The incidence of neovascular membranes and visual field defects from optic nerve head drusen in children

doi:10.1016/j.jaapos.2015.10.013

Journal of American Association for Pediatric Ophthalmology and Strabismus

Volume 20, Issue 1, February 2016, Pages 44-48

Presented in part as a poster at the 40th Annual Meeting of the American Association for Pediatric Ophthalmology and Strabismus, Palm Springs, California, April 2-6, 2014.

https://doi.org/10.1016/j.jaapos.2015.10.013 Get rights and content

Purpose

To evaluate the incidence of visual field defects and choroidal neovascular membranes (CNVM) in a cohort of pediatric patients with optic nerve head drusen (ONHD).

Methods

The medical records of children with ONHD seen at a single center from January 2012 to July 2014 were retrospectively reviewed to identify patients who had a dilated fundus examination, fundus photography, spectral domain optical coherence tomography (SD-OCT)/enhanced-depth imaging (EDI) of the optic nerve head (ONH), SITA fast 24-2 Humphrey visual field (HVF) testing, lumbar puncture, and ocular ultrasound. A masked neuro-ophthalmologist analyzed fundus photographs, OCT, and fields. Retinal nerve fiber layer (RNFL) data were compared to age-matched controls.

Results

A total of 52 children (98 eyes) were included. Mean age was 10.8 ± 3.3 years. Of these, 42 patients had visual fields (57 eyes deemed reliable), and 19 eyes had documented visual field deficits (8 were reproducible across ≥1 sitting [frequency 14%]). After correction of plotting errors (40 eyes), RNFL thickness was 111.9 ± 17.9 μm. CNVM were present in 24 of 98 eyes (24.5%), with 21 of 24 located nasally (87.5%). Neither RNFL thinning nor identification of ONHD on fundus photography correlated with the presence of visual field defects.

Conclusions

Visual field defects due to ONHD can be reliably identified in children. In eyes of children with ONHD, RNFL protocol is frequently unreliable and may overestimate RNFL thickness. EDI scans through the ONH revealed peripapillary CNVM in nearly a quarter of the patients. Further longitudinal studies looking at the progression of CNVM and visual field deficits are warranted.

Section snippets

Subjects and Methods

This retrospective review was approved by the Duke University Health System Institutional Review Board and conformed to the requirements of the US Health Insurance Portability and Accountability Act of 1996.

The medical records of patients <18 years of age at presentation who were referred to the pediatric neuro-ophthalmology clinic to rule out papilledema reviewed to identify those found to have pseudopapilledema due to ONHD. The absence of papilledema was confirmed by the presence of ONHD on

Results

A total of 52 children (average age, 10.8 ± 3.3 years) were enrolled (98 eyes, 90.4% bilateral). Race distribution was as follows: 44 white, 5 Hispanic, 2 Asian, 1 African American (Table 1).

Automated visual field data was available for 42 patients, of whom 28 of were deemed reliable in the right eye (average age, 12.3 ± 2.6 years) and 29 reliable in the left eye (average age, 12.3 ± 2.6 years). Average ages for those children who did not have reliable visual fields in the right eye and left

Discussion

Children with pseudopapilledema due to ONHD can have significant visual field deficits and CNVM. Central vision is unlikely to be affected. To our knowledge, this is the first study to evaluate the incidence of CNVM in pediatric patients with ONHD. Literature describing reliable visual field deficits in children with ONHD is also scarce.⁷

Patients are often referred to subspecialty clinics to rule out dangerous causes of optic nerve head elevation, most notably optic disk edema.¹² Work-up of

Literature Search

The authors searched PubMed without date restriction in July 2015 using the following terms in combination: optic nerve head drusen, choroidal neovascular membrane, children, incidence.

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Cited by (28)

Distinguishing Papilledema from Pseudopapilledema in Children
2024, Advances in Ophthalmology and Optometry
The visual morbidity of optic nerve head drusen: a longitudinal review
2023, Journal of AAPOS
The ophthalmologic complications of optic nerve head drusen (ONHD) in adults have been documented, whereas data on the degree of visual morbidity from OHND in children are limited.
The medical records of all patients diagnosed with ONHD at a single, tertiary care ophthalmology department from January 1, 2010, until July 1, 2018, were reviewed retrospectively. Patients were identified using ICD-9 and ICD-10 codes. Inclusion criteria were age ≤18 years of age and formal documentation of ONHD by ancillary testing.
A total of 213 patients (386 eyes with ONHD) met inclusion criteria. Mean age at diagnosis was 10.13 ± 4.09 years, and mean follow-up was 2.76 ± 2.91 years. Formal visual fields were available for 208 eyes. Repeatable visual field defects were noted in 24 eyes (11.5%). The most common defect was a nasal step, which occurred in 11 eyes (45.8%). Fifteen eyes had visual field defects at presentation, and 9 eyes developed field loss within 1.39 ± 0.55 years of diagnosis. There was no correlation found between intraocular pressure and degree of visual field loss. Choroidal neovascular membranes were clinically apparent in 5 eyes and treatment was required in 3 eyes. Nonarteritic ischemic optic neuropathy developed in 2 eyes.
Visual morbidity associated with ONHD in children is common and may develop in a short period of time after initial diagnosis. There was no correlation found with intraocular pressure.
Peripapillary choroidal neovascularization related to optic disc drusen treated by ranibizumab: Results at 6 years
2021, Journal Francais d'Ophtalmologie
Approach to patient with unilateral optic disc edema and normal visual function
2021, Journal of the Neurological Sciences
Citation Excerpt :
Confirming the diagnosis of ODD avoids further investigations including unnecessary neuroimaging, however, ODD is not always a benign finding. A peripapillary choroidal neovascular membrane may be present in one quarter of cases, which is usually asymptomatic but may require treatment in some [15]. ODD are also associated with higher risk of developing non-arteritic anterior ischemic optic neuropathy (NAION).
In patients with swollen optic nerve head and normal visual function, optic disc drusen (ODD) is the most common diagnosis. The best tests for detecting ODD are funds autofluorescence and enhanced-depth imaging ocular coherence tomography (EDIOCT). After ODD has been ruled out, asymmetric papilledema should be assumed to be the cause and MRI of the brain and orbits with contrast and venography should be performed in all patients. It allows one to look for indirect signs of increased inctracranial pressure (ICP), optic perineuritis, and other inflammatory or compressive processes affecting optic nerve or its sheath such as optic nerve sheath meningioma. If imaging signs of raised ICP are present, lumbar puncture should be performed with measurement of opening pressure and analysis of cerebrospinal fluid (CSF) contents in all patients with fever, meningismus or neurologic deficits as well as patients who are not in the typical demographic group for idiopathic intracranial hypertension (IIH). Optic nerve sheath enhancement on MRI should prompt work-up for causes of optic perineuritis. When the appropriate neuroimaging is normal, the differential diagnosis is limited and ophthalmological consultation is necessary to determine whether other subtle ocular abnormalities are present on biomicroscopic and dilated fundus examination.
Retinal Manifestations of Idiopathic Intracranial Hypertension
2021, Ophthalmology Retina
Citation Excerpt :
Choroidal neovascular membranes associated with optic nerve abnormalities have been well described and result in paracentral scotomas distinct from the optic neuropathy. Choroidal neovascular membranes also have been reported in association with other optic nerve abnormalities, including optic disc drusen and optic neuritis60–62; they also may be idiopathic.63 The pathophysiologic features are thought to be related to axoplasmic flow stasis, choroidal hypoxia, and secondary angiogenesis.38,50,52,64
This review presents a case series and systematic review to understand retinal changes in patients with idiopathic intracranial hypertension (IIH) using fundus photography and OCT.
IIH is a condition of raised intracranial pressure of unknown cause, usually observed in young, obese women. Ophthalmologic manifestations of IIH such as papilledema and abducens nerve palsy are well recognized, but less common retinal manifestations may occur.
A retrospective institutional chart review (July 2018–March 2020) was performed on consecutive IIH patients. Fundus photographs were obtained followed by neuro-ophthalmology assessment to elicit clinical characteristics and for diagnosis. Patients who met the modified Dandy criteria were included. A systematic review of observational studies was conducted using Ovid MEDLINE and EMBASE to November 17, 2019, to supplement the case series data.
Of 144 consecutive IIH clinical patients reviewed, 10 (6.9%) and over 182 patients from the literature showed retinal findings (% in case series, % in literature, respectively): subretinal fluid (SRF; 30,9), chorioretinal folds (30,68), macular exudate (ME; 20,5), choroidal neovascular membrane (CNVM; 10,15), venous stasis retinopathy (VSR; 10,2), choroidal infarction (0,1), and branch retinal artery occlusion (BRAO; 0,1). Eight clinical patients were women (80%), average age was 32.00 ± 13.99 years, body mass index was 40.63 ± 7.43 kg/m², baseline visual acuity (VA) was 0.79 ± 0.30 in both eyes, and visual field (VF) results were –9.89 ± 11.52 dB in both eyes. Among clinical patients, 2 (1 with SRF, 1 with CNVM) had distinctive retina-related VF defects at presentation. Outer retinal abnormalities persisted on OCT in patients after resolution of SRF and papilledema. Surgical treatment (peritoneal shunt) was required for 2 patients (1 with VSR, 1 with SRF); others were treated with weight loss and acetazolamide alone. The patient with significant ME had hypertension that was treated.
Significant retinal manifestations associated with IIH include CNVM, ME, SRF, VSR, chorioretinal folds, choroidal infarction, and BRAO. These may reduce VA or cause VF defects unrelated to papilledema, emphasizing the importance of a detailed dilated fundus examination. Consultation with a retina specialist is advised in patients with peripapillary CNVM.
Choroidal neovascularisation associated with optic nerve head drusen: Case report and review of literature
2019, Archivos de la Sociedad Espanola de Oftalmologia
Varón de 21 años en seguimiento en el servicio de oftalmología por drusas del nervio óptico (DNO). En una revisión rutinaria se evidenció la presencia de neovascularización coroidea (NVC) bajo el haz papilomacular en el ojo derecho (OD). El paciente fue tratado con 2 inyecciones intravítreas de anti-vascular endothelial growth factor (anti-VEGF, por sus siglas en inglés) con buena respuesta. La aparición de NVC en el haz papilomacular es una complicación infrecuente en los pacientes con DNO. Estas NVC presentan una buena respuesta al tratamiento con inyecciones intravítreas de anti-VEGF.
A 21-year-old man was seen for follow-up of optic nerve head drusen (ONHD) in the ophthalmology department. Funduscopy revealed the presence of choroidal neovascularisation (CNV) in the papillomacular bundle of his right eye (RE). The patient was successfully treated with two intravitreal injections of anti-vascular endothelial growth factor (anti-VEGF). Peri-papillary choroidal neovascularisation in papillomacular bundle is a rare complication in patients with ONHD. These forms of CNV show a good response to anti-VEGF treatment.

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Major ArticleThe incidence of neovascular membranes and visual field defects from optic nerve head drusen in children

Purpose

Methods

Results

Conclusions

Section snippets

Subjects and Methods

Results

Discussion

Literature Search

J AAPOS

Ophthalmology

Am J Ophthalmol

Ophthalmology

J AAPOS

J AAPOS

Ophthalmology

Am J Ophthalmol

Optic disc drusen

Surv Ophthalmol

Echographic evaluation of optic disc drusen

J Clin Neuroophthalmol

Visual field defects and retinal nerve fiber layer defects in eyes with buried optic nerve drusen

Am J Ophthalmol

Visual field defects due to optic disk drusen in children

Graefes Arch Clin Exp Ophthalmol

Major Article
The incidence of neovascular membranes and visual field defects from optic nerve head drusen in children