Resting energy expenditure in children at risk of hypothalamic dysfunction

Objective: Children with suprasellar brain damage are at risk of hypothalamic dysfunction (HD). HD may lead to decreased resting energy expenditure (REE). Decreased REE, however, is not present in all children with HD. Our aim was to assess which children suspect for HD have low REE, and its association with clinical severity of HD or radiological hypothalamic damage. Patients and methods: A retrospective cohort study was performed. Measured REE (mREE) of children at risk of HD was compared to predicted REE (pREE). Low REE was defined as mREE <90% of predicted. The mREE/pREE quotient was associated to a clinical score for HD symptoms and to radiological hypothalamic damage. Results: In total, 67 children at risk of HD (96% brain tumor diagnosis) with a mean BMI SDS of +2.3 ± 1.0 were included. Of these, 45 (67.2%) had low mREE. Children with severe HD had a significant lower mean mREE/pREE quotient compared to children with no, mild, or moderate HD. Mean mREE/pREE quotient of children with posterior hypothalamic damage was significantly lower compared to children with no or anterior damage. Tumor progression or tumor recurrence, severe clinical HD, and panhypopituitarism with diabetes insipidus (DI) were significant risk factors for reduced REE. Conclusion: REE may be lowered in children with hypothalamic damage and is associated to the degree of clinical HD. REE is, however, not lowered in all children suspect for HD. For children with mild or moderate clinical HD symptoms, REE measurements may be useful to distinguish between those who may benefit from obesity treatment that increases REE from those who would be better helped using other obesity interventions

Fluorine-18 fluorodeoxyglucose positron emission tomography (PET) to detect vital retinoblastoma in the eye: Preliminary experience

Background/aims: To report our first experience with FDG-PET in the detection of vital retinoblastoma. Methods: Four newly diagnosed retinoblastoma patients, two treated retinoblastoma patients, and four control patients were enrolled in this pilot study. F18-FDG uptake was assessed in the light of clinical and histopathological features. Results: PET discriminated between new patients and controls, although tumor uptake varied widely. PET added no useful information with regard to possible vital tissue in tumor scars in the eye of the two treated retinoblastoma patients. Moreover, PET findings did not correlate with clinical or histopathological features. Conclusion: Based on this small pilot study, F18-PET shows little promise in the detection of retinoblastoma. More research on other radiofarmacons is recommended

The importance of specialized sleep investigations in children with a suprasellar tumor

Purpose: Disruption of sleep has great impact on quality of life. In children with a suprasellar tumor and hypothalamic-pituitary dysfunction, the circadian rhythm may be disturbed causing sleep problems. However, also other factors may influence sleep. Awareness of these different etiologies and careful history taking with appropriate additional diagnostics will aid in restoring sleep quality. Methods: We present the workup of 4 cases with a suprasellar tumor and disturbances of sleep initiation, sleep maintenance, and daytime sleepiness. In parallel, we developed a flowchart, to aid clinicians in the diagnostics of sleep problems in children after treatment for a (supra) sellar brain tumor. Results: All four patients, known with hypopituitarism, presented with sleep complaints and increased daytime sleepiness. In all four, the cause of sleep problems showed to be different. In the first case, sleep evaluation revealed a severe obstructive sleep apnea, whereupon nocturnal ventilation was started. The second case revealed poor sleep hygiene in combination with an obsessive compulsive disorder. Sleep hygiene was addressed and psychiatric consultation was offered. Dexamphetamine treatment was started to reduce her obsessive compulsive complaints. The third case showed a delayed sleep phase syndrome, which improved by educational support. The fourth case revealed a secondary organic hypersomnia for which modafinil treatment was started. Conclusion: Sleep disturbances in children with hypopituitarism due to a (supra) sellar tumor can have different entities which require specific therapy. Awareness of these different entities is important to enable appropriate counseling. Referral to an expertise sleep center may be advised, if standard educational support is insufficient

The development of hypothalamic obesity in craniopharyngioma patients : A risk factor analysis in a well-defined cohort

Background: Hypothalamic obesity (HO) is a major concern in patients treated for craniopharyngioma (CP). The influence of degree of resection on development of HO, event-free survival (EFS), and neuroendocrine sequelae is an issue of debate. Procedure: A retrospective cohort consisting of all CP patients treated between 2002 and 2012 in two university hospitals was identified. Multivariable logistic regression was used to study the associations between preoperative BMI, age at diagnosis, tumor volume, performed surgical resection, and presence of HO at follow-up. Results: Thirty-five patients (21 children and 14 adults) were included. Median follow-up time was 35.6 months (4.1–114.7). Four patients were obese at diagnosis. HO was present in 19 (54.3%) patients at last follow-up of whom eight were morbidly obese. Thirteen (37.1%) patients underwent partial resection (PR) and 22 (62.9%) gross total resection (GTR). GTR was related to HO (OR 9.19, 95% CI 1.43–59.01), but for morbid HO, obesity at diagnosis was the only risk factor (OR 12.92, 95% CI 1.05–158.73). EFS in patients after GTR was 86%, compared to 42% after PR (log-rank 9.2, P = 0.003). Adjuvant radiotherapy after PR improved EFS (log-rank 8.2, P = 0.004). Panhypopituitarism, present in 15 patients, was mainly seen after GTR. Conclusions: HO is less frequent after PR than after GTR, but PR cannot always prevent the development of morbid obesity in patients with obesity at diagnosis. PR reduces the occurrence of panhypopituitarism. When developing a treatment algorithm, all these factors should be considered

Management and consequences of postoperative fluctuations in plasma sodium concentration after pediatric brain tumor surgery in the sellar region : a national cohort analysis

Purpose: Severe fluctuations in plasma sodium concentration and plasma osmolarity, including central diabetes insipidus (CDI), may have significant influence on postoperative morbidity and mortality after pediatric brain tumor surgery.The aim of this study was to describe the frequency, severity and neurological consequences of these fluctuations in pediatric brain tumor survivors. Methods: A retrospective, multi-institutional chart review was conducted among all children who underwent brain tumor surgery in the sellar or suprasellar region in seven university hospitals in the Netherlands between January 2004 and December 2013. Results: Postoperative CDI was observed in 67.5% of 120 included children. Fluctuations of plasma sodium concentration ≥ 10 mmol/L/24 h during the first ten postoperative days were seen in 75.3% of patients with CDI, with a maximum delta of 46 mmol/L/24 h. When compared to patients without CDI, altered mental status occurred more frequently in patients with postoperative CDI (5.1 vs. 23.5% respectively, p = 0.009). Low plasma sodium concentration was related to altered mental status and the occurrence of seizures. Frequency and severity of fluctuations in plasma sodium concentration during the first ten postoperative days were significantly higher in patients with permanent CDI at last follow-up than in patients with transient CDI or without CDI (p = 0.007). Conclusion: Postoperative CDI is a common complication after pediatric brain tumor surgery in the sellar or suprasellar region. Extreme plasma sodium concentrations and large intra-day fluctuations still occur and seem to influence the postoperative neurological course. These results illustrate the need for intensive monitoring in a highly experienced center

Landelijke richtlijnen voor follow-up van overlevenden van kinderkanker

Members of the Late Effects Taskforce of the Dutch Childhood Oncology Group (DCOG) and of the Haematology-Oncology Section of the Dutch Paediatric Association are involved in the development of guidelines for the follow-up of childhood cancer survivors. The recommendations of these guidelines are based on the best available clinical evidence, current guidelines and clinical experience of late effects specialists. The guidelines will lead to a uniform and standardised post-treatment care and long-term follow-up of childhood cancer survivors in the Netherlands. The information in the guidelines will be of importance for care providers in paediatrics, general medicine, internal medicine, gynaecology/obstetrics as well as for other specialists and particularly for childhood cancer survivors themselves. The information will lead to an increased awareness for all Dutch care providers who are responsible for the health problems of childhood cancer survivors. The development of guidelines for childhood cancer survivors is an important part of a new Dutch project: Late Effects Registry (LATER). Within this new national project patient and treatment data as well as follow-up data on childhood cancer survivors in the Netherlands will be registered. The project LATER aims at: to coordinate and to evaluate care of the survivors, and to stimulate new research in the field of late effects of childhood cancer

Ophthalmological Findings in Youths with a Newly Diagnosed Brain Tumor

Importance: Visual impairment is an irreversible adverse effect in individuals who experienced a childhood brain tumor. Ophthalmological evaluation at diagnosis enables early detection of vision loss, decision-making about treatment, and when applicable, the timely use of visual interventions. However, awareness of visual impairment in clinical practice is suboptimal, and adherence to ophthalmological evaluation needs to be improved. Objective: To assess the prevalence and types of abnormal ophthalmological findings in youths with a newly diagnosed brain tumor. Design, Setting, and Participants: In this nationwide, prospective cohort study, youths aged 0 to 18 years with a newly diagnosed brain tumor between May 15, 2019, and August 11, 2021, were consecutively enrolled in 4 hospitals in the Netherlands, including the dedicated tertiary referral center for pediatric oncology care. Exposures: A standardized and comprehensive ophthalmological examination, including orthoptic evaluation, visual acuity testing, visual field examination, and ophthalmoscopy, was performed within 4 weeks from brain tumor diagnosis. Main Outcomes and Measures: The main outcomes were prevalence and types of visual symptoms and abnormal ophthalmological findings at brain tumor diagnosis. Results: Of 170 youths included in the study (96 [56.5%] male; median age, 8.3 years [range, 0.2-17.8 years]), 82 (48.2%) had infratentorial tumors; 53 (31.2%), supratentorial midline tumors; and 35 (20.6%), cerebral hemisphere tumors. A total of 161 patients (94.7%) underwent orthoptic evaluation (67 [41.6%] preoperatively; 94 [58.4%] postoperatively); 152 (89.4%), visual acuity testing (63 [41.4%] preoperatively; 89 [58.6%] postoperatively); 121 (71.2%), visual field examination (49 [40.4%] preoperatively; 72 [59.6%] postoperatively); and 164 (96.5%), ophthalmoscopy (82 [50.0%] preoperatively; 82 [50.0%] postoperatively). Overall, 101 youths (59.4%) presented with visual symptoms at diagnosis. Abnormal findings were found in 134 patients (78.8%) during ophthalmological examination. The most common abnormal findings were papilledema in 86 of 164 patients (52.4%) who underwent ophthalmoscopy, gaze deficits in 54 of 161 (33.5%) who underwent orthoptic evaluation, visual field defects in 32 of 114 (28.1%) with reliable visual field examination, nystagmus in 40 (24.8%) and strabismus in 32 (19.9%) of 161 who underwent orthoptic evaluation, and decreased visual acuity in 13 of 152 (8.6%) with reliable visual acuity testing. Forty-five of 69 youths (65.2%) without visual symptoms at diagnosis had ophthalmological abnormalities on examination. Conclusions and Relevance: The results of this study suggest that there is a high prevalence of abnormal ophthalmological findings in youths at brain tumor diagnosis regardless of the presence of visual symptoms. These findings support the need of standardized ophthalmological examination and the awareness of ophthalmologists and referring oncologists, neurologists, and neurosurgeons for ophthalmological abnormalities in this patient group