Clinical characteristics of pseudopapilledema combined with peripapillary hyper-reflective ovoid mass-like structures in children_Chinese Journal of Ocular Fundus Diseases

Authors：

Lin Xuemei ¹ , Liu Pei ¹ , Wang Jing ¹ , Chang Qiaoqiao ¹ , Lu Huiqin ² , Zhao Jun ² ,  Wu Songdi ¹

1. Department of Neuro-ophthalmology, The First Hospital of Xi’an, Affiliated of The First Hospital of Northwest University, Xi’an 710002, China;
2. Department of Ophthalmology, The First Hospital of Xi’an, Affiliated of The First Hospital of Northwest University, Xi’an 710002, China;

Corresponding author：

Wu Songdi, Email: wusongdi@gmail.com

Keywords：

Pseudopapilledema; Peripapillary hyper-reflective ovoid mass-like structures; Optic disc drusen; Tomography, optical coherence

DOI：

10.3760/cma.j.cn511434-20211108-00636

Video：

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Objective To observe the clinical characteristics and optical coherence tomography (OCT) features of pseudopapilledema (PPE) combined with peripapillary hyper-reflective ovoid mass-like structures (PHOMS) in children. Methods A retrospective observational study. From October 2019 to May 2021, total 22 eyes from 12 children diagnosed as PPE combined with PHOMS in the Neuro-ophthalmology Department of The First Hospital of Xi’an (Affiliated of The First Hospital of Northwest University) were recruited. Among the children, 6 were male and 6 were female. The average age was (10.6±2.7) years. The average course from disease onset to diagnosis of PPE combined with PHOMS was (8.0±7.5) months. All patients underwent best corrected visual acuity (BCVA), relative afferent papillary defect (RAPD), Ishihara's test, fundus photography, OCT, fundus autofluorescence (FAF), ocular B-mode ultrasound, visual field and patternvisual evoked potential (P-VEP). The clinical and OCT characteristics of the patients were observed. Results The anterior segments of the patients were normal. The intraocular pressures and Ishihara's test were all normal. All RAPD were negative. Total 22 eyes, BCVA was 1.0 in 21 eyes and one eye was 0.12. The fundus photography revealed blurred optic discs margin, showing mild to moderate edema-like elevation with more prominent in the nasal parts, presenting as a “C” shape halo. No obvious abnormal fluorescence was observed in FAF. The OCT scan of involvement eyes showed an elevated appearance in vary degrees, and the sharply marginated ovoid hyper-reflective mass-like structures which laterally herniated into the peripapillary region under retinal nerve fiber layer and above the Bruch membrane were detected with consecutive nasal enlargement scanning, corresponding to the nasal parts in the fundus photography. The higher degree of elevation, the larger the volume. Macular retina pigment epithelium layer and ganglion cell thickness were normal. Ocular B-mode ultrasound showed that the head of the optic nerve in the posterior wall of the eyeball (in front of the optic disc) was elevated in all affected eyes, and there was no strong signal echo in it. Visual field examination showed physical blind spot enlargement in 3 eyes and visual field defect in 2 eyes. P-VEP examination showed that the peak was slightly delayed in 3 eyes and the amplitude was slightly reduced in 3 eyes. Conclusions Enlarged nasal optic disc OCT scan can improve the detection rate of PHOMS. PHOMS were detected bilaterally in the cases with binocular PPE while only in the effected eye in the cases of monocular PPE; the higher degree of PPE, the lager volume of PHOMS. PHOMS were could contribute to the diagnosis of PPE in children.

Citation： Lin Xuemei, Liu Pei, Wang Jing, Chang Qiaoqiao, Lu Huiqin, Zhao Jun, Wu Songdi. Clinical characteristics of pseudopapilledema combined with peripapillary hyper-reflective ovoid mass-like structures in children. Chinese Journal of Ocular Fundus Diseases, 2023, 39(7): 569-575. doi: 10.3760/cma.j.cn511434-20211108-00636 Copy

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1. 田国红, 陈倩视盘水肿的鉴别及病因分析[J]. 中国眼耳鼻喉科杂志2017175378381. DOI: 10.14166/j.issn.1671-2420.2017.05.020..Tian GH, Chen QDiscrimination and etiology analysis of optic disc edema[J]. Chin J Ophthalmol and Otorhinolaryngol2017175378381. DOI: 10.14166/j.issn.1671-2420.2017.05.020.
2. Fraser JA, Sibony PA, Petzold A, et alPeripapillary hyper-reflective ovoid mass-like structure (PHOMS): an optical coherence tomography marker of axoplasmic stasis in the optic nerve head[J]. J Neuroophthalmol2021414431441. DOI: 10.1097/WNO.0000000000001203.
3. Mezad-Koursh D, Klein A, Rosenblatt A, et alPeripapillary hyperreflective ovoid mass-like structures—a novel entity as frequent cause of pseudopapilloedema in children[J]. Eye202035412281234. DOI: 10.1038/s41433-020-1067-x.
4. Freund P, Margolin E. Pseudopapilledema[M]. Treasure Island (FL): StatPearls Publishing, 2021.
5. Malmqvist L, Bursztyn L, Costello F, et alThe optic disc drusen studies consortium recommendations for diagnosis of optic disc drusen using optical coherence tomography[J]. J Neuroophthalmol2018383299307. DOI: 10.1097/WNO.0000000000000585.
6. Frisén LSwelling of the optic nerve head: a staging scheme[J]. J Neurol Neurosurg Psychiatry19824511318. DOI: 10.1136/jnnp.45.1.13.
7. Chang MY, Velez FG, Demer JL, et alAccuracy of diagnostic imaging modalities for classifying pediatric eyes as papilledema versus pseudopapilledema[J]. Ophthalmology20171241218391848. DOI: 10.1016/j.ophtha.2017.06.016.
8. Kovarik JJ, Doshi PN, Collinge JE, et alOutcome of pediatric patients referred for papilledema[J]. J AAPOS2015194344348. DOI: 10.1016/j.jaapos.2015.05.007.
9. Rebolleda G, Kawasaki A, de Juan V, et alOptical coherence tomography to differentiate papilledema from pseudopapilledema[J]. Curr Neurol Neurosci Rep2017171074. DOI: 10.1007/s11910-017-0790-6.
10. Wang DD, Leong JCY, Gale J, Wells APMultimodal imaging of buried optic nerve head drusen[J]. Eye (Lond)201832611451146. DOI: 10.1038/s41433-017-0009-8.
11. Pichi F, Romano S, Villani E, et alSpectral-domain optical coherence tomography findings in pediatric tilted disc syndrome[J]. Graefe's Arch Clin Exp Ophthalmol20142521016611667. DOI: 10.1007/s00417-014-2701-8.
12. Hamann S, Malmqvist L, Wegener M, et alYoung adults with anterior ischemic optic neuropathy: a multicenter optic disc drusen study[J]. Am J Ophthalmol2020217174181. DOI: 10.1016/j.ajo.2020.03.052.
13. Malmqvist L, Sibony PA, Fraser CL, et alPeripapillary ovoid hyperreflectivity in optic disc edema and pseudopapilledema[J]. Ophthalmology20181251016621664. DOI: 10.1016/j.ophtha.2018.04.036.
14. Petzold A, Coric D, Balk LJ, et alLongitudinal development of peripapillary hyper-reflective ovoid masslike structures suggests a novel pathological pathway in multiple sclerosis[J]. Ann Neurol2020882309319. DOI: 10.1002/ana.25782.
15. Ahn YJ, Park YY, Shin SYPeripapillary hyperreflective ovoid mass-like structures (PHOMS) in children[J]. Eye (Lond)2022363533539. DOI: 10.1038/s41433-021-01461-w.
16. Lyu IJ, Park KA, Oh SYAssociation between myopia and peripapillary hyperreflective ovoid mass-like structures in children[J]. Sci Rep20201012238. DOI: 10.1038/s41598-020-58829-3.
17. Lee KM, Woo SJ, Hwang JMMorphologic characteristics of optic nerve head drusen on spectral-domain optical coherence tomography[J]. Am J Ophthalmol2013155611391147. DOI: 10.1016/j.ajo.2013.01.024.
18. 谭耀, 高玲视盘玻璃疣的研究现状及进展[J]. 中华眼底病杂志2020368648652. DOI: 10.3760/cma.j.cn511434-20181204-00414..Tan Y, Gao LOptic disc drusen: a review[J]. Chin J Ocul Fundus Dis2020368648652. DOI: 10.3760/cma.j.cn511434-20181204-00414.
19. Rotruck JA review of optic disc drusen in children[J]. Int Ophthalmol Clin20185846782. DOI: 10.1097/iio.0000000000000236.

Chinese Journal of Ocular Fundus Diseases

Clinical characteristics of pseudopapilledema combined with peripapillary hyper-reflective ovoid mass-like structures in children

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