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Alagille Syndrome
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Long-term Visual Outcomes in Children with NF1-associated Optic Pathway Gliomas

Posted on April 21, 2020
Long-term Visual Outcomes in Children with NF1-associated Optic Pathway Gliomas

In patients with Neurofibromatosis type 1 (NF1), optic pathway gliomas (OPGs) are common and can cause significant visual morbidity.

In this retrospective observational case series, the visual acuity (VA) per eye, the VA per patient, and the presence of optic nerve head pallor, was evaluated in 45 children with NF1-associated OPG at ≥ 10 years follow-up.  

In 36% and 11% of patients, abnormal VA, defined as moderate to severe impairment, was found in the most affected eye and both eyes, respectively. In 62% of patients, optic nerve head pallor of one or both nerves were present. Good vision in both eyes was preserved in all but one patient. One patient had normal VA and normal appearing nerves at presentation but at long term follow-up had moderate to severe VA loss.

The authors concluded that “children with NF1-associated OPG who had a normal initial exam had excellent very long-term visual and anatomical outcomes. VA and optic nerve head appearance at presentation predict long term outcome.”

Reference

Kinori M, Armarnik S, Listernick R, et al. Neurofibromatosis type 1 associated optic pathway glioma in children – a follow up of 10 years or more. Am J Ophthalmol. 2020; DOI: https://doi.org/10.1016/j.ajo.2020.03.053.

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