Neuropsychological outcomes of children with Optic Pathway Glioma

Abstract

Optic Pathway Glioma (OPG) is a relatively common brain tumour in childhood; however, there is scarce understanding of neuropsychological sequelae in these survivors. In this study, 12 children with diagnosis of OPG before 6 years of age received a comprehensive standardised assessment of visual perception, general intelligence and academic achievement, using adjustments to visual materials of the tests, to examine the extent of concurrent impairment in these functional domains. Information about vision, clinical and socio-demographic factors were extracted from medical records to assess the associations of neuropsychological outcomes with clinical and socio-demographic factors. Children with OPG exhibited high within-patient variability and moderate group-level impairment compared to test norms. Visual perception was the most impaired domain, while scholastic progression was ageappropriate overall. For cognition, core verbal and visuo-spatial reasoning skills were intact, whereas deficits were found in working memory and processing speed. Visual function was associated with tasks that rely on visual input. Children with OPG are at moderate risk of neuropsychological impairment, especially for visual perception and cognitive proficiency. Future research should elucidate further the relative contribution of vision loss and neurofibromatosis type 1 co-diagnosis within a large sample. Optic Pathway Gliomas (OPGs) represent about 5% of all intracranial neoplasms in childhood 1,2. They are a relatively homogeneous group of benign lesions that arise in any structure along the visual pathway and histologically classify as pilocytic astrocytoma. Half or more OPGs are associated with Neurofibromatosis type 1 (NF1) 3,4 , a multisystem genetic disorder that is associated with neuropsychological sequelae, including mild intellectual impairment, inattention, visuospatial deficits and learning disability 5-9. While survival rates of OPG exceed 90% 10 , most children experience some degree of visual impairment that significantly affects their quality of life 11,12. Both NF1 comorbidity and visual impairment place children with OPG at risk of neuropsychological difficulties, but research has rarely focused on functional outcomes beyond vision. Retrospective investigations of OPG survivorship at two large north American institutions showed that only 26% to 42% of the whole patients cohort received sufficient psychological evaluation, and they reported only broad measures of Intelligence Quotient (IQ) 13 , in a descriptive manner 10. One prospective study 14 documented the neuropsychological profile of 21 school-aged children with OPG initially treated with chemotherapy integrating an IQ test for cognitive function with a collection of subtests from different batteries for individual perceptual and scholastic skills. This study was limited by the fact that over a third of the children had been treated with additional radiotherapy, the most critical risk factor for neurocognitive decline 15,16. In addition, this does not reflect th...

Endoscope-Assisted Microsurgery in Pediatric Cases With Pineal Region Tumors: A Study of 18 Cases Series

“…However, microscopes have their inherent disadvantages such as a narrow field of view, shallow depth of field, and limited angle ( 27 ). As a result, it is difficult to detect residual tumors and blood clots in the “blind areas” with microscope ( 6 , 9 , 20 ). This is more visible in pediatric patients, as pineal region tumors in children tend to be large and extend in various directions, particularly from the third ventricle to the lateral ventricle ( 21 , 28 ).…”

“…However, the difficulty and risk of surgery are considerable because pineal region tumors are deep-seated and surrounded by vital neurovascular structures (6). Additionally, the limited viewing angle of the surgical field makes it difficult to perform total resection and cerebral aqueduct exploration under microscopy, especially for the pediatric patients (7)(8)(9). Endoscope-assisted microsurgery (EAM) was recently successfully applied to resection of pineal region tumors (10)(11)(12)(13).…”

Long-term cognitive deficits in pediatric low-grade glioma (LGG) survivors reflect pretreatment conditions—report from the German LGG studies

“…Results for visual-motor integration were noticeably lower for the small subgroup of NF1 patients with visual pathway glioma following chemotherapy as compared to the population norm. 41 Chemotherapy-associated changes in fractional anisotropy are increased in NF1, and these white matter changes may contribute to the differences in cognitive results. 42 Within our older cohort, we could not replicate reports on language difficulties in young children with NF1 as stressed by Brei et al 43 This may be due to specific support for dyslectic children in the German school system.…”

A systematic review of ongoing clinical trials in optic pathway gliomas

“… Psychological assessment Social functioning Children with brain tumours are at risk for a number of late psychological effects, including neurocognitive and social deficits. These can be assessed with psychological questionnaires [ 16 ]. …”

“…This multifaceted care underlies the complexity of the disease. Advances in the understanding and treatment of OPG could come from a variety of sources, including novel or repurposed pharmacological agents, emerging biological agents and tumour vaccines, refinement of surgical approaches, diagnostic and therapeutic radiological developments or quality of lifefocussed research [10][11][12][13][14][15][16]. The aim of this systematic review was to formulate a comprehensive list of all current ongoing clinical trials that were specifically looking at a clinical care of OPGs in order to identify trends in current research and provide an overview of the field to guide future research efforts.…”