Peripapillary hyperreflective ovoid mass-like structures with cystoid macular edema: a case report.

BACKGROUND: Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are newly characterized lesions wedged around the optic discs, which used to be misdiagnosed. Better understanding and identifying PHOMS are important for monitoring the condition of optic nerve. CASE PRESENTATION: A young female presented to the ophthalmic clinic with blurred vision of both eyes. Protrusions resembling "C-shaped donut" were found circling the optic discs bilaterally. These lesions were homogenous hyperreflective on OCT, while they were also hypoautofluorescent and hypoechogenic. Meanwhile, cystoid macular edema (CME) was also identified in both eyes. The patient was then diagnosed as PHOMS with CME. A short-term glucocorticoids therapy was prescribed systemically. The logMAR best-corrected visual acuity (BCVA) of both eyes reached 0.0 in 4 months with recovery of CME, while the PHOMS remained. CONCLUSIONS: There is currently no report on PHOMS with CME. More attentions should be paid to PHOMS, for they are potential biomarkers for axoplasmic stasis involved in different diseases of the optic nerve.

Association of peripapillary hyper-reflective ovoid masslike structures and disease duration in primary progressive multiple sclerosis.

BACKGROUND AND PURPOSE: Peripapillary hyper-reflective ovoid masslike structures (PHOMS) are a novel finding during retinal optical coherence tomography in patients with multiple sclerosis (MS). To date, there are no data on the occurrence of PHOMS in early MS. The aim of this study was to investigate the frequency of PHOMS in patients with first diagnosed early relapsing-remitting MS (RRMS) and to search for associations of PHOMS with disease patterns in different MS subtypes. METHODS: This was a cross-sectional analysis in two different cohorts: cohort 1, consisting of early RRMS patients (n = 349); cohort 2, consisting of patients with primary progressive MS (PPMS) (n = 66) and RRMS (n = 65). RESULTS: Peripapillary hyper-reflective ovoid masslike structures were detected in 18.3% of patients with early RRMS. The occurrence of PHOMS was not associated with age, disease duration and disability. Investigating clinical patterns and the occurrence of PHOMS (cohort 2), an association of PHOMS with higher Expanded Disability Status Scale measures (PHOMS 4.9, 3.7-6.1; no PHOMS 3.5, 3.0-5.3; p = 0.03) and longer disease durations (PHOMS 6.5 years, 1.9-11.0; no PHOMS 1.0 years, 0.0-4.0, p = 0.0007) was found in patients with PPMS but not RRMS. After p value adjustment, the disease duration appeared to be more relevant (ß = 0.16, p = 0.06). CONCLUSION: Peripapillary hyper-reflective ovoid masslike structures were found in 18% of patients with early MS. The presence of PHOMS might be associated with disease progression only in PPMS but not RRMS, suggesting that PHOMS might be embedded in neurodegenerative processes.

Multirater Validation of Peripapillary Hyperreflective Ovoid Mass-like Structures (PHOMS).

Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are a new retinal optical coherence tomography (OCT) finding. The Optic Disc Drusen Studies Consortium had made recommendations to distinguish PHOMS from true optic disc drusen (ODD) in 2018. While publications on PHOMS have increased since then, the accuracy of the definition of PHOMS and reliability of detection is unknown. In this multi-rater study, we demonstrate that the 2018 definition of PHOMS resulted in a poor multi-rater kappa of 0.356. We performed a Delphi consensus process to develop a consistent and refined definition of PHOMS with clear principles around the nature of PHOMS and how they differ from normal anatomy. Fifty explanatory teaching slides, provided as supplementary material, allowed our expert group of raters to achieve a good level of agreement (kappa 0.701, 50 OCT scans, 21 raters). We recommend adopting the refined definition for PHOMS.

Peripapillary hyperreflective ovoid mass-like structures and the retinal nerve fiber layer thinning.

AIM: We describe a report of three cases of bilateral Peripapillary Hyperreflective Ovoid Mass-Like Structures (PHOMS), their respective multimodal imaging, and retinal nerve fiber layer (RNFL) analysis over time. METHODS: We performed an elaborated multimodal imaging of three pediatric patients with PHOMS. We performed a visual acuity testing, followed by a biomicroscopic and fundus examination, an additional Optical Coherence Tomography (OCT), fundus autofluorescence (FAF), infra-red (IR), fluorescein angiography (FA), Scanning laser Ophtalmoscopy (SLO), and retro-mode imaging. Furthermore, we analyzed RNFL thickness over several consecutive visits. RESULTS: The multimodal approach exhibited similar characteristics of PHOMS in all eyes, namely a torus-like shaped that was particularly demarcated and well visualized using SLO and retro-mode tool. In all the eyes, we found a downward trend of the RNFL over time. In both the right and left eye, RNFL at presentation averaged at 152.33 ± 25.42 and 130 ± 18.33 microns, respectively. Several weeks after, it averaged at 142 ± 30.34 and 125.67 ± 14.84 microns, respectively. CONCLUSIONS: Our report shows a thinning trend of the RNFL thickness over time in patients with PHOMS.

Peripapillary Hyperreflective Ovoid Mass-Like Structures in Stickler Syndrome.

PURPOSE: To report a previously undescribed finding of peripapillary hyperreflective ovoid mass-like structures (PHOMS) in Stickler syndrome. DESIGN: Noncomparative case series. SUBJECTS: Twenty-two eyes with anomalous optic disc from 11 Stickler syndrome patients were identified and imaged. METHODS: Peripapillary hyperreflective ovoid mass-like structures were graded using enhanced-depth imaging OCT (EDI-OCT) according to the consensus recommendations of the Optic Disc Drusen Studies Consortium. All EDI-OCT scans were obtained using the Heidelberg Spectralis (Heidelberg Engineering) with a dense horizontal raster (15 × 10°, 97 sections) centered on the optic nerve head and graded by 2 independent assessors. In case of disagreement, the image was graded by a third assessor. The presence of any coexisting optic disc drusen was also assessed using EDI-OCT and autofluorescence. MAIN OUTCOME MEASURES: The presence of PHOMS, clinical characteristics and genetic mutations. RESULTS: A pilot sample of 22 eyes with phenotypic optic disc abnormalities from 11 Stickler syndrome patients were identified and imaged. Eight patients were female and 3 were male. The mean age was 31 years (13-58 years). Peripapillary hyperreflective ovoid mass-like structures were present in 91% (n = 20) of imaged eyes. Seventy percent (n = 14) were type 1 Stickler syndrome and 30% (n = 6) were type 2 Stickler syndrome. All eyes were myopic and the degree of myopia did not seem to affect whether or not PHOMS was present in this cohort. One eye with PHOMS had retinal detachment, and 77.3% (n = 17) of eyes had undergone 360o prophylactic retinopexy. Thirty-two percent (n = 7) of eyes with PHOMS were present in patients with coexisting hearing loss and 22.7% (n = 5) had orofacial manifestation of Stickler syndrome in the form of a cleft palate. Seventy-seven percent (n = 15) of eyes with PHOMS were present in patients who reported joint laxity or symptoms of arthritis. No coexisting optic disc drusen were identified and raised intracranial pressure was also excluded after neurological investigation. CONCLUSIONS: These data suggest that PHOMS are a novel finding in Stickler syndrome patients and should be considered when evaluating the optic nerves of these patients. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Application of novel non-invasive ophthalmic imaging to visualize peripapillary wrinkles, retinal folds and peripapillary hyperreflective ovoid mass-like structures associated with elevated intracranial pressure.

Background: Elevated intracranial pressure (ICP) is a serious and potentially life-threatening condition, for which clinically useful non-invasive measures have been elusive, in some cases due to their inadequate sensitivity and specificity. Our aim was to evaluate novel non-invasive ophthalmic imaging of selected pathological features seen in elevated ICP, namely peripapillary hyperreflective ovoid mass-like structures (PHOMS), peripapillary wrinkles (PPW) and retinal folds (RF) as potential biomarkers of elevated ICP. Methods: This single-center pilot study included subjects with untreated or incompletely treated high ICP. The retinas of these subjects were evaluated with averaged en-face optical coherence tomography (OCT), OCT retinal cross-sections (OCT B-scans), adaptive optics scanning light ophthalmoscopy (AOSLO), and fundus photos. Results: Seven subjects were included in the study. 6 subjects with high ICP (5 idiopathic intracranial hypertension, 1 medication induced, 30.8 ± 8.6 years, 75% female, 5 with papilledema) and 1 control (20-25 years) were included. PHOMS, PPW and RF were present in all subjects with papilledema, but neither in the high ICP subject without papilledema nor in the control subject. Averaged en-face OCT scans and AOSLO were more sensitive for PPW and RF than OCT B-scans and commercial fundus photos. Conclusion: PPW, RF and PHOMS volume have potential as non-invasive biomarkers of ICP. Novel imaging modalities may improve sensitivity. However, lack of automated image acquisition and processing limits current widespread adoption in clinical settings. Further research is needed to validate these structures as biomarkers for elevated ICP and improve clinical utility.

Peripapillary hyper-reflective ovoid mass-like structures (PHOMS) in AQP4-IgG-positive neuromyelitis optica spectrum disease (NMOSD) and MOG-IgG-associated disease (MOGAD).

BACKGROUND: Peripapillary hyperreflective ovoid mass-like structures (PHOMS) have recently been described as new optical coherence tomography (OCT) marker. It is not yet clear whether the occurrence of PHOMS is disease-specific or disease-spanning. PHOMS have been described in 16-18% of patients with multiple sclerosis (MS). Currently, no data on the prevalence of PHOMS in other demyelinating diseases including aquaporine-4-IgG-positive neuromyelitis optica spectrum disease (AQP4 + NMOSD) or myelin oligodendrocyte glycoprotein-IgG-associated disease (MOGAD) are reported. METHODS: We performed a cross-sectional, retrospective spectral domain OCT study evaluating the frequency of PHOMS in AQP4 + NMOSD (n = 47) and MOGAD (n = 44) patients. To test the association with retinal neuroaxonal damage, we compared demographic and clinical data as well as retinal layer thicknesses between eyes with vs. eyes without PHOMS. RESULTS: PHOMS were detected in 17% of AQP4 + NMOSD and 14% of MOGAD patients. Intra-cohort analysis revealed that AQP4 + NMOSD patients with PHOMS were significantly older [mean (years): 57.5 vs. 50.0; p value = 0.04]. We found no association of PHOMS with retinal neuroaxonal degeneration. In addition, in subjects with only one eye affected by PHOMS compared with the unaffected fellow eye, no differences in retinal parameters were observed (n = 4). CONCLUSIONS: In summary, we found PHOMS in 17% of AQP4 + NMOSD and 14% of MOGAD patients. This is comparable to the prevalence of published MS PHOMS data. Therefore, a disease-specific occurrence of PHOMS is unlikely. Interestingly, PHOMS do not seem to depend on retinal neuroaxonal degeneration.

Optic Nerve Drusen Evaluation: A Comparison between Ultrasound and OCT.

This observational study compared optic coherence tomography (OCT) and B-scan in the detection of optic disc drusen. In total, 86 eyes of 50 patients with optic disc drusen (ODD) (36 bilateral) with a mean age of 34.68 ± 23.81 years, and 54 eyes of 27 patients with papilledema, with a mean age of 35.42 years ± 17.47, were examined. Patients with ODD, diagnosed with ultrasound, underwent spectral-domain OCT evaluation. With US, 28 ODD cases were classified as large (4 buried and 24 superficial), 58 were classified as point-like (6 buried, 49 superficial and 3 mixed). Then, all patients underwent OCT. OCT was able to detect the presence of ODD and/or peripapillary hyperreflective ovoid mass structure (PHOMS) in 69 eyes (p < 0.001). In particular, 7 eyes (8.14%) showed the presence of ODD alone, 25 eyes (29.07%) showed only PHOMS and 37 eyes (43.02%) showed ODD and PHOMS. In 17 eyes (19.77%) no ODD or PHOMS were detected. In the papilledema group, no ODD were observed with both US and OCT. OCT showed the presence of drusen or similar lesions in only 80.23% of the cases highlighted by the US scan, so it does not allow for certain ODD diagnoses, especially in the case of buried ODD.

Volumetric Measurement of Peripapillary Hyperreflective Ovoid Masslike Structures in Patients with Optic Disc Drusen.

Purpose: To develop a method to determine the volume of peripapillary hyperreflective ovoid masslike structures (PHOMS) and to examine the correlation between PHOMS and anatomic optic nerve head characteristics in a large cohort of patients with optic disc drusen (ODD). Design: Retrospective, observational study of patients with ODD. Participants: Patients with ODD seen in a 3-year period. Methods: We determined the prevalence of PHOMS. We then developed a method to calculate the volume of PHOMS and measured this in all patients where radial scans on OCT were available. We analyzed the correlation between PHOMS volume and patient age, size of Bruch's membrane opening (BMO), ODD visibility, and anatomic location of ODD in the optic nerve. Main Outcome Measures: Prevalence and characteristics of PHOMS in patients with ODD. Results: In 247 (77%) eyes with ODD, PHOMS were found. Among these, 80% were in the first decade of life, 87% were in the second decade, 89% were in the third decade, 85% were in the fourth decade, 74% were in the fifth decade, 73% were in the sixth decade, 58% were in the seventh decade, 40% were in the eighth decade, and 0% were in the ninth decade. The ophthalmoscopic visibility of ODD increased with age. The volume of PHOMS decreased with age, but with no correlation to the size of BMO. The median volume of PHOMS was 0.27 mm3 (interquartile range [IQR], 0.13-0.49 mm3). Predominantly, PHOMS were observed in the nasal peripapillary area (87.5% nasal, 78.5% superior, 67% inferior, and 63.5% temporal). Conclusions: In patients with ODD, PHOMS are seen frequently, with the highest prevalence in younger individuals. The volume of PHOMS decreases with age, and PHOMS are seen more frequently in patients with superficial ODD.

OCT Based Interpretation of the Optic Nerve Head Anatomy and Prevalence of Optic Disc Drusen in Patients with Idiopathic Intracranial Hypertension (IIH).

We aimed to systematically examine the optic nerve head anatomy in patients with idiopathic intracranial hypertension (IIH) using a standardized optical coherence tomography (OCT) protocol. The study retrospectively included 32 patients diagnosed from 2014 to 2021 with IIH. Using OCT, in accordance with a standardized scanning protocol for patients with optic disc drusen, the presence of optic disc drusen, prelaminar hyperreflective lines, peripapillary hyperreflective ovoid mass-like structures, the retinal nerve fiber layer thickness, and macular ganglion cell layer volume was obtained. Optic disc drusen were found in 3.1%, hyperreflective lines in 31.3%, and peripapillary hyperreflective ovoid mass-like structures in 81.3% of all IIH patients at least three months after the time of diagnosis. We found no significant differences in retinal nerve fiber layer thickness or macular ganglion cell layer volume in patients with hyperreflective lines or PHOMS respectively compared to patients without hyperreflective lines (p = 0.1285 and p = 0.1835). In conclusion, the prevalence of optic disc drusen in IIH patients is similar to the reported prevalence in the general population. The high prevalence of hyperreflective lines and peripapillary hyperreflective ovoid mass-like structures in IIH patients suggest these structures be a result of crowding in the optic nerve head caused by papilledema.

Elimination of mother-to-child transmission of HIV: lessons learned from success in Thailand.

In 1988, the generalised HIV/AIDS epidemic in Thailand began and in the same year the first HIV-exposed infant in Thailand was born at King Chulalongkorn Memorial Hospital, Bangkok. From the early to mid-1990s, an epidemic wave of HIV-infected women and infants occurred. Heterosexual HIV transmission, as described in the Asian Epidemic Model, was the major mode of spread in Thailand, causing an increasing number of HIV-infected pregnant women. The early and concerted multi-sectoral response of Thai society reduced the prevalence of HIV infection in pregnant women from 2% in the mid-1990s to 0.6% in 2015 and mother-to-child transmission of HIV (MTCT) from an estimated 20-40% to 1.9%. Thus, Thailand became the first Asian country to achieve the World Health Organization's (WHO) targets for the elimination of MTCT. In this narrative review, the key historic evolutions of the science and policy of prevention of mother-to-child transmission of HIV (PMTCT) in Thailand that addressed the four prongs of the recommended WHO PMTCT strategy are described, and the lessons learned are discussed.