Table 1.
Overview of Quality of Life and Psychosocial Effects in Patients with PWS
| Author(s) | Country | N | Study Design | Participants | Measures Used | Outcomes Measured | Outcomes Measured | LOE † |
|---|---|---|---|---|---|---|---|---|
| Augustin et al (1998) 22 |
Germany | 76 | Cross-sectional study | Patient with PWS during FPDL therapy | Symptom Checklist 90R, ALLTAG, Chronic Skin disease Questionnaire, Frankfurt Body Image Scales, specific questionnaire for port wine stains | Emotional well-being, quality of life, body perception, disease-specific stress, expectations of therapy | Patients with PWS showed significantly higher emotional stress than healthy controls, wherein they have impaired quality of life and felt less attractive. | 3 |
| Demellweek et al (1997) 28 |
United Kingdom | 6 | Cross-sectional study | Ages 8–11 years old (grades 4, 5 and 6) | 5-point scale (agree a lot to disagree a lot) their response to each item was scored 0 to 4 | Head and shoulder videoclips of children with PWS and without, lasting 10–15 seconds | Participants thought PWS would attract staring and teasing; for the boy it would cause self-pity and, for one of the girls, it would make getting a boyfriend more difficult, but the stain did not significantly affect participants’ assessment of the attractiveness and character or willingness to interact/become friendly with the children on the video. | 4 |
| Hagen et al (2017) 23 |
USA | 244 | Cross-sectional study | 18 years or older with PWS | Skindex-29 instrument | QoL questionnaire addressing: Emotions, symptoms and functioning | PWS can negatively affect QoL especially from an emotional perspective. Factors associated with reduced QoL included comorbid depression, limited facial mobility, and presence of other skin conditions. The QoL scores were similar to those of CTCL, rosacea, alopecia, and vitiligo. | 3 |
| Hansen et al (2003) 31 |
USA | 164 | Retrospective Cross-sectional study | Patients or parents of minors with PWS who underwent PDL treatment | Patient questionnaire | Physical, social, and psychological outcomes of treatment | Majority (60%) worried less about their appearance after treatment. Overall, 48% of patients indicated satisfaction with treatment. 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. | 3 |
| Heiser et al (2020) 6 |
USA | 262 | Case-control | Case- Hypertrophic Facial PWS and laser-treated facial PWS; control- Monocular and binocular blindness | Visual analogue scale, standard gamble, time trade-off | Quality-adjusted life years | Laser-treated facial PWS showed significantly higher utility measures than untreated hypertrophic state (p < 0.001, all measures), with a difference of 3.24 quality-adjusted life years. Linear regression analysis revealed that non-Caucasian race and higher level of education were associated with lower SG and TTO utility scores for the hypertrophic facial PWS state among naïve observers. | 2 |
| Kalick et al (1981) 26 |
USA | 82 | Cross-sectional study | Patients with PWS, aged 7 to 66 years old | Eysenck Personality Inventory, Multiple Affect Adjective Checklist, State-Trait Anxiety Inventory | Neurotic tendencies, anxiety, depression, hostility | PWS patients have slightly lower average scores on all the scales (slightly lower score in extroversion, but also in neuroticism, depression, hostility, and various kinds of anxiety). The test instruments did not detect greater emotional disturbance in the PWS patient sample than in normal control populations. | 3 |
| Lanigan et al (1989) 19 |
United Kingdom | 71 | Cross-sectional study | 15 years or older with PWS | Questionnaire | Assessment of PWS, status related to laser treatment, use of cosmetic camouflage | Patients with PWS suffer a significant degree of psychological morbidity, which they suppress. | 3 |
| Malm et al (1988) 33 |
Stockholm | 23 | Cross-sectional study | Patients ages 25–60 years old, treated with argon laser, and classified either with large or small PWS | Patient interview | Discovery, social interplay, treatment expectations and realities | Patients with large PWS were found to have reality problems. Patients with small PWS showed no reality problems but experienced great individual psychological problems. | 3 |
| Masnari et al (2013) 24 |
Switzerland | 88 | Cross-sectional study | Families of a child with a visible facial difference (burn scar, infantile hemangioma, PWS or congenital melanocytic nevus) | Child Behavior Checklist, KIDSCREEN-27, TNO-AZL Preschool Quality of Life Questionnaire, and Perceived Stigmatization Questionnaire | Psychological assessment and health-related quality of life, | Psychological adjustments was within normal. In preschool children, HRQOL was good. In 7–16 years old, HRQOL was impaired including their psychological well-being. | 3 |
| Miller et al (1999) 13 |
New York | 46 | Cross-sectional study | Parents of children with Facial PWS receiving PDL treatment | Parenting Stress Index, Family Satisfaction Scale, and Parental Concerns Questionnaire | Demographic characteristics, family satisfaction, parental concerns, parents’ knowledge regarding the PWS, and parent-staff communication. | Five parents (11%) scored in the clinical range for stress. Parents of children with facial PWS reported to be in the average range for psychological stress. Factors associated with lower stress include younger children, more family cohesion and adaptation, fewer parental concerns, and greater satisfaction with parent-staff communication. | 3 |
| Schiffner et al (2002) 25 |
Germany | 36 | Cross-sectional study | Patients with facial PWS who completed laser treatments | Chronic Skin Disease Questionnaire (CSDQ) and Short Form-36 Health Survey (SF-36) | Willingness to pay, time trade-off | 92% of the respondents 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. Patients would pay an average of €16 per single treatment and €192 for the whole course of treatments. | 3 |
| Sheerin et al (1995) 27 |
Scotland | 32 | ObservationalCase-control | Categorized into case and control groups: children aged 7 to 16 years with facial PWS (n= 32) and children with PE (n=42) vs control (n=32) | Harter Self-Perception Profile, Revised Children’s Manifest Anxiety Scale, Children’s Depression Inventory, Disfigurement Perception Scale, Child Behavior Checklist | Self-perception scores, perception of disfigurement and psychosocial adjustment, behavior, depression and anxiety scores | Children with PWS functioned as well as or better than nondisfigured peers on measurements of psychosocial adjustment, while children with PE scored lower than nondisfigured peers on measures of self-perception and parent-rated social and attention problems. There was no correlation between the degree of disfigurement and level of psychosocial adjustment. | 3 |
| Troilius et al (2000) 30 |
Sweden | 163 | Retrospective Cross-sectional study | Treated four to nine times with PDL until the PWS had cleared or until there was no further improvement | Questionnaire developed with a psychiatrist | Psychosocial behavior and reactions after treatment and as they remembered them before treatment | Forty-five percent (61/135) of the patients thought they had lower self-esteem than their age group; the older the patient, the more negative was the grading of their self-esteem (P < 0.001). After the treatments their self-esteem improved significantly (P < 0.001), more so in older patients (P <0.001). | 3 |
| Troilius et al (1998) 29 |
Sweden | 259 | Cross-sectional study | Patients and families with PWS | Questionnaire developed with a psychiatrist | Self-esteem, acceptance of PWS, influence of PWS in their lives, school or work problems and relationships | 73% of the patients were mostly disturbed by PWS (ages 9–20 years). 75% answered that PWS negatively influenced their life and 61% were convinced that their life would change if their PWS could be eliminated. 47% reported suffering because of low self-esteem. According to 28%, PWS made their school life and education more difficult. After the laser treatment, all of the distress parameters were relieved. | 3 |
| van der Horst et al (1997) 20 |
Netherlands | 82 | Cross-sectional study | Children and adults with facial PWS not previously treated | RAND Health Insurance Questionnaire and Child Behavioral Checklist | Quality of life (7 categories) and problem behavior and skills in children | Adolescents and adults reported little effect on role and social functioning, but showed low scores for mental health, self-perceived health and vitality/health. Children’s parents reported no clinically significant problem on behaviour (T-score > 70). Adolescents and adults showed statistically significant negative consequences of their PWS in social contacts compared to children (P ≤ 0.01, Mann–Whitney). | 3 |
| van Raath et al (2018) 2 |
Netherlands | 108 | Cross-sectional study | Patients with PWS who had visited the laser department | Self-administered questionnaire | PWS localization, stress level, number of treatments, lesional clearance, satisfaction, willingness to participate in a new study, acceptable travel time, willingness to pay | 65% would participate in clinical studies and 49% would accept intravenous drugs. For an effective treatment, 58% was prepared to pay over €2,000 and 48% would travel more than 6 h. Travel time was inversely correlated with age, clearance rate, and satisfaction. Facial PWS patients had undergone more treatments, were less satisfied, and less willing to participate in studies or accept intravenous drugs. Stress levels were higher in females. | 3 |
| Wang et al (2017) 5 |
China | 393 | Case-control | Categorized into 2 groups: case- PWS (n= 197) and control - vitiligo (n= 196) | DLQI | QoL comparison between PWS and vitiligo (control) | DLQI scores of PWS patients were mainly distributed from 2 to 5 (52.29%) and from 6 to 10 (42.13%), which suggested that PWS had a small and moderate effect on QoL. Single-factor analysis and multiple-factor logistic regression analysis showed that the main influencing factors were female sex, skin hypertrophy, and lesion area >30 cm2 (P<0.05; odds ratio, >1). | 2 |
Note: †Based on the Oxford Centre for Evidence-Based Medicine 2011 Levels of Evidence.
Abbreviations: PWS, port wine stain; DLQI, Dermatology Life and Quality Index; QoL, quality of life; CTCL, cutaneous T-cell lymphoma; h, hour; PDL, pulsed dye laser; PE, prominent ears; FPDL, flashed lamp-pumped pulsed dye laser; ALLTAG, A questionnaire to record functional capacity in everyday life; HRQOL, health-related quality of life; SG, standard gamble; TTO, time trade-off.