Peripapillary Hyperreflective Ovoid Mass-like Structures (PHOMS)
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Peripapillary Hyperreflective Ovoid Mass-like Structures (PHOMS)

  • Definition: PHOMS are oval, hyperreflective structures visualized on OCT B-scans around the optic nerve head, considered a marker of axoplasmic stasis.

  • Prevalence: Found in 7% of eyes across a broad spectrum of neurologic disorders; higher prevalence (up to 44% in intracranial hypertension [IH]) compared to 3-4% in healthy controls.

  • Associated Conditions:

    • Neuroimmunologic diseases (NID) (e.g., multiple sclerosis, neuromyelitis optica spectrum disorders): 4% prevalence.

    • Epilepsy: 7% prevalence.

    • Movement disorders (MD): 6% prevalence.

    • Intracranial hypertension (IH): 44% prevalence, strongly associated with increased intracranial pressure (ICP).

    • Inborn errors of metabolism (IEM): 9% prevalence.

  • Localization: Predominantly nasal (>65%), with minimal temporal involvement (5-10%); exclusively temporal PHOMS reported in Leber’s hereditary optic neuropathy.

  • Volume:

    • Median PHOMS volume: 0.06 mm³.

    • Significantly larger in IH (median 0.23 mm³) compared to NID (0.03 mm³), epilepsy (0.05 mm³), and MD (0.02 mm³).

    • Larger volumes in IH suggest a link to elevated ICP.

  • Intensity: Comparable to optic nerve intensity (0.99 ± 0.19), lower than retinal layers, higher than outer nuclear layer; no significant intensity differences across cohorts.

  • Correlations:

    • Positive correlation with peripapillary retinal nerve fiber layer (pRNFL) thickness (global, nasal-inferior, temporal-inferior, temporal segments).

    • Positive correlation with Bruch membrane opening minimum rim width (BMO MRW), likely due to peripapillary retinal layer deflection.

    • Negative correlation with age (excluding IH patients), suggesting larger PHOMS in younger patients.

    • No correlation with body mass index (BMI) or BMO surface area.

  • Pathophysiology Hypotheses:

    • Axoplasmic stasis (supported by histopathologic findings in papilledema).

    • Impairment of glymphatic drainage or translaminar pressure gradient.

  • Diagnostic Importance: PHOMS are a non-specific marker but more frequent in neurologic disorders; must differentiate from optic disc drusen or papilledema.

  • Clinical Pearl: Larger PHOMS volumes in IH are a key exam question; consider PHOMS in OCT interpretation for neurologic patients to avoid misdiagnosis.

  • Research Implications: PHOMS presence impacts OCT parameters (e.g., pRNFL, BMO MRW), requiring consideration in non-ophthalmic neurologic studies.

  • Limitations: Cross-sectional study design and cohort heterogeneity (age, disease duration) limit conclusions on causality or disease-specific mechanisms.

Citation

Gemert JA, Christmann T, Kaufmann E, et al. Characterization of Peripapillary Hyperreflective Ovoid Mass-like Structures in a Broad Spectrum of Neurologic Disorders. Ophthalmology. 2025;132(5):590-597. https://doi.org/10.1016/j.ophtha.2024.12.013

Intrapapillary Hemorrhage with Adjacent Peripapillary Subretinal Hemorrhage (IHAPSH)

General Overview

  • IHAPSH is an increasingly recognized condition, often associated with myopia-related optic disc changes.

  • Typically presents in young adults and school-aged children, particularly in East Asian populations.

  • Spontaneous resolution of hemorrhages and optic disc swelling is common without treatment.

  • Strong association with myopia, suggesting structural optic nerve head abnormalities as a primary cause.

Clinical Presentation

  • Age at onset: 12–30 years (mean 18.6 years).

  • Sex distribution: Predominantly female (4:1 female-to-male ratio).

  • Affected eyes: Unilateral (most cases), with rare bilateral involvement.

  • Refractive error: Myopic, ranging from -1.75 to -7.00 D (mean -5.38 D).

  • Axial length: 24.73–27.34 mm (mean 26.30 mm).

  • Visual function: Mild impairment, with normal best-corrected visual acuity (BCVA) (e.g., 20/16) and no significant color vision or light reflex abnormalities.

  • Visual field defects: Mariotte’s blind spot enlargement or decreased lower visual field sensitivity in some cases, often resolving with hemorrhage.

  • Funduscopic findings:

    • Crescent-shaped subretinal hemorrhage adjacent to the nasal superior optic disc.

    • Optic disc hemorrhage and swelling.

    • Vitreous hemorrhage in some cases (3/6 eyes).

    • Peripapillary chorioretinal atrophy (PPA) in all cases.

  • Associated optic disc abnormalities:

    • Optic disc drusen, tilted disc, small disc, or superior segmental optic hypoplasia (SSOH).

  • Systemic history: No significant systemic diseases or trauma; one case linked to exercise (running).

Diagnostic Imaging

  • Optical Coherence Tomography (OCT):

    • Peripapillary hyperreflective ovoid mass-like structures (PHOMS) observed in all cases, located above Bruch’s membrane.

    • Optic disc drusen identified as low-reflective structures within the optic disc.

    • Subretinal hemorrhage visible on B-scans.

    • Affected optic nerve head shows a narrower and deeper cup compared to the contralateral eye.

    • Larger tilt angle in affected eyes.

  • OCT Angiography (OCTA): No significant abnormalities reported in most cases.

  • Fundus Autofluorescence:

    • Hyperfluorescence within the optic disc, indicative of optic disc drusen.

  • Fluorescein Angiography:

    • Hyperfluorescence at the nasal edge of the optic nerve head, suggestive of tissue staining.

  • Fundus Photography:

    • Documents optic disc hemorrhage, subretinal hemorrhage, and optic disc abnormalities (e.g., SSOH, PPA).

Pathophysiology and Risk Factors

  • Myopia-related structural abnormalities of the optic nerve head are a primary cause.

  • Weak adhesion between retinal layers in myopic eyes increases hemorrhage risk.

  • Optic disc abnormalities (small disc, tilted disc, optic disc drusen) may cause compression or circulation disturbances, predisposing to IHAPSH.

  • PHOMS:

    • Not specific to IHAPSH; also seen in pseudopapilledema, choked disc, anterior ischemic optic neuropathy, central retinal vein occlusion, and optic neuritis.

    • Associated with myopia, tilted discs, and smaller Bruch’s membrane opening.

    • Prevalence: 8.9% in healthy children aged 11–12 years.

  • Social factors: Increased tablet computer use among children may contribute to myopia progression and IHAPSH incidence.

 

Management and Prognosis

  • No treatment required: Hemorrhages and optic disc swelling resolve spontaneously within ~1 month.

  • No recurrence observed in affected eyes, and no onset in contralateral eyes in the study.

  • Monitoring:

    • Regular fundus examination and OCT to confirm resolution.

    • Visual field testing to assess Mariotte’s blind spot or sensitivity changes.

  • Long-term follow-up needed to understand IHAPSH’s relation to myopic optic neuropathy.

Epidemiology

  • Predominantly reported in East Asian populations.

  • Increasing incidence in Japan, possibly linked to myopia progression in younger populations.

  • Associated with axial length elongation during childhood and young adulthood.

Citation

  • Takemoto D, Ohkubo S, Udagawa S, Higashide T. Clinical presentation and optical coherence tomography findings of intrapapillary hemorrhage with adjacent peripapillary subretinal hemorrhage. Am J Ophthalmol Case Rep. 2025;38:102329. Available at: www.ajocasereports.com.