Purpose
Profound, permanent vision loss may occur in children with papilledema because of irreversible damage to the optic nerves. The underlying etiologies for papilledema include idiopathic intracranial hypertension (IIH) and secondary causes of intracranial hypertension (SIH). Little has been reported on the causes and visual outcomes in pediatric patients with SIH. The purpose of this study was to identify the primary causes of SIH and to evaluate visual outcomes in a large pediatric cohort.
Methods
A five-year retrospective review of pediatric patients with papilledema (ICD-9 377) seen at a single tertiary care center was performed. Underlying diagnosis, demographic information, clinical presentation, and complete ophthalmic findings were recorded. Pediatric patients with IIH were excluded.
Results
A total of 84 patients (38 females, 45%) were identified. The median age was 11 years (range 6 months to 29 years). Mean follow-up was 17 months. Underlying etiologies for SIH included obstructive hydrocephalus from brain tumor in 30 patients (37%), a congenital cause of hydrocephalus in 18 patients (21%), meningitis-related in 13 patients (15%), dural sinus thrombosis in 6 patients (7%), and trauma in 6 patients (7%). Headache was the most common initial symptom. At presentation, 8/61 (13%) patients able to complete optotype acuity had vision <20/40 in the worse seeing eye compared with 7/61 (11%) on follow up. Initial visual field testing showed an abnormality in 25/61 (41%) patients, with 15/61 (25%) of initial visual fields showing blind spot enlargement, and 10/61 (16%) showing other visual field defects. At follow up, 12/43 (28%) visual fields recorded showed either an enlarged blind spot (6) or other visual field defects (6). Optic atrophy was noted in 9/65 (14%) patients at their final visit.
Conclusions
Secondary IH can be associated with significant morbidity including vision loss, visual field defects, and optic atrophy. Although addressing the primary etiology is the mainstay of treatment, this study stresses the importance of continued ophthalmic monitoring in order to intervene before permanent vision loss ensues.
Keywords: 612 neuro-ophthalmology: diagnosis •
613 neuro-ophthalmology: optic nerve •
759 visual impairment: neuro-ophthalmological disease