Clinical and multimodal imaging characteristics of pigmented paravenous chorioretinal atrophy_Chinese Journal of Ocular Fundus Diseases

Authors：

Feng Sijia , Liu Wei ,  Wu Jing

Department of Ophthalmology, Daping Hospital, Army Medical Center of PLA, Army Medical University, Chongqing 400042, China;

Corresponding?author：

Wu Jing, Email: wu_jing1205@tmmu.edu.cn

Keywords：

Pigmented paravenous chorioretinal atrophy; Multimodal imaging; Fundus autofluorescence; optical coherence tomography; Clinical features

DOI：

10.3760/cma.j.cn511434-20250923-00414

Video：

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Objective To systematically analyze the clinical features, multimodal imaging characteristics, and subtype distribution of pigmented paravenous chorioretinal atrophy (PPCRA). Methods A retrospective case series. From January 2015 to June 2025, 19 patients (34 eyes) diagnosed with PPCRA at Department of Ophthalmology of Daping Hospital, Army Medical Center of PLA of Army Medical University were included in the study. Among them, there were 9 males and 10 females; the age was 52.0 (18, 59) years old. All affected eyes underwent fundus color photography examination; the numbers of eyes that underwent fundus autofluorescence (FAF), optical coherence tomography (OCT), fluorescein fundus angiography (FFA), and indocyanine green angiography (ICGA) were 17, 23, 15, and 10 respectively. Based on the results of fundus color photography and/or FAF examination, PPCRA was classified into perivenous subtype, focal subtype, and confluent subtype. All images were classified by two experienced ophthalmologists in a blinded and independent manner. The Fisher exact test was used to compare the differences in macular involvement rates among different types. Results Among the 34 eyes, 16 eyes (47.1%, 16/34) were perivenous subtype, 13 eyes (38.2%, 13/34) had the confluent subtype, and 5 eyes (14.7%, 5/34) had the focal subtype. The consistency rate of the independent judgments by the two readers was 79.4% (27/34) (κ= 0.653, 95% confidence interval 0.427-0.878, P＜0.001), indicating a substantial consistency. Among the 23 eyes that underwent OCT examination, 12 eyes (52.2%, 12/23) had macular involvement, including 9 eyes (90.0%, 9/10) of the fusion type and 3 eyes (30.0%, 3/10) of the venous side type; no macular involvement was observed in the focal subtype. There was a statistically significant difference in the rate of macular involvement among different types (P=0.003). FAF typically showed paravenous band-like hypoautofluorescence with irregular rim hyperautofluorescence. FFA predominantly demonstrated transmission hyperfluorescence without leakage; venous leakage was noted in both eyes of one patient. ICGA commonly showed persistent paravenous hypofluorescence. OCT revealed attenuation or loss of the ellipsoid zone and interdigitation zone, with outer retinal thinning. In severely atrophic areas, OCT also showed reduced or absent reflectivity of the retinal pigment epithelium, together with choroidal thinning. Conclusions The perivenous subtype was the most common subtype of PPCRA. Macular involvement was associated with the confluent subtype, suggesting a potentially higher risk of macular structural involvement in this phenotype. Multimodal imaging can clearly present lesion features and is valuable for lesion assessment and follow-up.

Citation： Feng Sijia, Liu Wei, Wu Jing. Clinical and multimodal imaging characteristics of pigmented paravenous chorioretinal atrophy. Chinese Journal of Ocular Fundus Diseases, 2026, 42(7): 589-596. doi: 10.3760/cma.j.cn511434-20250923-00414 Copy

1.	Pearlman JT, Kamin DF, Kopelow SM, et al. Pigmented paravenous retinochoroidal atrophy[J]. Am J Ophthalmol, 1975, 80(4): 630-635. DOI: 10.1016/0002-9394(75)90393-1.
2.	Antropoli A, Arrigo A, Pili L, et al. Pigmented paravenous chorioretinal atrophy: updated scenario[J]. Eur J Ophthalmol, 2024, 34(4): 941-951. DOI: 10.1177/11206721231199118.
3.	Shona OA, Islam F, Robson AG, et al. Pigmented paravenous chorioretinal atrophy: detailed clinical study of a large cohort[J]. Retina, 2019, 39(3): 514-529. DOI: 10.1097/IAE.0000000000001950.
4.	Lee EK, Lee SY, Oh BL, et al. Pigmented paravenous chorioretinal atrophy: clinical spectrum and multimodal imaging characteristics[J]. Am J Ophthalmol, 2021, 224: 120-132. DOI: 10.1016/j.ajo.2020.12.010.
5.	Takagi S, Hirami Y, Takahashi M, et al. Use of wide-field fundus camera, fundus autofluorescence, and OCT in cases of pigmented paravenous retinochoroidal atrophy[J]. Ophthalmol Retina, 2018, 2(1): 79-81. DOI: 10.1016/j.oret.2017.05.004.
6.	王建倉, 杜非凡, 田海霞, 等. 色素性靜脈旁視網膜脈絡膜萎縮伴左眼黃斑缺損一例[J]. 中華眼底病雜志, 2019, 35(1): 81-82. DOI: 10.3760/cma.j.issn.1005-1015.2019.01.018.Wang JC, Du FF, Tian HX, et al. Pigmented paravenous chorioretinal atrophy with macular defect in the left eye: a case report[J]. Chin J Ocul Fundus Dis, 2019, 35(1): 81-82. DOI: 10.3760/cma.j.issn.1005-1015.2019.01.018.
7.	潘昕, 于麗, 駱立夫, 等. 色素性靜脈旁視網膜脈絡膜萎縮一例[J]. 中華眼底病雜志, 2020, 36(10): 808-809. DOI: 10.3760/cma.j.cn511434-20190514-00177.Pan X, Yu L, Luo LF, et al. Pigmented paravenous chorioretinal atrophy: a case report[J]. Chin J Ocul Fundus Dis, 2020, 36(10): 808-809. DOI: 10.3760/cma.j.cn511434-20190514-00177.
8.	陳叢, 張文婧, 鄧玉夢, 等. 色素性靜脈旁脈絡膜視網膜萎縮合并缺血性視神經病變[J]. 中華眼底病雜志, 2025, 41(4): 306. DOI: 10.3760/cma.j.cn511434-20241202-00455.Chen C, Zhang WJ, Deng YM, et al. Pigmented paravenous chorioretinal atrophy with ischemic optic neuropathy[J]. Chin J Ocul Fundus Dis, 2025, 41(4): 306. DOI: 10.3760/cma.j.cn511434-20241202-00455.
9.	Landis JR, Koch GG. The measurement of observer agreement for categorical data[J]. Biometrics, 1977, 33(1): 159-174. DOI: 10.2307/2529310.
10.	Choi JY, Sandberg MA, Berson EL. Natural course of ocular function in pigmented paravenous retinochoroidal atrophy[J]. Am J Ophthalmol, 2006, 141(4): 763-765. DOI: 10.1016/j.ajo.2005.11.009.
11.	Choi WJ, Joo K, Park KH. Pigmented paravenous retinochoroidal atrophy[J]. Korean J Ophthalmol, 2020, 34(1): 90-91. DOI: 10.3341/kjo.2019.0078.
12.	Hashimoto Y, Kase S, Saito W, et al. Abnormalities of fundus autofluorescence in pigmented paravenous chorioretinal atrophy[J]. Open Ophthalmol J, 2012, 6: 125-128. DOI: 10.2174/1874364101206010125.
13.	Barteselli G. Fundus autofluorescence and optical coherence tomography findings in pigmented paravenous retinochoroidal atrophy[J/OL]. Can J Ophthalmol, 2014, 49(6): e144-e146[2014-12-01]. https://pubmed.ncbi.nlm.nih.gov/25433750/. DOI: 10.1016/j.jcjo.2014.08.019.
14.	Yanagi Y, Okajima O, Mori M. Indocyanine green angiography in pigmented paravenous retinochoroidal atrophy[J]. Acta Ophthalmol Scand, 2003, 81(1): 60-67. DOI: 10.1034/j.1600-0420.2003.00003.x.
15.	Noble KG, Carr RE. Pigmented paravenous chorioretinal atrophy[J]. Am J Ophthalmol, 1983, 96(3): 338-344. DOI: 10.1016/S0002-9394(14)77825-0.
16.	Yamaguchi K, Hara S, Tanifuji Y, et al. Inflammatory pigmented paravenous retinochoroidal atrophy[J]. Br J Ophthalmol, 1989, 73(6): 463-467. DOI: 10.1136/bjo.73.6.463.
17.	Batioglu F, Atmaca L S, Atilla H, et al. Inflammatory pigmented paravenous retinochoroidal atrophy[J]. Eye (Lond), 2002, 16(1): 81-84. DOI: 10.1038/sj.eye.6700021.

1. Pearlman JT, Kamin DF, Kopelow SM, et al. Pigmented paravenous retinochoroidal atrophy[J]. Am J Ophthalmol, 1975, 80(4): 630-635. DOI: 10.1016/0002-9394(75)90393-1.
2. Antropoli A, Arrigo A, Pili L, et al. Pigmented paravenous chorioretinal atrophy: updated scenario[J]. Eur J Ophthalmol, 2024, 34(4): 941-951. DOI: 10.1177/11206721231199118.
3. Shona OA, Islam F, Robson AG, et al. Pigmented paravenous chorioretinal atrophy: detailed clinical study of a large cohort[J]. Retina, 2019, 39(3): 514-529. DOI: 10.1097/IAE.0000000000001950.
4. Lee EK, Lee SY, Oh BL, et al. Pigmented paravenous chorioretinal atrophy: clinical spectrum and multimodal imaging characteristics[J]. Am J Ophthalmol, 2021, 224: 120-132. DOI: 10.1016/j.ajo.2020.12.010.
5. Takagi S, Hirami Y, Takahashi M, et al. Use of wide-field fundus camera, fundus autofluorescence, and OCT in cases of pigmented paravenous retinochoroidal atrophy[J]. Ophthalmol Retina, 2018, 2(1): 79-81. DOI: 10.1016/j.oret.2017.05.004.
6. 王建倉, 杜非凡, 田海霞, 等. 色素性靜脈旁視網膜脈絡膜萎縮伴左眼黃斑缺損一例[J]. 中華眼底病雜志, 2019, 35(1): 81-82. DOI: 10.3760/cma.j.issn.1005-1015.2019.01.018.Wang JC, Du FF, Tian HX, et al. Pigmented paravenous chorioretinal atrophy with macular defect in the left eye: a case report[J]. Chin J Ocul Fundus Dis, 2019, 35(1): 81-82. DOI: 10.3760/cma.j.issn.1005-1015.2019.01.018.
7. 潘昕, 于麗, 駱立夫, 等. 色素性靜脈旁視網膜脈絡膜萎縮一例[J]. 中華眼底病雜志, 2020, 36(10): 808-809. DOI: 10.3760/cma.j.cn511434-20190514-00177.Pan X, Yu L, Luo LF, et al. Pigmented paravenous chorioretinal atrophy: a case report[J]. Chin J Ocul Fundus Dis, 2020, 36(10): 808-809. DOI: 10.3760/cma.j.cn511434-20190514-00177.
8. 陳叢, 張文婧, 鄧玉夢, 等. 色素性靜脈旁脈絡膜視網膜萎縮合并缺血性視神經病變[J]. 中華眼底病雜志, 2025, 41(4): 306. DOI: 10.3760/cma.j.cn511434-20241202-00455.Chen C, Zhang WJ, Deng YM, et al. Pigmented paravenous chorioretinal atrophy with ischemic optic neuropathy[J]. Chin J Ocul Fundus Dis, 2025, 41(4): 306. DOI: 10.3760/cma.j.cn511434-20241202-00455.
9. Landis JR, Koch GG. The measurement of observer agreement for categorical data[J]. Biometrics, 1977, 33(1): 159-174. DOI: 10.2307/2529310.
10. Choi JY, Sandberg MA, Berson EL. Natural course of ocular function in pigmented paravenous retinochoroidal atrophy[J]. Am J Ophthalmol, 2006, 141(4): 763-765. DOI: 10.1016/j.ajo.2005.11.009.
11. Choi WJ, Joo K, Park KH. Pigmented paravenous retinochoroidal atrophy[J]. Korean J Ophthalmol, 2020, 34(1): 90-91. DOI: 10.3341/kjo.2019.0078.
12. Hashimoto Y, Kase S, Saito W, et al. Abnormalities of fundus autofluorescence in pigmented paravenous chorioretinal atrophy[J]. Open Ophthalmol J, 2012, 6: 125-128. DOI: 10.2174/1874364101206010125.
13. Barteselli G. Fundus autofluorescence and optical coherence tomography findings in pigmented paravenous retinochoroidal atrophy[J/OL]. Can J Ophthalmol, 2014, 49(6): e144-e146[2014-12-01]. https://pubmed.ncbi.nlm.nih.gov/25433750/. DOI: 10.1016/j.jcjo.2014.08.019.
14. Yanagi Y, Okajima O, Mori M. Indocyanine green angiography in pigmented paravenous retinochoroidal atrophy[J]. Acta Ophthalmol Scand, 2003, 81(1): 60-67. DOI: 10.1034/j.1600-0420.2003.00003.x.
15. Noble KG, Carr RE. Pigmented paravenous chorioretinal atrophy[J]. Am J Ophthalmol, 1983, 96(3): 338-344. DOI: 10.1016/S0002-9394(14)77825-0.
16. Yamaguchi K, Hara S, Tanifuji Y, et al. Inflammatory pigmented paravenous retinochoroidal atrophy[J]. Br J Ophthalmol, 1989, 73(6): 463-467. DOI: 10.1136/bjo.73.6.463.
17. Batioglu F, Atmaca L S, Atilla H, et al. Inflammatory pigmented paravenous retinochoroidal atrophy[J]. Eye (Lond), 2002, 16(1): 81-84. DOI: 10.1038/sj.eye.6700021.

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Clinical and multimodal imaging characteristics of pigmented paravenous chorioretinal atrophy

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