Health-related quality of life in children with congenital vascular malformations

Abstract

A cross-sectional study was performed to evaluate health-related quality of life (HRQOL) in children with congenital vascular malformations (CVM) and to investigate factors associated with an impaired HRQOL. Children (2–17 years) with CVMs who visited the HECOVAN expertise center between 2016–2018 were included. The PedsQL 4.0 Generic Core Scales were used and a score ≥ 1.0 SD below the normative mean was defined as an impaired HRQOL. Factors associated with impairment were investigated using univariate and multivariate logistic regression analysis. The median overall HRQOL was 84.8/100 (n = 207; 41% boys, 59% girls; self-reported IQR 73.9–92.4 and parent-reported IQR 71.4–92.4). Patients aged 13–17 years reported significantly worse physical functioning than those aged 8–12 years (median 84.4, IQR 71.1–93.8 versus median 90.6, IQR 81.3–96.9; p = 0.02). Parents reported a significantly lower overall HRQOL than their children (median 80.4, IQR 70.7–90.8 versus median 85.9, IQR 76.1–92.4; p = 0.001). HRQOL was impaired in 25% of patients. Impairment occurred significantly more often in lower extremity CVMs (38%, p = 0.01) and multifocal CVMs (47%, p = 0.01) compared to CVMs in the head/neck region (13%). Other associated factors included invasive management (31% versus 14%; p = 0.01), age at first treatment ≤ 5 years (48% versus 25%; p = 0.02) and ongoing treatment (38% versus 18%; p = 0.004). After correction for other factors, significance remained for lower extremity CVMs and ongoing invasive treatment.Conclusions: Overall median HRQOL was reasonable and not significantly different from the norm sample. Parental ratings were significantly lower than their children’s ratings. A quarter of the patients had an impaired HRQOL, which seemed to worsen with age. Independently associated factors included a lower extremity CVM and invasive management.What is Known:• Congenital vascular malformations could affect health-related quality of life (HRQOL).• Studies on pediatric patients are limited and either very small or in combination with adult patient series.What is New:• This study raises awareness of an impaired HRQOL in 25% of pediatric patients with congenital vascular malformations.• Associated factors included a lower extremity CVM and invasive management.