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Craniopharyngioma is a rare, benign central nervous system tumor, which may be a source of multiple complications, from endocrinology to vision, neurology and neurocognitive functions. This morbidity can lead to reduced participation in life activities, as described in the International Classification of Functioning, Disability, and Health. The primary objective of this study was to measure participation in life activities in a population of children and young adults affected by childhood craniopharyngioma, using the LIFE-H questionnaire (Assessment of Life Habits), validated as a social participation measurement tool in various pediatric disabilities. We also describe complications in our population and examined the potential links between tumor characteristics, complications, and participation in life activities.This was a descriptive study, including all patients having presented childhood craniopharyngioma (before 18 years of age), followed in the Lyon region between 2007 and 2013. The main criterion was the LIFE-H results, completed by the patient or the carer.Of 21 patients included in the study, 14 completed the questionnaire, a mean 6.7 years after the diagnosis (SD: 3.9 years). The mean total LIFE-H score was 8.4 (SD: 1.03) for a normal score estimated at 10 in the general population. The lowest scores affected the nutrition, community life, and recreation dimensions. No patient had a normal score on all dimensions; 57% of the patients had more than three dimensions affected. The variability of the results between patients was lower for some dimensions with high means (fitness, personal care, communication, housing, mobility, responsibilities, and education) than in others (nutrition, interpersonal relationships, community life, employment, and recreation) with rather low means. All patients had an endocrinological deficit, 19% hypothalamic syndrome, 52% an impaired fulfillment feeling, 76% visual impairment, 14% neurologic impairment, and 91% neurocognitive impairment. In the entire group of patients, 57% were able to attend a normal school (of which 38% needed a personal helper), 43% had to enter a specialized school. In patients in a specialized school, the LIFE-H results were significantly lower in the nutrition, communication, housing, and recreation dimensions. Girls also had significantly better results in many dimensions than boys.Heavy comorbidity goes with childhood craniopharyngioma and affects patients' participation in life activities, which is not very low but multidimensional (more than three affected dimensions). This participation is mainly affected in social dimensions, whereas in cerebral palsy, where the LIFE-H has been tested several times, the daily activities dimensions are more affected.Patients with childhood craniopharyngioma are affected in their participation in daily activities, mainly in the social dimensions. This could be improved with systematic diagnosis of these participation impairments, with the goal of adapted multidisciplinary management. |
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