Quality of life among children and adolescents with neurofibromatosis 1: a systematic review of the literature

The aim of this research is to identify, within a systematic review, aspects of quality of life (QoL) that are adversely affected in children and adolescents with neurofibromatosis (NF), and to report predictors of quality of life in this population. Published reports of original research were included if they described QoL in children and/or adolescents with NF, and met methodological quality according to a list of predefined criteria. Seven studies conducted between 2006 and 2013 met inclusion criteria. All seven studies examined patients with NF1 and reported that these patients have lower general QoL compared to population norms. Parents’ proxy ratings of QoL were generally lower than children’s self-report ratings. By parent proxy, familial NF1 was a strong protective factor for QoL, while the opposite was found by child report. By parent proxy, male sex was significantly associated with lower scores on the parental time impact of QoL. Skin-related QoL was only slightly altered in this population, vision-specific QoL was impacted only in patients with vision loss. The majority of findings regarding predictors of QoL in children with NF1 were weak, due to a lack of studies, heterogeneity of samples, and heterogeneity of measurements/predictors assessed. Future studies should examine more comprehensively the psychosocial factors affecting the NF population, especially in young patients with NF2 and schwannomatosis, who have been neglected in prior research. The use of consistent QoL measures is preferred to allow better comparison among studies and conditions. Interventions, including comprehensive mind–body treatments, are warranted to address impaired QoL in children and adolescents with NF1.