High rate of concurrent retinal non perfusion encountered in Pediatric patients with optic nerve hypoplasia

A new study published in the American Journal of Ophthalmology suggests that there seems to be a significant rate of concomitant retinal nonperfusion in pediatric optic nerve hypoplasia (ONH) patients.

This study was carried out by Natasha da Cruz and colleagues to report the correlation between peripheral retinal nonperfusion, secondary problems, and optic nerve hypoplasia in pediatric patients.

The Bascom Palmer Eye Institute conducted the Retrospective case series investigation between January 2015 and January 2022. Age under 18 years old, a clinical diagnosis of optic disc hypoplasia, and an acceptable-quality FA were the inclusion criteria.

The key findings of this study were:

1. Seven patients (11 eyes) satisfied the criteria for inclusion.

2. The mean follow-up duration was 34.28 months (range 2 - 87 months), and the average

age at presentation was 3.5 years (range 1 month - 8 years).

3. Four patients (57.14%) had bilateral hypoplasia of the optic disc.

4. Peripheral retinal nonperfusion was seen in all eyes on FA, with mild severity occurring

in 7 eyes (63.63%), moderate severity in 2 eyes (18.18%), severe severity in one eye

(9.09%), and extreme severity in one eye (9.09%).

5. In eight eyes (72.72%), there was proof of complete retinal nonperfusion.

6. At the time of diagnosis, two patients (18.18%) had concomitant retinal detachments that were judged inoperable. Researchers witnessed every case without taking any action. During follow-up, no patient was seen to have any problems.

This discovery is not entirely understood, and the association between optic nerve hypoplasia and retinal disorders including familial exudative vitreoretinopathy as well as its connection with septo-optic dysplasia has not yet been thoroughly defined.

In conclusion, peripheral retinal nonperfusion, which is frequent in children with optic nerve hypoplasia, can be challenging to identify without anesthetic testing or fluorescein angiography. FA is a useful technique to identify peripheral nonperfusion in these circumstances. Retinal Results can be faint in certain situations, and in children with poor imaging conducted without a physical examination under anesthesia, they could not be discernible at all.

Reference: 

da Cruz, N. F. S., Sengillo, J. D., Fan, J., Negron, C. I., Felder, M. B., & Berrocal, A. M. (2023). Peripheral Retinal Nonperfusion in Pediatric Patients with Optic Disc Hypoplasia. In American Journal of Ophthalmology. Elsevier BV. https://doi.org/10.1016/j.ajo.2023.03.024