Peripapillary hyperreflective ovoid mass-like structures-a novel entity as frequent cause of pseudopapilloedema in children

Abstract

Background: Optic nerve head drusen (ONHD) are considered the most common cause for pseudopapilloedema in children. We aimed to investigate and further characterize a new type of optic nerve head lesion on enhanced depth imaging optical coherence tomography (EDI-OCT) named peripapillary hyperreflective ovoid mass-like structures (PHOMS), and ONHD in asymptomatic children with pseudopapilloedema.

Methods: Retrospective cohort study including 64 eyes from 32 patients with pseudopapilloedema due to PHOMS and/or ONHD. Mean age was 9.0 ± 3.1 years. PHOMS and ONHD were identified and classified on EDI-OCT and infrared images. Ultrasound images were classified for the presence of hyperechogenic structures of the optic nerve head.

Results: On EDI-OCT, PHOMS were detected in 63 out of 64 eyes (98.4%). In 60 eyes (93.8%), small hyperreflective foci inside the PHOMS were present. In all cases, we identified a new ring sign visible on infrared images, corresponding clearly to the edge of the PHOMS as seen on EDI-OCT. On ultrasound, we describe a new feature of PHOMS appearing as small hyperechogenic structures without posterior shadowing. In 13 eyes (20.3%), ONHD were present on EDI-OCT and ultrasound.

Conclusion: This is the first study showing that PHOMS are the most common cause for pseudopapilloedema in children. PHOMS is a new entity of optic nerve head lesions. It might be a precursor of buried optic nerve head drusen, which can lead to visual field defects, haemorrhages and CNV. This study offers new tools to identify and follow-up these lesions early in childhood using EDI-OCT.

Fig. 1. Right eye of a 7-year-old boy with peripapillary hyperreflective ovoid-like mass structures.

a–d Infrared images (a, c) showing a ring (arrows) nasal to the optic disc, correlating with the exact location of the peripapillary hyperreflective ovoid-like mass structures (PHOMS) edge shown on enhanced depth imaging optical coherence tomography (EDI-OCT; b, d, arrows). e Ultrasound B-scan showing a hyperechogenic structure (arrow) at the optic nerve head, correlating with the PHOMS seen on EDI-OCT.

Fig. 2. Right eye of a 10-year-old boy with peripapillary hyperreflective ovoid-like mass structures.

a, b Infrared image (a) showing a ring (arrows) nasal to the optic disc, correlating with the exact location of the peripapillary hyperreflective ovoid-like mass structures (PHOMS) edge shown on enhanced depth imaging optical coherence tomography (EDI-OCT) in (b) (arrows). The PHOMS contains small hyperreflective spots (encircled). c Ultrasound B-scan showing a hyperechogenic structure (arrow) at the optic nerve head, correlating with the PHOMS seen on EDI-OCT.

Fig. 3. Left eye of a 12-year-old girl with optic nerve head drusen and peripapillary hyperreflective ovoid-like mass structures.

a, c Autofluorescence images showing the optic nerve head drusen (ONHD) as a hyperautofluorescent structure (arrow). b Enhanced depth imaging optical coherence tomography (EDI-OCT) showing peripapillary hyperreflective ovoid-like mass structures (PHOMS; arrow heads) and d showing the ONHD as a hyporeflective lesion with hyperreflective edge. e Ultrasound B-scan showing a flat superficial hyperechogenic structure (asterisk), correlating to the PHOMS and a larger deeper hyperechogenic structure correlating with the ONHD.

Fig. 4. Left eye of a 9-year-old girl with peripapillary hyperreflective ovoid-like mass structures.

a–c Infrared image (a) showing a ring (arrow) temporal to the optic disc, correlating with the exact location of the peripapillary hyperreflective ovoid-like mass structures (PHOMS) edge shown on enhanced depth imaging optical coherence tomography (EDI-OCT) in (c) (arrow). The PHOMS contains small hyperautofluorescent spots seen on fundus autofluorescence (b, encircled), and hyperreflective spots on the infrared images (c, encircled). d Ultrasound B-scan showing a hyperechogenic structure (arrow) at the optic nerve head, correlating with the PHOMS seen on EDI-OCT.