Evaluation and Factors of Quality of Life Among Patients With Neurofibromatosis Type 1–Associated Craniofacial Plexiform Neurofibromas

Objectives: The aim was to evaluate overall quality of life (QOL) and investigate impact factors in Chinese neurofibromatosis type 1 (NF1) patients, particularly in those with craniofacial plexiform neurofibromas (pNFs). Methods: The Impact of NF1 on quality of life (INF1-QOL) Questionnaire were completed from a department of plastic and reconstructive surgery by 27 patients. Patients were 3 to 49 years of age. The correlation between subdomains were calculated using Pearson correlation. The difference between groups were evaluated using Fisher exact t -test. P value <0.05 were considered significant. Results: In age group of craniofacial pNFs, significant difference presented in cosmetic appearance, role and outlook on life and general QOL. Higher impact on general QOL in adults (6/8) than children (1/7) pointed to more impaired QOL in adults, as well as 2 subdomains including appearance, role and outlook on life. The patients who have more than 50 cutaneous neurofibromas (cNFs) (6/7) presented a significantly greater negative impact on the role and outlook of life. No statistically significant difference of QOL were detected between craniofacial and non-craniofacial pNFs patients. Conclusions: Age and cNFs were 2 main factors that have a negative impact on QOL in craniofacial pNFs patients. Adults reported lower QOL in cosmetic appearance, the role and outlook of life and general QOL. Patients with more than 50 cNFs reported more negative impact on the role and outlook of life. A multidiscipline management for these patients is required, including psychosocial intervention.