Legal Degree of Disability in Childhood-Craniopharyngioma Survivors During Long-Term Follow-Up

Abstract

Cranioparyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar region. The prognosis after diagnosis during childood and adolescence is influenced by (neuro)endocrine long-term sequelae. A legal status of the degree of disability (GdB), according to the German Social Code Book V that is worthy of support provides financial means for psychosocial integration and participation of craniopharyngioma survivors. HIT-Endo is a German registry study on craniopharyngioma patients aged≤18 years at diagnosis . In a sample of 108 patients, the degree of disability and the association with endocrine, ophthalmological, neuropsychological (QLQ-C30; MFI-20; FMH-scale) and psychosocial parameters was analyzed after a mean follow-up period of 16 years. 47 patients (43%) did not receive a GdB or received a GdB of 30-40, 43 patients (40%) a GdB of 50-90 and 18 patients (17%) the maximal GdB of 100. Higher GdB were associated with lower education, higher body mass index standard deviation and a higher degree of visual impairment and hypothalamic involvement of the craniopharyngioma. Patients with a GdB of 100 reported loss in physical and cognitive function, dyspnea, and pain (QLQ-C30), as well as fatigue (MFI-20), and limitations in social and occupational contexts. They further had a lower functional capacity (German daily life ability scale (FMH)) compared to those with a smaller GdB. The GdB is associated with psychosocial and physical impairments and reflects the long-term consequences of craniopharyngioma. A low functional capacity may indicate a high GdB in later life of craniopharyngioma survivors.