Optic nerve hypoplasia in children and adolescents; prevalence, ocular characteristics and behavioural problems

Fahnehjelm, Kristina Teär; Dahl, Sara; Martin, Lene; Ek, Ulla

doi:10.1111/aos.12270

Cited by 36 publications

(43 citation statements)

References 48 publications

(48 reference statements)

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“…The median score of patients with unilateral ONH was comparable to that in the current study, while patients with bilateral ONH scored higher (Tear Fahnehjelm et al. ). While there were no bilateral MGDA patients in this study for comparison, a report by Nawratzki and co‐workers described a patient with bilateral MGDA and autism (Nawratzki et al.…”

Section: Discussionsupporting

confidence: 81%

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Morning Glory Disc Anomaly in childhood – a population‐based study

Ceynowa

Wickström

Olsson

et al. 2015

Acta Ophthalmologica

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ABSTRACT.Purpose: To report prevalence, ocular characteristics and coexisting neurological, behavioural, somatic and neuroradiological abnormalities in children and adolescents with morning glory disc anomaly (MGDA). Methods: In a cross-sectional population-based study, 12 patients with MGDA, aged 2-20 years, were identified. All 12 agreed to ophthalmological assessments including visual functions, refraction, fundus photography, optical coherence tomography (OCT) and ocular motor score (OMS). Neurological examinations and behavioural/developmental screening were carried out. Data from previous or new neuroradiological investigations were collected. Results: The prevalence of MGDA was 2.6/100 000. MGDA was unilateral in 11/12 patients with a best-corrected visual acuity (BCVA) in the MGDA eye ranging from hand motion to 0.65 (median 0.06). Severe microphthalmus prevented unilaterality to be determined in one adolescent. All patients had a binocular BCVA of ≥0.5. OMS showed abnormalities in pupil response, vestibulo-ocular reflex, stereo visual acuity, strabismus and convergence. OCT revealed peripapillary or macular oedema in 5/8 patients and foveal aplasia in 3/8 patients. Three patients had extensive capillary hemangiomas, of which one had PHACES syndrome and one had additional cerebrovascular anomalies and corpus callosum agenesis. Neuroradiology showed craniovascular anomalies in two patients. Neurology was mostly normal. Behavioural/developmental screening showed attention deficit hyperactivity disorder in one patient. Conclusions: The prevalence data, previously not reported, of morning glory disc anomaly was 2.6/100 000. Coexisting retinal peripapillary or macular oedema was common, as were cerebral abnormalities and/or cutaneous vascular malformations. The associated findings may not be discovered through routine ophthalmological examination why OCT and neuroimaging are called for.

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Section: Discussionsupporting

confidence: 81%

“…The prevalence of optic nerve hypoplasia has previously been reported as 17.3/100 000 in patients <18 years of age in the same population (Tear Fahnehjelm et al. ) showing that MGDA is a less common condition. The frequency of intracranial anomalies and vascular malformations in patients with MGDA, hitherto not reported, was 25%.…”

Section: Discussionmentioning

confidence: 83%

Morning Glory Disc Anomaly in childhood – a population‐based study

Ceynowa

Wickström

Olsson

et al. 2015

Acta Ophthalmologica

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“…The diagnosis is clinical and characterised by a thin optic nerve with a reduced number of retinal ganglion cell axons in one or both eyes. We have previously reported that the prevalence of ONH in children was 17.3/100 000 in the county of Stockholm and unilateral in 45% of the patients .…”

Section: Introductionmentioning

confidence: 99%

High prevalence of pituitary hormone deficiency in both unilateral and bilateral optic nerve hypoplasia

et al. 2019

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Aim This study examined the prevalence of neurological impairment and pituitary hormone deficiency (PHD) in patients with unilateral and bilateral optic nerve hypoplasia (ONH). Methods A population‐based cross‐sectional cohort study of 65 patients (51% female) with ONH was conducted in Stockholm. Of these were 35 bilateral and 30 unilateral. The patients were below 20 years of age, living in Stockholm in December 2009 and found through database searching. The median age at the analysis of the results in January 2018 was 16.1 years (range 8.1–27.5 years). Neurological assessments and blood sampling were conducted, neuroradiology was reviewed and growth curves were analysed. Diagnoses of PHDs were based on clinical and biochemical evidence of hormone deficiency. Results Neurological impairments were identified in 47% of the patients and impairments in gross and fine motor function were more prevalent in bilateral ONH (p < 0.001). In addition, 9% had cerebral palsy and 14% had epilepsy. The prevalence of PHD was 29 and 19% had multiple PHD. Conclusion Children with ONH had a high risk of neurological impairment, especially in bilateral disease. Both unilateral and bilateral ONH signified an increased prevalence of PHD and all these children should be endocrinologically followed up until completed puberty.

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“…Optic nerve hypoplasia (ONH) is a developmental disorder in which there is a small optic nerve head and/or a reduced number of fibres in the optic nerve. It is the most common congenital optic nerve disorder and is a leading cause of childhood vision impairment in both the US and Europe, affecting as many as 12% of visually impaired children …”

Section: Introductionmentioning

confidence: 99%

Association of refractive error with optic nerve hypoplasia

Pang

Frantz

Roberts

2015

Ophthalmic Physiologic Optic

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Optic nerve hypoplasia subjects were more likely than control subjects to exhibit significant refractive errors, particularly hyperopia. Anisometropia tended to be more likely to occur in subjects with unilateral ONH than in bilateral ONH. Based on our findings, we recommend that clinicians perform a comprehensive eye examination on all patients with ONH and prescribe for existing refractive error when visual acuity or general visual function can realistically be improved.

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Optic nerve hypoplasia in children and adolescents; prevalence, ocular characteristics and behavioural problems

Cited by 36 publications

References 48 publications

Morning Glory Disc Anomaly in childhood – a population‐based study

Morning Glory Disc Anomaly in childhood – a population‐based study

High prevalence of pituitary hormone deficiency in both unilateral and bilateral optic nerve hypoplasia

Association of refractive error with optic nerve hypoplasia

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