Optic Nerve Hypoplasia in Children

Skarf, Barry; Hoyt, Creig S.

doi:10.1001/archopht.1984.01040030046032

Cited by 123 publications

(19 citation statements)

References 42 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Current studies, although cross-sectional in design, point to an increased risk of developing pituitary hormone deficiencies when structural abnormalities of the pituitary gland are present [9, 10], as well as ophthalmologic factors such as bilateral ONH and blindness [2, 11]. Neurodevelopmental issues, including seizures, autism, and behavioral difficulties, may also occur more frequently in children with ONH [2, 12, 13].…”

Section: Introductionsupporting

confidence: 39%

Pituitary Dysfunction in Pediatric Patients with Optic Nerve Hypoplasia: A Retrospective Cohort Study (1975–2014)

et al. 2017

View full text Add to dashboard Cite

Background/Aims: The risk factors for pituitary hormone dysfunction (PHD) in children with optic nerve hypoplasia (ONH) are not well understood. This study identified the type, timing, and predictors of PHD in children with ONH. Methods: ONH patient charts were reviewed retrospectively. The incidence rate of PHD was calculated assuming a Poisson distribution. Predictors of PHD were identified through a multivariable Cox proportional hazards model. Results: Among 144 subjects with ONH, 49.3% (n = 71) developed PHD over 614.7 person-years of follow-up. The incidence was 11.55 (95% confidence interval [CI]: 9.02–14.57/100 person-years). The median time to first PHD was 2.88 (interquartile range: 0.02–18.72) months. Eighty-two percent developed their first PHD by their 5th and 90% by their 10th birthday, and 89% within 5 years of ONH diagnosis. Prematurity (adjusted hazard ratio [aHR]: 0.33; 95% CI: 0.1–1.07), blindness (aHR: 1.72; 95% CI: 1.03–2.86), maternal substance abuse (aHR: 1.51; 95% CI: 0.91–2.48), abnormal posterior pituitary (aHR: 3.8; 95% CI: 2.01–7.18), and hypoplastic/absent anterior pituitary (aHR: 2.52; 95% CI: 1.29–4.91) were significant predictors of PHD. Conclusions: The clinical predictors of PHD included blindness, pituitary gland abnormalities, and maternal substance abuse. These predictors help clinical decision-making related to the need for and frequency of hormone testing in pediatric patients with ONH.

show abstract

Section: Introductionsupporting

confidence: 39%

Pituitary Dysfunction in Pediatric Patients with Optic Nerve Hypoplasia: A Retrospective Cohort Study (1975–2014)

et al. 2017

View full text Add to dashboard Cite

show abstract

“…Our cohort of ONH had a predominance of bilaterally affected cases, similar to previous reports [6, 10, 17, 22, 26, 29, 31]. A diagnosis of ONH in one or both eyes was not associated with increased prevalence of absent septum pellucidum, but bilateral disease was associated with a higher risk of other brain malformations.…”

Section: Discussionsupporting

confidence: 90%

“…Unilateral cases were not protected from brain malformations, and in fact, nearly 50% of the unilateral cases in our cohort had at least one identified brain malformation. This refutes previous reports that brain malformations are exclusive to bilateral ONH[9, 12, 26, 28, 31]. …”

Section: Discussionsupporting

confidence: 89%

Brain Malformations Do Not Predict Hypopituitarism in Young Children with Optic Nerve Hypoplasia

et al. 2017

View full text Add to dashboard Cite

Background: Optic nerve hypoplasia (ONH), a leading cause of pediatric blindness, is associated with brain malformations and hypopituitarism in the constellation known as septo-optic dysplasia. Neuroimaging is used to anticipate hypopituitarism, but with unconfirmed reliability. We report prospective findings on the association of hypopituitarism with brain malformations. Methods: Children (<24 months) with ONH (n = 146; 87% bilateral) underwent baseline MRI and annual examinations and hormonal testing. Hypopituitarism status at age 5 years was classified. Results: A total of 74% had brain malformation(s). Hypopituitarism (69%) was not associated with brain malformations (p = 0.351); this persisted after adjusting for the laterality of ONH and the timing of MRI (p_adj = 0.869). No association was noted for absent septum pellucidum (38%; p = 0.073), corpus callosum abnormality (51%; p = 0.625), and major malformations (22%; p = 0.407). A malformation conferred a positive predictive value of 71% (95% CI: 62%, 80%), and a negative predictive value of 37% (95% CI: 22%, 54%). Overall, 10% (n = 15) of the cohort presented with a triad of absent septum pellucidum, corpus callosum abnormality, and other major malformation; only half (n = 8) of these had hypopituitarism. All 13 subjects with pituitary malformations manifested hypopituitarism, conferring predictive values of 100% (positive) and 34% (negative). Conclusions: Hypopituitarism and brain malformations are highly prevalent, but have unrelated associations with ONH. Brain MRI in infants and toddlers with ONH is an unreliable screen for hypopituitarism risk.

show abstract

“…A retrospective review of patients with optic nerve hypoplasia [13] found that 6 of 51 children (12%) had experienced NH (< 1.38 mmol/L for preterm and < 1.94 mmol/L for full term babies). However, a larger prospective study of optic nerve hypoplasia revealed that only 5 of 93 (5%) children with optic nerve hypoplasia had experienced NH [14]. …”

Section: Neonatal Hypoglycaemia and Ocular Developmentmentioning

confidence: 99%

Neonatal Hypoglycaemia and Visual Development: A Review

et al. 2017

View full text Add to dashboard Cite

Background: Many newborn babies experience low blood glucose concentrations, a condition referred to as neonatal hypoglycaemia (NH). The effect of NH on visual development in infancy and childhood is of interest because the occipital lobes, which include the primary visual cortex and a number of extrastriate visual areas, may be particularly susceptible to NH-induced injury. In addition, a number of case series have suggested that NH can affect eye and optic nerve development. Objective: To review the existing literature concerning the effect of NH on the visual system. Methods: A PubMed, Embase, Medline, and Google Scholar literature search was conducted using prespecified MeSH terms. Results: The literature reviewed revealed no clear evidence for an effect of NH on the development of the eye and optic nerve. Furthermore, occipital and occipital-parietal lobe injuries following NH often occurred in conjunction with comorbid conditions and were not clearly linked to subsequent visual dysfunction, possibly due to difficulties in measuring vision in young children and a lack of studies at older ages. A recent, large-scale, prospective study of NH outcomes at 2 years of age found no effect of mild-to-moderate NH on visual development. Conclusion: The effect of NH on visual development is unclear. It is currently unknown whether NH affects visual function in mid-to-late childhood when many visual functions reach adult levels.

show abstract

Optic Nerve Hypoplasia in Children

Cited by 123 publications

References 42 publications

Pituitary Dysfunction in Pediatric Patients with Optic Nerve Hypoplasia: A Retrospective Cohort Study (1975–2014)

Pituitary Dysfunction in Pediatric Patients with Optic Nerve Hypoplasia: A Retrospective Cohort Study (1975–2014)

Brain Malformations Do Not Predict Hypopituitarism in Young Children with Optic Nerve Hypoplasia

Neonatal Hypoglycaemia and Visual Development: A Review

Contact Info

Product

Resources

About