Optic Nerve Diseases in Children
The optic nerve is the vital structure that transmits the electrical signal from the retina in to the brain allowing us to see. Many conditions can damage the optic nerve in children but some are rare and not well understood. Research focusing on these rare optic nerve diseases (neuropathies) include those arising from raised intracranial pressure, perinatal ischaemia, vitamin A deficiency and genetic causes.
– Idiopathic intracranial hypertension is a disorder of raised pressure in the brain which can cause the optic nerves to swell. In children this is a rare disorder. A study is ongoing to identify children with this condition to better understand why it happens and its impact on the optic nerves.
– Optic atrophy (thinning of the optic nerves) can arise from events relating to birth such as stroke. This rare complication of optic atrophy is thought to arise from retrograde degeneration of visual pathways in the brain leading to loss of optic nerve fibres. It is a recognised complication of stroke in adults but not well understood or published in children. This research project characterises this condition in five patients.
– Optic atrophy from vitamin A deficiency in children is a very rare and poorly recognised complication of severe dietary restriction in autism spectrum disorder. Four boys of Māori and Pasific ethnicity have been identified with this rare association which causes skull thickening and optic nerve compression as well as nutritional optic neuropathy. Collaboration with paediatric neurologists, paediatric neurosurgeons and paediatricians is essential in managing these patients. As well as publishing the outcomes of these patients to improve awareness, projects are planned to investigate access to vitamin supplementation for these patients.
Publishing this research will help raise awareness about paediatric optic nerve conditions and aid in the investigation of vitamin supplementation for these patients. - Dr Sarah Hull
Visual fields are a sensitive method of detecting the presence of a disease and in particular detecting progression of disease in patients with a huge range of eye conditions. The best known application is in glaucoma, but its applications are far reaching. This research supported by the Vision Research Foundation uses a specific type of visual field machine called an Octopus to detect change in patients with rare eye diseases. Additionally, the field machine is of great benefit for children who cannot perform traditional visual fields reliably. The Octopus has modifiable fast programs particularly useful in children.
Our research team includes Dr Sarah Hull, Senior Lecturer at Auckland University and Ophthalmic Specialist in Auckland, Professor Helen Danesh-Meyer CNZM, scientific director at Vision Research Foundation and Dr Daniel Scott, Ophthalmology registrar and includes collaborations with paediatric neurologists, neurosurgeons and paediatricians.
Dr Sarah Hull
Dr Sarah Hull is an Ophthalmology Consultant and Senior Lecturer in Auckland specialising in Paediatrics, Genetics and Strabismus. Her medical school training was at Cambridge University and Imperial College, London, UK. She entered the North London Ophthalmology training scheme in 2007 with the majority of her training at the world-renowned Moorfields Eye Hospital.
From 2013-2016 she completed a PhD at the Institute of Ophthalmology, UCL and Moorfields on paediatric retinal genetics. She has authored more than 50 peer-reviewed articles and book chapters and presents annually at international and national meetings.
