stress and family satisfaction in parents of children with facial port-wine stains.

A cross-sectional survey was employed to assess parenting stress, family satisfaction, and parental concerns and to determine predictors of stress in parents of children with port-wine stains (PWSs). The participants were 46 parents of 24 children receiving treatment with pulsed dye laser photocoagulation for facial PWS at an outpatient dermatology clinic based at a university medical center. Outcome measures used were self-report instruments assessing psychosocial adjustment (Parenting Stress Index, Family Satisfaction Scale, and Parental Concerns Questionnaire). As a group, parents scored in the average range on the stress and family satisfaction measures when compared with a normative sample; five parents (11%) scored in the clinical range for stress. Forty-nine percent of the variance in parenting stress was accounted for by four variables: the child's age (beta = 0.34; p = 0.031), the parents' degree of family satisfaction (beta = -0.27; p = 0.077), the level of parental concern regarding the child's facial PWS (beta = 0.45; p = 0.005), and the parents' satisfaction with staff communication (beta = -0. 51; p = 0.002). The data suggest that while, as a group, parents of children with a facial PWS report to be in the average range for psychological stress, some do not fare as well as others. Factors associated with lower stress include younger children, more family cohesion and adaptation, fewer parental concerns, and greater satisfaction with parent-staff communication. The potential for the development of medical complications and psychological problems over time suggests the need for treatment of the PWS at an early age. Health care providers should be prepared to screen for clinical levels of distress and to refer parents for psychological intervention when needed.

Potential psychological benefits from early treatment of port-wine stains in children.

There is a commonly held conception among referring doctors that very small children with congenital capillary malformations, so-called port-wine stains (PWS), should not be treated until they are older. Our experience leads us to believe that the flashlamp pulsed dye laser is a safe and effective treatment even for infants. We have not encountered any persistent pigmentation changes, post-treatment scarring or other adverse effects. It is important to quantify the psychological disabilities associated with this disorder to assess the need for and the benefits of treatment. Questionnaires were distributed to 259 patients and their families who visited our clinic because of their PWS. Patients who were on the waiting list for laser treatment, undergoing treatment or had completed their treatment received different questionnaires. The response rate was 89%. High emotional distress was encountered. During the age period 10-20 years, 73% (125 patients) were most disturbed by their PWS. That the PWS influenced their life negatively was experienced by 75% (171 patients), and 62% (106 patients) were convinced that their life would change radically if their PWS could be eliminated. Suffering from low self-esteem (in comparison with the same age group) was reported by 47% (87% patients). The PWS made their school life and education more difficult according to 28% (51 patients) of the sample. Of the families of patients, 76% (106 relatives) considered the patient to be negatively affected in some way by the PWS. After the laser treatment, all of these distress parameters were significantly relieved, together with a need to cover their PWS, their fear of going into conflict or quarrels, their social relationships, problems with the opposite sex, rage attacks, depressions and abnormal reactions from their peers. We believe there is potential psychological benefit in starting the treatments of PWS (including non-facial) at as early an age as possible.