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[Optic pathway gliomas in neurofibromatosis type I. Longitudinal study of 30 cases in two multidisciplinary
Y Mikaeloff1, Y Chaix, J Grill
1Service de neuropédiatrie, hôpital Saint-Vincent-de-Paul, 82, avenue Denfert-Rochereau, 75674 Paris, France. yann.mikaeloff@free.fr
Insights
Most optic pathway gliomas in children with neurofibromatosis type 1 are stable. Close ophthalmological follow-up is crucial, with treatment reserved for progressive cases.
Area of Science:
- Pediatric Ophthalmology
- Neuro-oncology
- Genetics
Context:
- Neurofibromatosis type 1 (NF1) is a genetic disorder associated with increased risk of optic pathway gliomas (OPGs).
- OPGs in children with NF1 require careful monitoring due to potential vision impairment.
Purpose:
- To analyze the outcomes of OPGs in 30 children with NF1.
- To determine treatment indications, and establish follow-up and screening protocols for pediatric OPGs.
Summary:
- This study reviewed 30 children with NF1 and OPGs, with a median follow-up of six years.
- Thirty-seven percent of patients required treatment for progressive ophthalmological signs; the majority (63%) remained stable.
- Systematic screening MRI was employed for children under six or with neuropsychological deficits.
Impact:
- Highlights the generally stable nature of OPGs in NF1, emphasizing the need for vigilant ophthalmological surveillance.
- Supports a consensus for conservative management, limiting treatment to progressive cases to minimize intervention risks.
Unlabelled:
The aim of this study was to analyse the outcome of optic pathway gliomas in 30 children with neurofibromatosis type 1, the indications of treatment, and the follow-up and screening protocol.
Patients And Methods:
All patients with a minimal two years follow-up (median six years, range two to 19 years), in two multidisciplinary consultations of Saint-Vincent-de-Paul (Paris) and Purpan (Toulouse) hospitals, were included in the study. In our series, we practiced systematic screening MRI in children under six years' of age or with neuropsychological deficiency that may imply an unreliable ophthalmological examination.
Results:
Thirty-seven percent (11 patients) had progressive ophthalmological signs and were treated, and 63% (19 patients) were not progressive. Our study confirmed that most of optic pathway gliomas were stable during evolution, but rare cases may have bad prognosis.
Conclusion:
Our study supported the importance of close ophthalmological follow-up during childhood for which screening methods are discussed. There is a consensus to limit treatment for patients with progressive ophthalmological symptoms.