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. 2024 Sep 25;103(2):e94–e103. doi: 10.1111/aos.16761

Peripapillary choroidal vascularity of paediatric myopic eyes with peripapillary hyperreflective ovoid mass‐like structures

Furkan Kirik 1,✉, Didem Dizdar Yiğit 2, Mehmet Orkun Sevik 2, Kamile Melis Ertürk 1, Farid İskandarov 1, Özlem Şahin 2, Hakan Özdemir 1
PMCID: PMC11810547  PMID: 39320010

Abstract

Purpose

To assess the peripapillary choroidal vasculature in paediatric myopic patients with and without peripapillary hyperreflective ovoid mass‐like structures (PHOMS).

Methods

This prospective study includes 60 eyes of 60 myopic (spherical equivalent [SE] <−1.00 dioptre [D]) patients with (n = 30) and without (n = 30) PHOMS (PHOMS [+] and PHOMS [−] groups, respectively), and 30 eyes of 30 age‐ and sex‐matched emmetropic children (control group). Peripapillary choroidal parameters, including total choroidal (TCA), luminal (LA), and stromal areas (SA) and choroidal vascularity index (CVI) calculated from vertical and horizontal single‐line enhanced depth imaging‐optical coherence tomography scans centred on optic nerve head.

Results

Peripapillary retinal nerve fibre layer thicknesses were not different between the groups (p > 0.05). In the PHOMS (+) group, TCA, LA and SA were lower, and CVI was higher in all quadrants compared to the control (p < 0.05). However, only the mean TCA and LA in the inferior and nasal quadrants and the mean SA in the nasal quadrant were lower in PHOMS (+) than in PHOMS (−) (p < 0.05). In the PHOMS (−) group, higher CVI was observed in all quadrants except temporal compared to the control group. Although the mean CVI of the PHOMS (+) group was also higher than in the PHOMS (−) group, this difference was not statistically significant.

Conclusion

This study indicates that choroidal parameters differ in paediatric myopic patients with PHOMS. Further studies with larger sample sizes are needed to understand the details of choroidal parameters in eyes with PHOMS.

Keywords: choroidal vascularity index, myopia, peripapillary hyperreflective ovoid mass‐like structures

1. INTRODUCTION

The peripapillary hyperreflective ovoid mass‐like structure (PHOMS) is a spectral domain (SD) optical coherence tomography (OCT) finding suggested to result from herniation of the retinal nerve fibre layer (RNFL) at the optic nerve head (ONH) into the peripapillary region due to axoplasmic stasis (Fraser et al., 2021; Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018). PHOMS was previously considered a buried variant or a preceding form of optic disc drusen (ODD) (Lee & Woo, 2017; Traber et al., 2017). However, in 2018, the Optic Disc Drusen Studies (ODDS) Consortium redefined PHOMS as a separate entity due to its distinct enhanced depth imaging OCT (EDI‐OCT) features such as ovoid inhomogeneous hyperreflectivity, lack of sharp hyperreflective margins and hyporeflective core as well as a toroidal peripapillary extent (Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018; Sibony et al., 2020). Along with the properties such as lack of autofluorescence and absence of ultrasonographic visibility distinguishing PHOMS from an ODD, the Consortium also further emphasized the coexistence of PHOMS with different ONH pathologies and suggested classifying PHOMS as (1) optic disc oedema‐associated, (2) ODD‐associated and (3) tilted/anomalous disc‐associated PHOMS (Fraser et al., 2021; Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018; Sibony et al., 2020).

Pseudopapilledema related to tilted/anomalous disc‐associated PHOMS is frequently being speculated as an under‐recognized diagnosis, leading to a redundant neurological work‐up, particularly in paediatric myopic patients (Eshun et al., 2022; Fraser et al., 2021; Lyu et al., 2020; Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018; Petzold et al., 2020; Pichi et al., 2014; Pratt et al., 2023; Sim & Milea, 2024). Recently, a cross‐sectional, population‐based study from Denmark reported a PHOMS prevalence of 8.9% in 1314 healthy children aged 11–12 years without ODD or optic disc oedema, with tilted disc being the most common associated ONH finding (51.3%) (Behrens et al., 2023). The study also revealed that the odds ratio (OR) of having PHOMS increases exponentially with increasing myopia.

While several pathologies associated with PHOMS have been identified, the anatomical and functional implications of PHOMS are still unclear (Fraser et al., 2021; Sibony et al., 2020). An optical coherence tomography angiography (OCTA) study investigating paediatric cases with PHOMS showed that peripapillary vessel densities were decreased in cases with extensive PHOMS (Ahn et al., 2022). Also, some case reports indicate that PHOMS could have individual vascular complexes demonstrated by OCTA, even connected with choroidal neovascularization, suggesting there might be associated vascular changes (Borrelli et al., 2021; Heath Jeffery & Chen, 2023; Hedels et al., 2021; Xie et al., 2022). Considering the dual vascular supply of the ONH mainly provided by choroidal circulation except for the surface retinal nerve fibre layer (RNFL) (Hayreh, 2001), investigating the choroidal vascular changes in PHOMS might further improve our understanding of this clinical OCT sign.

EDI‐OCT or swept‐source OCT is recommended to exclude ODD and establish a reliable diagnosis of PHOMS due to their deeper tissue penetration, and they are almost invariably used in all PHOMS studies (Ahn et al., 2022; Behrens et al., 2023; Lyu et al., 2020; Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018; Petzold et al., 2020; Pratt et al., 2023; Wang et al., 2024; Xie et al., 2022). However, to the best of our knowledge, there are no studies investigating choroidal vascularity index (CVI), a relatively novel EDI‐OCT (or swept‐source OCT) parameter evaluating choroidal vascular area compared to the total choroidal area (Agrawal et al., 2016), in PHOMS. Therefore, this study aims to assess peripapillary CVI in paediatric myopic patients with and without PHOMS and compare it to healthy controls.

2. METHODS

This exploratory, cross‐sectional, comparative study was conducted between November 2022 and July 2023 at Bezmialem Vakif University and Marmara University School of Medicine Hospitals, Istanbul, Turkey. The study protocol was approved by the Ethics Committee on Human Research of Bezmialem Vakif University (2022‐302‐84 988) and was conducted following the latest amendments of the Declaration of Helsinki ethical principles. All study participants and their legal guardians were provided written informed consent to participate and have their medical data used for research purposes.

2.1. Study participants

All paediatric patients with cycloplegic refraction of a SE less than +1.00 D between the ages of 6 and 18 were consecutively evaluated for study enrolment in the outpatient clinics of two study centres between the prespecified dates of November 2022 and July 2023. The study exclusion criteria were determined as patients with an age of less than six and more than 18, inability to cooperate in ophthalmological examination, cycloplegic refraction of a SE more than +1.00 D, clinical signs of pathological myopia (presence of a stage more advanced than A0T0N0 or A1T0N0 according to the previous classification; Ruiz‐Medrano et al., 2019), manifest strabismus, amblyopia, history of ocular surgery, and any other ophthalmological or systemic diseases that might be related to PHOMS (papilledema, ODD, other causes of OD oedema). The paediatric neurology department consultation was provided for the patients with PHOMS to exclude conditions leading to increased intracranial pressure, such as idiopathic intracranial hypertension or hydrocephalus. Patients unable to cooperate with SD‐OCT imaging and low‐quality SD‐OCT images were also excluded from the study. Two independent observers (FK and Fİ) assessed the EDI‐OCT images of all eyes for the presence or absence of PHOMS, and in case of any disagreement, the eyes were excluded from the study. The flowchart of the study enrolment is provided in Figure 1.

FIGURE 1.

FIGURE 1

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The flow chart of the study enrolment. D, dioptre; MRI, magnetic resonance imaging; PHOMS, peripapillary hyperreflective ovoid mass‐like structure; SD‐OCT, spectral domain optical coherence tomography; SE, spherical equivalent.

After the exclusion criteria were applied, 60 eyes of 60 paediatric myopic patients with a cycloplegic refractive error of a spherical equivalent (SE) less than −1.00 dioptre (D) and 30 eyes of 30 healthy emmetropic paediatric controls (control group) with a cycloplegic refractive error of a SE between −1.00 D and +1.00 D were included in the study. Myopic patients were further divided into two groups according to the presence (PHOMS [+] group, n = 30) and absence (PHOMS [−] group, n = 30) of PHOMS in their study eyes. The right eyes of the patients were determined as the study eye in the PHOMS (−) and control groups. For the PHOMS (+) group, the eye with PHOMS, or in the case of bilaterality, the right eye of the patient was selected as the study eye.

2.2. Study evaluations

All participants had a comprehensive ophthalmological examination, including manifest and cycloplegic refraction, best‐corrected visual acuity, slit‐lamp biomicroscopy, intraocular pressure measurement with Goldmann applanation tonometry or non‐contact tonometry (Nidek NT510; Nidek Inc., Fremont, CA) according to participant compliance, dilated fundus examination, macular and peripapillary SD‐OCT (Heidelberg Spectralis; Heidelberg Engineering, Heidelberg, Germany) and axial length (AL) measurement with optical biometry (IOL‐Master 500; Carl‐Zeiss Meditec AG, Jena, Germany).

For cycloplegic refraction, %1 cyclopentolate hydrochloride drops were instilled three times 5 min apart, and auto‐refractometry (Nidek ARK 560A; Nidek Inc., Fremont, CA) was performed 45 min after the last drop was instilled.

The presence or absence of PHOMS and exclusion of ODD was determined according to ODDS Consortium definition by conducting optic disc‐centred horizontal and vertical SD‐OCT raster scans (15 × 10°, 97 sections, averaging 100 frames) in EDI mode, fundus autofluorescence (Heidelberg Spectralis; Heidelberg Engineering, Heidelberg, Germany) averaging 100 frames, and 15 MHz B‐scan ocular ultrasonography (Compact Touch, Quantel Medical, Clermont‐Ferrand, France) (Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018).

Exploratory SD‐OCT evaluation consisted of a macular raster scan (20 × 20°, 25 sections, averaging 12 frames) to evaluate any macular pathology, optic disc‐centred peripapillary 12° circular scan to assess global and sectoral (temporal, inferior, superior and nasal) retinal nerve fibre layer (RNFL) thicknesses, and optic disc‐centred horizontal and vertical single‐line B‐scans in EDI mode to evaluate choroid. The EDI‐OCT single‐line B‐scans were obtained by averaging 100 frames provided by the eye‐tracking system of the Spectralis device (Figure 2). The EDI‐OCT scans were repeated until high‐quality B‐scans where the choroid‐sclera junction was clearly identified and a Q‐score of at least 30 was obtained. All SD‐OCT scans were conducted after pupillary dilation, in a relaxed position to avoid any effect of the Valsalva manoeuvre, and between 1:00 PM and 4:00 PM to avoid diurnal variations in evaluated parameters (Sevik et al., 2022). The ONH tilt angle was measured on horizontal EDI‐OCT images using a previously described method (Hosseini et al., 2013).

FIGURE 2.

FIGURE 2

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Sample images of peripapillary choroidal imaging in control (a, b), PHOMS (−) (c, d) and PHOMS (+) (e, f) groups with enhanced depth imaging‐spectral domain optical coherence tomography (EDI‐OCT). Horizontal (temporal‐to‐nasal) (a, c, e) and vertical (inferior‐to‐superior) (b, d, f) optic disc‐centred single‐line B‐scan EDI‐OCT imaging was performed for choroidal analysis. The margins of the peripapillary hyperreflective ovoid mass‐like structures (PHOMS) in a myopic eye are shown as white dots on the near‐infrared reflectance image (e). The white arrowhead in the cross‐sectional EDI‐OCT image indicates the subretinal located PHOMS in the peripapillary area (e, f).

For CVI assessment, the vertical and horizontal single‐line EDI‐OCT B‐scans were uploaded to the open‐access Fiji image processing software (https://fiji.sc/). Peripapillary choroidal segmentation was employed, slightly modifying the previously described method for subfoveal CVI measurement (Figure 3) (Agrawal et al., 2016). In brief, after converting the images to 8‐bit, a peripapillary area of 1500 μm was manually selected in each direction (i.e. superior and inferior, or temporal and nasal, depending on the vertical or horizontal EDI‐OCT scan selected, respectively) starting from the retinal pigment epithelium (RPE)‐Bruch's membrane opening. Then, the Niblack auto‐local threshold is applied for image binarization. The area between the sclerochoroidal junction posteriorly and RPE/Bruch's complex anteriorly was selected with the polygonal selection tool of the software and uploaded to the ROI manager as the total choroidal area (TCA). Then, the binarized image was converted to an RGB with the colour threshold tool, and the luminal area (LA) was marked by selecting the dark pixels. The software calculated TCA and LA automatically, and the stromal area (SA) was calculated by subtracting the LA from TCA. The ratio of LA to TCA was determined as CVI (%) and calculated for each of the four quadrants (i.e. superior, inferior, temporal and nasal) for each participant.

FIGURE 3.

FIGURE 3

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Measurement of peripapillary choroidal vascularity index in a patient in the PHOMS (+) group. Both horizontal (temporal‐to‐nasal) (a, b) and vertical (inferior‐to‐superior) (e, f) optic disc‐centred single‐line B‐scan enhanced depth imaging spectral domain optical coherence tomography (EDI‐OCT) was performed. The 1500 μm‐width peripapillary choroidal areas in the cross‐sectional EDI‐OCT images were selected as the region of interest and binarized with the Niblack auto‐local thresholding method (c, g). In these images, dark pixels indicate the luminal area and white pixels indicate the stromal area. Cross‐sectional EDI‐OCT images overlaid on the binarized region of interest are presented in d and h. The white asterisk in Figure 2b indicates PHOMS in the cross‐sectional EDI‐OCT image.

2.3. Statistical analysis

Statistical Package for the Social Sciences (SPSS) version 26.0 (IBM Corp., Armonk, NY, USA) for IOS was used for data analysis. Kurtosis and skewness statistics were used to evaluate the data distribution, and a value between ±1.50 for both was considered normality of distribution. Qualitative and quantitative data were presented as frequency (percentage) and mean ± standard deviation, respectively. Qualitative data were compared using the Chi‐squared test. Quantitative three‐group comparisons were employed with one‐way analysis of variance (ANOVA) with post‐hoc Tukey test or Kruskal–Wallis test with post‐hoc Bonferroni correction depending on parametric or nonparametric data distribution. Inter‐observer agreement for the detection of PHOMS was calculated using the kappa coefficient based on SD‐OCT images of the ONH from 111 eyes with interpretable EDI‐OCT images (Figure 1). A p‐value of less than 0.05 was considered statistically significant.

3. RESULTS

The demographics of the patients and eyes included in the PHOMS (+), PHOMS (−), and control groups were given in Table 1. The kappa coefficient for inter‐observer agreement in PHOMS detection was 0.851. There was no significant difference considering the mean age (p = 0.456, ANOVA) and the sex (p = 0.510, Chi‐squared test) of the patients between the three study groups. The mean SE was significantly lower, and the mean AL was significantly higher in the eyes of the PHOMS (+) and PHOMS (−) groups than of the control group (p < 0.001, for all). ONH tilt angle was significantly higher in the PHOMS (+) group compared to the other groups (p < 0.001). There was also no significant difference in global or any sectoral RNFL thicknesses among the three study groups (Table 2).

TABLE 1.

The demographic and clinical features of the study population and their eyes in the study groups.

PHOMS (+) group (n = 30) PHOMS (−) group (n = 30) Control group (n = 30) p Post‐hoc c
(+)/(−) (+)/C (−)/C
Age, years
Mean ± SD (min–max) 12.6 ± 2.9 (7–18) 13.0 ± 2.9 (8–17) 13.5 ± 2.4 (10–17) 0.476 a – – –
Gender, n (%)
Female 20 (66.7) 19 (63.3) 23 (76.7) 0.510 b – – –
Male 10 (33.3) 11 (36.7) 7 (23.3)
SE, D
Mean ± SD (min–max) −2.88 ± 1.34 (−7.25 to −1.25) −3.28 ± 1.61 (−8.13 to −1.25) −0.11 ± 0.58 (−0.88 to 0.88) <0.001 a 0.436 <0.001 <0.001
AL, mm
Mean ± SD (min–max) 24.55 ± 0.83 (23.08–27.16) 24.68 ± 1.05 (23.15–26.95) 23.30 ± 0.53 (22.38–24.22) <0.001 a 0.804 <0.001 <0.001
ONH tilt, °
Mean ± SD (min–max) 8.09 ± 2.31 (3.98–14.33) 3.50 ± 1.51 (1.21–7.33) 0.60 ± 0.32 (0.00–3.02) <0.001 a <0.001 <0.001 <0.001
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Note: Bold p‐values indicate statistical significance.

Abbreviations: AL, axial length; D, dioptre; max, maximum; min, minimum; ONH, optic nerve head; PHOMS, peripapillary hyper‐reflective ovoid mass‐like structures; SD, standard deviation; SE, spherical equivalent.

a

One‐way analysis of variance test.

b

Chi‐squared test.

c

Tukey test, p‐values adjusted for pairwise comparisons.

TABLE 2.

The comparison of the peripapillary retinal nerve fibre layer thicknesses among the study groups.

PHOMS (+) group (n = 30) PHOMS (−) group (n = 30) Control group (n = 30) p
Temporal RNFLT
Mean ± SD (min–max) 79.57 ± 14.56 (61–100) 74.97 ± 10.10 (59–121) 78.00 ± 8.64 (66–100) 0.287 a
Inferior RNFLT
Mean ± SD (min–max) 122.50 ± 16.38 (99–149) 122.73 ± 13.67 (94–161) 125.01 ± 12.99 (109–156) 0.089 a
Nasal RNFLT
Mean ± SD (min–max) 68.87 ± 15.68 (46–117) 74.80 ± 15.93 (48–104) 73.90 ± 12.80 (47–95) 0.255 a
Superior RNFLT
Mean ± SD (min–max) 125.03 ± 15.42 (77–155) 125.53 ± 14.93 (101–149) 132.80 ± 15.36 (103–168) 0.093 a
Global RNFLT
Mean ± SD (min–max) 99.10 ± 8.89 (75–117) 99.43 ± 9.52 (81–118) 103.70 ± 7.80 (91–120) 0.082 a
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Abbreviations: max, maximum; min, minimum; PHOMS, peripapillary hyper‐reflective ovoid mass‐like structures; RNFLT, retinal nerve fibre layer thickness; SD, standard deviation.

a

One‐way analysis of variance.

Considering the evaluated choroidal areas, the mean TCA, LA and SA were significantly lower in the PHOMS (+) group than in the control group in all four peripapillary quadrants (p < 0.05, for all). Meanwhile, the mean TCA, LA and SA were also lower in the PHOMS (−) group than in the control group, reaching statistical significance in most of the four peripapillary quadrants, except the mean LA in the temporal and inferior quadrants. However, although the mean TCA, LA and SA were lower in the PHOMS (+) group than in the PHOMS (−) group in all four quadrants, only the mean TCA and LA in the inferior and nasal quadrants, and the mean SA in the nasal quadrant reached statistical significance (Table 3; Figure 4).

TABLE 3.

The comparison of the peripapillary choroidal parameters among the study groups.

PHOMS (+) group (n = 30) PHOMS (−) group (n = 30) Control group (n = 30) p a Post‐hoc b
(+)/(−) (+)/C (−)/C
Temporal (mean ± SD)
TCA, mm2 0.66 ± 0.21 0.78 ± 0.26 0.95 ± 0.26 <0.001 0.167 <0.001 0.045
LA, mm2 0.46 ± 0.15 0.54 ± 0.18 0.64 ± 0.20 0.001 0.215 0.001 0.089
SA, mm2 0.19 ± 0.07 0.24 ± 0.09 0.31 ± 0.12 <0.001 0.155 <0.001 0.019
CVI, % 71.07 ± 5.42 69.46 ± 4.50 67.69 ± 3.56 0.019 0.364 0.014 0.292
Inferior (mean ± SD)
TCA, mm2 0.54 ± 0.18 0.67 ± 0.21 0.81 ± 0.23 <0.001 0.041 <0.001 0.032
LA, mm2 0.37 ± 0.12 0.46 ± 0.14 0.53 ± 0.15 <0.001 0.037 <0.001 0.133
SA, mm2 0.17 ± 0.07 0.21 ± 0.07 0.28 ± 0.09 <0.001 0.094 <0.001 0.005
CVI, % 69.58 ± 5.39 69.06 ± 4.54 65.83 ± 4.56 0.007 0.908 0.010 0.031
Nasal (mean ± SD)
TCA, mm2 0.55 ± 0.14 0.66 ± 0.20 0.95 ± 0.26 <0.001 0.031 <0.001 <0.001
LA, mm2 0.37 ± 0.09 0.45 ± 0.13 0.60 ± 0.18 <0.001 0.045 0.001 <0.001
SA, mm2 0.18 ± 0.06 0.22 ± 0.07 0.34 ± 0.09 <0.001 0.044 <0.001 <0.001
CVI, % 68.21 ± 4.93 67.50 ± 3.61 63.35 ± 4.26 <0.001 0.797 <0.001 0.001
Superior (mean ± SD)
TCA, mm2 0.64 ± 0.20 0.77 ± 0.23 1.04 ± 0.30 <0.001 0.100 <0.001 <0.001
LA, mm2 0.44 ± 0.12 0.52 ± 0.14 0.67 ± 0.19 <0.001 0.110 <0.001 0.001
SA, mm2 0.20 ± 0.09 0.25 ± 0.10 0.38 ± 0.12 <0.001 0.126 <0.001 <0.001
CVI, % 70.10 ± 5.93 68.71 ± 4.55 64.01 ± 3.81 <0.001 0.511 <0.001 0.001
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Note: Bold values indicate statistical significance.

Abbreviations: C, control; CVI, choroidal vascularity index; LA, luminal area; PHOMS, peripapillary hyper‐reflective ovoid mass‐like structures; SA, stromal area; SD, standard deviation TCA, total choroidal area.

a

One‐way analysis of variance test.

b

Post‐hoc Tukey test, p‐values adjusted for pairwise comparisons.

FIGURE 4.

FIGURE 4

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Bar graphs showing the mean total choroidal area (a), luminal area (b), stromal area (c) and choroidal vascularity index (d) of study groups according to peripapillary quadrants. PHOMS, peripapillary hyper‐reflective ovoid mass‐like structure. *Statistical significance in one‐way analysis of variance with post‐hoc Tukey test. Error bars indicate standard deviation.

The mean CVI was significantly higher in the PHOMS (+) group in all peripapillary quadrants and in the PHOMS (−) group in all but temporal peripapillary quadrants than in the control group. Although the mean CVI of the PHOMS (+) group was also higher than in the PHOMS (−) group, this difference was not statistically significant (Table 3; Figure 4).

4. DISCUSSION

To the best of our knowledge, this is the first study to evaluate the relationship of PHOMS with peripapillary choroidal structures in paediatric myopic patients. This study demonstrated consistently lower mean TCA, LA and SA values and consistently higher mean CVI values in all quadrants in the myopic eyes with PHOMS compared to myopic eyes without PHOMS and the emmetropic control group. This difference was statistically significant for myopic eyes with and without PHOMS only in the mean TCA and LA of the inferior and the mean TCA, LA and SA of the nasal quadrants. Also, all parameters were statistically significant for myopic eyes with PHOMS compared to the control group, and all but LA and CVI in temporal and LA in nasal quadrants for myopic eyes without PHOMS compared to the control group. Those findings suggest that higher peripapillary CVI might accompany eyes with PHOMS compared to those without PHOMS in myopic children.

To date, PHOMS has been demonstrated in a wide variety of clinical conditions, including papilledema, ODD, optic neuritis, multiple sclerosis, non‐arteritic ischaemic optic neuropathy, central retinal vein occlusion and myopic/tilted/anomalous optic discs (Fraser et al., 2021; Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018; Petzold et al., 2020). Therefore, it is considered an OCT accompaniment to several diseases involving the ONH (Fraser et al., 2021; Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018; Petzold et al., 2020). The clinical and histopathological evidence suggests that PHOMS consists of centrifugally bulged optic nerve fibre herniations resulting from axoplasmic stasis around the peripapillary region (Fraser et al., 2021; Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018; Petzold et al., 2020). Additionally, longitudinal studies showed PHOMS can occur de novo, progress with the disease severity and regress with the treatment of the underlying condition (Fraser et al., 2021; Malmqvist et al., 2018a, 2018b; Malmqvist, Sibony et al., 2018; Petzold et al., 2020).

In myopic children, apart from the pathological states causing ONH oedema and ODD, the most prevalent aetiology of PHOMS is tilted/anomalous optic discs (Behrens et al., 2023). Although tilted/anomalous optic discs are considered a common variant of ONH morphology without causing acute injury, they have been implicated as a predisposing factor for focal chronic axoplasmic stasis leading to the formation of PHOMS (Fraser et al., 2021; Kim et al., 2022; Lyu et al., 2020; Pichi et al., 2014). In a study evaluating ONH morphology in children with and without PHOMS, PHOMS was significantly associated with the degree of myopia and ONH tilt angle (Lyu et al., 2020). The authors concluded that ONH tilt in myopic shift seen during adolescence might mediate PHOMS (Lyu et al., 2020). Subsequently, Kim et al. (2022) demonstrated the early course of PHOMS nascency and progression with increasing myopia and ONH tilt in their case series of two children. The authors emphasized the ONH tilt as the leading cause of compressive stress, resulting in nerve fibre bulging and PHOMS formation. A population‐based cohort study from Denmark also further emphasized the association of myopic refraction and ONH tilt with PHOMS (Behrens et al., 2023). The study revealed that ONH tilt increases the risk of PHOMS with an OR of 5.96 (95% confidence interval [CI] = 3.92–9.08). Also, the risk of having PHOMS was reported to be increased with an increase in ONH tilt by 1° and decreased with a decrease in SE by 1.00 D with ORs of 1.38 (95% CI = 1.13–1.69) and 0.51 (95% CI = 1.13–1.69), respectively. These findings together lead to an interpretation of refraction as the unifying factor between ONH tilt and PHOMS (Behrens et al., 2023; Fraser et al., 2021; Kim et al., 2022; Lyu et al., 2020).

During the last few years, microvasculature networks within PHOMS have also been demonstrated with the advent of high‐resolution OCTA imaging (Borrelli et al., 2021; Heath Jeffery & Chen, 2023; Hedels et al., 2021; Xie et al., 2022). In the first series by Borrelli et al. (2021), vascular complexes that do not seem to be in continuity with peripapillary retinal vasculature in two adult patients with tilted/anomalous disc‐associated and a 10‐year‐old boy with ODD‐associated PHOMS were demonstrated. The authors stated that those vascular complexes might represent prelaminar vessel displacement or neo‐vessels triggered by increased vascular endothelial growth factor levels resulting from ischaemia due to congestion and compression with PHOMS. Later, Hedels et al. (2021) further contributed this observation with the report of an 8‐year‐old boy having vascularized PHOMS interconnected with inactive subretinal neovascularization. Also, in a study analysing structural characteristics of macular and peripapillary vasculature in children with PHOMS, Ahn et al. (2022) demonstrated decreased papillary, peripapillary, and ONH vessel densities in children with large (vertical height of ≥500 μm) PHOMS. Therefore, we hypothesized that peripapillary choroidal vasculature would contribute to those observations since ONH has a dual vascular supply (Hayreh, 2001). Our observation of an increase in peripapillary CVI in myopic children with PHOMS compared to those without PHOMS might have presented a compensatory contribution of choroidal vasculature into ONH blood supply compromised by PHOMS. However, current findings cannot determine whether PHOMS affects choroidal vasculature or whether changes in choroidal vasculature occur by ONH tilt, leading to the development of PHOMS.

Despite its potential clinical importance, several challenges exist while evaluating peripapillary choroidal vascular parameters. First of all, there is no standardized measurement location such as subfoveal 1500 μm selected by Agrawal et al. (2016) while describing the macular CVI, which was chosen to represent the macula due to segmental blood supply properties of the choroid itself. However, even for macular CVI, further exploratory studies have used different modifications of the proposed methodology, including more extensive lengths, different orientations and even diverse three‐dimensional areas (Agrawal et al., 2017; Goud et al., 2019; Singh et al., 2018).

To the best of our knowledge, the first study evaluating peripapillary CVI is the study of Park et al. (2018) on glaucoma. This study used a 3.5‐mm‐sized ONH radial circle scan of the Spectralis Glaucoma Module Premium Edition software and its sectors as the location and extent for peripapillary CVI measurement. Many subsequent studies also adopted a similar 360‐degree radial circle scan methodology (Eski et al., 2023; Ozcelik Kose et al., 2022; Pieklarz et al., 2023; Qi et al., 2024; Qiao et al., 2023; Suh et al., 2019). However, one of the limitations of taking 360° peripapillary B‐scans is including the large‐sized retinal vasculature in the scans, leading to back shadowing on the choroid (Park et al., 2018; Pieklarz et al., 2023; Suh et al., 2019). This will result in potential incorrect measurements of both the TCA and LA and, thereby, CVI, especially in evaluating myopic patients, where choroidal thickness is expected to be affected by axial length (Agrawal et al., 2016; Jin et al., 2016). Therefore, in our study, we adopted vertical and horizontal single‐line EDI‐OCT B‐scans centred on the optic disc, as also previously described (Garlı et al., 2022; Guduru et al., 2019, 2020; Simsek et al., 2021; Singh et al., 2019). Also, to be less affected by the choroidal segmental vascular supply and with a hypothesis of more influence on the vascular supply of ONH, we evaluated choroidal vascularity parameters in a length of 1500 μm adjacent to ONH.

Macular CVI is generally considered a more stable choroidal parameter as being less affected by physiological parameters such as refractive status, axial length, age and intraocular pressure (Agrawal et al., 2016). However, later studies have shown that the macular TCA decreases in myopic eyes (Alshareef et al., 2017; Gupta et al., 2017; Kirik et al., 2024; Li et al., 2020; Yazdani et al., 2021). This decrease is mainly attributed to a greater decrease in the macular SA than LA, consequently leading to an increased macular CVI (Alshareef et al., 2017; Gupta et al., 2017; Kirik et al., 2024; Li et al., 2020; Yazdani et al., 2021). Although macular choroidal vascular changes were beyond our scope, peripapillary choroidal structural parameters differences in myopic eyes evaluated in our study seem compatible with the literature. However, more recent studies reported a lower peripapillary CVI with increasing myopia, opposing the available macular CVI literature (Qi et al., 2024; Qiao et al., 2023). In the prospective study of Qi et al. (2024) consisting of 5864 school children aged 6–9, a CVI decrease was observed from hyperopia to myopia in the macular region. Similar differences were also observed in peripapillary CVI (Qi et al., 2024). Yet, two main points should be taken into account while considering these study results. First, Qi et al. assessed CVI by an automated algorithm with a deep learning approach, resulting in CVI values of approximately 30%s, unlike the literature (which is broadly 50%–70%), and the LA seems to be underestimated with the inspection of the figures given by the authors. However, since there is no head‐to‐head study to compare, this interpretation cannot go beyond speculation. Second, the peripapillary choroid was evaluated in a 360‐degree circumpapillary manner, with its inherent risk of retinal vascular interferences on the calculation of corresponding choroidal areas (Qi et al., 2024). That might have affected the observed CVI values, as previously indicated (Park et al., 2018; Pieklarz et al., 2023; Suh et al., 2019). In the study of Qiao et al. (2023) evaluating peripapillary choroidal structure and vasculature in myopic anisometropia patients, the mean peripapillary CVI and CVI of the inferior peripapillary quadrant of more myopic eyes (with a mean of −5.88 D) were found significantly lower than less myopic eyes (with a mean of −4.38 D). Also, the CVI of the other peripapillary quadrants was consistently higher in less myopic eyes without statistical significance. However, the methodology of peripapillary CVI evaluation was again circumpapillary, making the direct comparison of our results inconvenient, apart from the inherent limitations of the methodology.

Certain limitations should be considered while interpreting the results of our study. First, the sample size is relatively small, potentially limiting the statistical power and the generalizability of the findings to a larger population. However, a recently published population‐based cohort study reported an 8.9% prevalence of PHOMS in children, regardless of the associated cause, suggesting that PHOMS is a relatively rare ocular condition in the paediatric population. Additionally, this study specifically focused on myopia‐associated PHOMS, excluding other potential causes, which further limited the number of eyes with PHOMS. The second major limitation of this study is its design. Due to the cross‐sectional nature of this study, caution should be exercised when establishing causality based on the presented findings. Although significant choroidal changes were observed in the myopia‐associated PHOMS (+) group compared to both the emmetropic control and PHOMS (−) groups, a conclusive determination regarding whether these changes are a cause or a consequence of PHOMS cannot be reached. In this cross‐sectional study with a limited sample size, patients were selected consecutively to minimize selection bias, with a particular focus on excluding non‐myopic PHOMS aetiologies. Additionally, only cases where two independent ophthalmologists agreed on the presence or absence of PHOMS were included. The strict criteria we followed to prevent selection bias were also detailed in the flowchart. Third, the location and size of the PHOMS lesions were not evaluated, retaining us to comment on quadrant associations of the study findings. Fourth, since the PHOMS are shown to be toroidal in shape, evaluation of the peripapillary choroidal vascular parameters in a single‐line EDI‐OCT scan would have underestimated the actual spatial effects of the lesion. Nonetheless, the assessment of peripapillary vasculature in single‐line EDI‐OCT scans adjacent to the ONH, considering that it provides direct visualization of the choroidal structure beneath the PHOMS and can better show the impact of choroidal vasculature on the ONH vascular supply, can be regarded as the strength of the study. The fact that 1500 μm width peripapillary choroidal areas were evaluated can be considered as another limitation of this study. However, since PHOMS is a pathology localized in the peripapillary region, we have analysed specified choroidal areas in this study. As we mentioned previously, although there is no standardized protocol for the evaluation of peripapillary choroidal areas, further studies conducted with different analysis protocols (e.g. 1000 μm width) may be valuable in interpreting the presented results.

In conclusion, this first study assessing the peripapillary choroidal vasculature in myopic paediatric patients with PHOMS demonstrated a tendency of lower TCA, LA and SA and higher CVI values in patients with PHOMS than those without PHOMS. Further studies addressing the limitations of this study would expand our understanding of this distinct OCT finding regarding its mechanism of formation, contents and potential implications on myopia progression. Also, further longitudinal studies with larger cohorts will be essential to confirm the presented results, investigate choroidal differences in more detail, and reveal whether choroidal changes are a precursor or a consequence of PHOMS.

Kirik, F. , Dizdar Yiğit, D. , Sevik, M.O. , Ertürk, K.M. , İskandarov, F. , Şahin, Ö. et al. (2025) Peripapillary choroidal vascularity of paediatric myopic eyes with peripapillary hyperreflective ovoid mass‐like structures. Acta Ophthalmologica, 103, e94–e103. Available from: 10.1111/aos.16761

DATA AVAILABILITY STATEMENT

The data that support the findings of this study are available from the corresponding author upon reasonable request.

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Associated Data

This section collects any data citations, data availability statements, or supplementary materials included in this article.

Data Availability Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.

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