Craniopharyngioma (CP) treatment, including surgery and radiotherapy, can have short- and long-term vascular side effects. Hypothalamic damage is related to morbid obesity and may increase the lifelong risk of experiencing vascular events in CP patients. This review summarized the available evidence regarding vascular complications in adamantinomatous or papillary CP patients, whatever their age at diagnosis. Three databases (Medline, CINAHL, Web of Science) were searched (06/2023) to retrieve eligible articles. The search was limited to peer-reviewed articles. Titles, abstracts, and full texts were screened by two independent reviewers, and data were extracted using a self-developed grid. Seventy-two studies were included in this review; the majority were case reports. Reported vascular sequela that occurred due to surgery were fusiform dilation of the carotid artery, stroke, vasospasm, hemorrhage, and aneurysm. Related conditions that emerged due to radiotherapy included Moyamoya syndrome and cavernoma. Cardiovascular morbidity and mortality often lead to hypothalamic obesity and metabolic syndrome in CP patients. Vascular damage is a rare complication of CP treatment. Surgical strategies should protect the surrounding hypothalamic and vascular structures. Patients receiving radiotherapy, particularly at a young age, should undergo magnetic resonance angiography monitoring to identify possible neurovascular sequela during post-treatment care.

Objective: It is well known that both genetic background and lifestyle influence the development of ‘general’ obesity. However, the role of parental body mass index (BMI) on the development of obesity in long-term survivors of childhood-onset craniopharyngioma (CP) is not well understood. This study analyzed the correlation of patients’ BMI at diagnosis and last visit and parental BMI at CP diagnosis and further explored potential risk factors for obesity in CP patients. Design: This is a registry-based retrospective cohort study. Methods: In total,291 CP patients and their parents recruited in the German KRANIOPHARYNGEOM studies were included. Correlations between patient’s BMI SDS at CP diagnosis and last visit and parental BMI at CP diagnosis were analyzed. The associations between hypothalamic damage, maternal/paternal BMI and CP patients’ obesity at last visit were analyzed by multivariable logistic regression. Results: At follow-up, 52% of CP patients developed obesity (BMI > 3SDS). Patient’s BMI SDS at last visit was moderately correlated with BMI-SDS at CP diagnosis (r = 0.48, 95% CI: 0.38–0.58, P < 0.001), and also with maternal BMI at diagnosis (r = 0.28, 95% CI: 0.17–0.38, P < 0.001) and paternal BMI at diagnosis (r = 0.3, 95% CI: 0.19–0.41, P < 0.001). However, the contributing role of parental BMI to the pathogenesis of obesity was small compared to the impact of hypothalamic damage. Conclusion: We conclude that besides hypothalamic damage, parental disposition for obesity is associated with the development of obesity in patients after CP. Our results indicate that also the family situation could have an influence on the development of obesity after CP and might be a therapeutic target. Significance statement: Survivors of childhood-onset craniopharyngioma are at risk of developing morbid obesity. So far, patients with posterior hypothalamic involvement and lesion were identified as a high risk group. With this study, the influence of parental body mass index on the risk of obesity was investigated. Patient’s body-mass-index at last visit was correlated with maternal and paternal body mass index at diagnosis. With increasing maternal or paternal body mass index, the likelihood of obesity in individuals with CP increased. Nevertheless, the parents’ weight had only a small effect on the development of patients’ obesity compared to hypothalamic damage.

Disclosure: J. Beckhaus: None. S. Boekhoff: None. L. Krieger: None. J. Peng: None. C. Friedrich: None. H.L. Muller: None. Craniopharyngiomas (CP) are congenital, benign, embryonic malformations which arise from ectoblastic remnants of Rathke’s pouch and are located in the (supra-)sellar region. Many patients show a reduced growth rate and an increased BMI early before the diagnosis of CP. However, it is unknown whether patients with CP present with increased head circumference before diagnosis. A cohort of 83 patients was screened for medical records of their developmental monitoring visits. We observed, that CP patients with hypothalamic involvement of their tumor tend to have an increased head circumference SDS before CP diagnosis. When comparing head circumference SDS before diagnosis at defined time points between birth and four years of age, all head circumferences were in the upper normal range. However, no statistically noticeable differences were found between hypothalamic involvement and no hypothalamic involvement of the tumor. Congenital embryonic malformations such as CP located in the parasellar area can have impact on cerebrospinal fluid circulation and lead higher head circumferences due to hydrocephalus early before diagnosis. Our results show, that head circumference SDS could be an effective early-indicator for CP. A standardized procedure for head circumference measurements during developmental monitoring visits should be implemented in clinical care. We anticipate that our study is the starting point for prospective studies with larger sample size to assess the potential predictive value of head circumference measurements for an early CP diagnosis. Presentation: Thursday, June 15, 2023

Disclosure: P. Sowithayasakul: None. S. Boekhoff: None. J. Beckhaus: None. C. Friedrich: None. H.L. Muller: None. Quality of life (QoL) has become a critical component of therapeutic outcomes in the survivors of childhood-onset craniopharyngioma (CP). Visual deficiency adversely affects daily functioning and QoL in childhood CP. This cohort study aimed to report the vision-related QoL in CP patients. The primary outcome measure was pediatric QoL (PEDQOL score) in patients with and without visual impairment from presentation at the time of CP diagnosis to three-year follow-up. The risk factors associated with visual impairment were also analyzed. A total of 120 patients were included in this study, with a median age at diagnosis of 10 years (ranging from 1.3 to 16.8 years). The most common presenting symptoms at the time of CP diagnosis were headache (42%), visual impairment (20%), and growth retardation (12%). Visual impairment was found in 87 patients (70%). Ophthalmologic examination and PEDQOL scores, which were grouped into seven domains: physical functioning, cognition, social functioning friends, social functioning family, and body image, were evaluated at the third month, first-year, and third-year after diagnosis. Parents of children with visual impairment report worse social functioning in their families than parents of children without visual impairment, one (p=0.017) and three years (p=0.011) after diagnosis. After three years, children with visual impairment were found to have worse quality of life in the autonomy domain of self- (p=0.029) and parental assessment (p=0.048). High tumor volume (>21 cm³), radiation therapy and extrasellar tumor location were identified as potential risk factors for visual impairment. We conclude that visual impairment has a negative impact on QoL after CP. Early detection of visual impairment, regular QoL assessment, and risk-appropriate follow-up care are recommended. Presentation: Thursday, June 15, 2023