Increasing incidence of optic nerve hypoplasia/septo-optic dysplasia spectrum: Geographic clustering in Northern Canada

Paediatrics & Child Health
Tanya KhaperBrandy Wicklow

Abstract

Owing to the shared embryonic origin, defects in development of optic nerves are often seen in conjunction with defects affecting the surrounding brain and pituitary gland. Optic nerve hypoplasia (ONH) and septo-optic dysplasia (SOD) represent a clinical spectrum associated with visual, pituitary and severe central nervous system structural abnormalities (SODplus). Based on changing clinical patterns, our primary objective was to examine trends in annual incidence of ONH/SOD and geographical clustering in Manitoba. This was a retrospective 1996 to 2015 chart review with extraction of anthropometric measures, radiologic findings, parental characteristics, endocrinopathies and neurologic symptoms from all involved in care. Postal codes were used to assign map co-ordinates and identify relevant census-based deprivation indices. Ninety-three children were identified in our catchment area; Poisson regression confirmed a striking 1.11-fold annual increase (95% confidence interval 1.07 to 1.16) or ~800% over two decades. The annual incidence (averaged 2010 to 2014 chart data) reached 53.3 per 100,000, affecting 1 in 1875 live births. Most (~55%) had SODplus. Common presenting features were hypoglycemia, nystagmus, seizures and develop...Continue Reading

References

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Citations

Jan 27, 2019·Cornea·Shaunak K BakshiJames Chodosh
Dec 8, 2019·Journal of Pediatric Endocrinology & Metabolism : JPEM·Heather D WadamsPeter J Tebben
Aug 20, 2019·Investigative Ophthalmology & Visual Science·Alicia KerrKonark Mukherjee
Feb 9, 2021·Curēus·Oluwatosin O OyadiranAhmad Khiami
Dec 15, 2020·Clinical Epigenetics·Patricia FauqueFabienne Pessione

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