CURRENT VIEW OF THE SPECTRUM OF PACHYCHOROID DISEASES. A REVIEW

Česká verze

Authors: A. Stepanov
Authors place of work: Oční klinika Fakultní nemocnice v Hradci Králové a Lékařské, fakulty Univerzity Karlovy v Hradci Králové, Hradec Králové
Published in the journal: Čes. a slov. Oftal., 79, 2023, No. 4, p. 163-166
Category: Přehledový článek
doi: https://doi.org/10.31348/2023/9

Summary

Introduction: The term "pachychoroid" (greek pachy- [παχύ] - thick) was first used by Warrow et al. in 2013. It is defined as an abnormal and permanent increase in choroidal thickness ≥ 300 μm, which is caused by dilatation of the choroidal vessels of the Haller's layer, thinning of the Sattler's layer and the choriocapillaris layer.

Methodology: Literary research focused on the current view of pachychoroid spectrum diseases, including clarification of the pathophysiological theories of the formation of "pachychoroid".

Results: It is assumed that “pachychoroid” disease has an autosomal dominant type of heredity. Depending on the further activity of various exogenous and/or endogenous factors, pachychoroid diseases may appear. According to the current knowledge, the spectrum of pachychoroid disease covers six clinical entities: pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoid choroidal vasculopathy, focal choroidal excavation and peripapillary pachychoroid syndrome. In this study, we describe the clinical symptoms and objective findings of focal choroidal excavation and peripapillary pachychoroid syndrome. The current pathophysiological theory of pachychoroid diseases is based on impaired venous outflow from the choroid ("venous overload choroidopathy") and thickening of the sclera in the eyes of affected patients.

Conclusion: Pachychoroid diseases should be included in the differential diagnosis of characteristic features observed during multimodal imaging analysis of choroidal changes.

Keywords:

pachychoroid – focal choroidal excavation – peripapillary pachychoroid syndrome – venae vorticosae – choroidopathy of venous congestion

Zdroje

1. Warrow DJ, Hoang QV, Freund KB. Pachychoroid pigment epitheliopathy. Retina. 2013;33(8):1659-1672.

2. Cheung CMG, Lee WK, Koizumi H, Dansingani K, Lai TYY, Freund KB. Pachychoroid disease. Eye (Lond). 2019;33(1):14-33.

3. Borooah S, Sim PY, Phatak S, et al. Pachychoroid spectrum disease. Acta Ophthalmol. 2021;99(6):e806-e822.

4. Akkaya S. Spectrum of pachychoroid disease. Int Ophthalmol. 2018;38(5):2239-2246.

5. Ayachit G, Ayachit A, Nadgir H, Joshi S. Validating the pachychoroid disease spektrum using multimodal imaging. Indian J Ophthalmol. 2018;66(7):1022-1024.

6. Stěpanov A, Studnicka J, Jirásková N. Pachychoroid Disease of the Macula – Case Report. Cesk Slov Oftalmol. 2018;74(5):208- 211.

7. Stěpanov A, Studnicka J, Středová M, Jirásková N. Pachychoroid disease of the macula. Cesk Slov Oftalmol. 2018;74(1):3-8.

8. Jampol LM, Shankle J, Schroeder R, et al. Diagnostic and therapeutic challenges. Retina 2006;26:1072-1076.

9. Verma S, Kumar V, Azad S, et al. Focal choroidal excavation: review of literature. Br J Ophthalmol. 2021;105(8):1043-1048.

10. Shinojima A, Kawamura A, Mori R, et al. Morphologic features of focal choroidal excavation on spectral domain optical coherence tomography with simultaneous angiography. Retina Phila Pa. 2014;34:1407-1414.

11. Margolis R. The expanded spectrum of focal choroidal excavation. Arch Ophthalmol 2011;129:1320.

12. Lee CS, Woo SJ, Kim Y-K, et al. Clinical and spectral-domain optical coherence tomography findings in patients with focal choroidal excavation. Ophthalmology 2014;121:1029-35.

13. Chung H, Byeon SH, Freund KB. Focal choroidal excavation and its association with pachychoroid spectrum disorders: a review of the literature and multimodal imaging findings. Retina 2017;37:199- 221.

14. Obata R, Takahashi H, Ueta T, et al. Tomographic and angiographic characteristics of eyes with macular focal choroidal excavation. Retina Phila Pa. 2013;33:1201-1210.

15. Chung CY, Li SH, Li KKW. Focal choroidal excavation-morphological features and clinical correlation. Eye Lond Engl. 2017;31:1373-1379.

16. Phasukkijwatana N, Freund KB, Dolz-Marco R, et al. Peripapillary pachychoroid syndrome. Retina. 2018;38(9):1652-1667.

17. Spaide RF, Ledesma-Gil G. Choriocapillaris vascular parameters in normal eyes and those with pachychoroid and without disease. Retina. 2021;41(4):679-685.

18. Hogan MJ, Alvarado JA, Weddell JE. Histology of the Human Eye. An Atlas and Textbook. 1971.

19. Castro-Navarro V, Behar-Cohen F, Chang W, et al. Pachychoroid: current concepts on clinical features and pathogenesis. Graefe’s Archive for Clinical and Experimental Ophthalmology, 2021;259(6):1385-1400.

20. Oyster CW. The Human Eye: Structure and Function. 1999, pp. 766.

21. Matsumoto H, Hoshino J, Mukai R, et al. Chronic choriocapillaris ischemia in dilated vortex vein region in pachychoroid neovasculopathy. Sci Rep. 2021;11(1):16274.

22. Imanaga N, Terao N, Nakamine S, et al. Scleral Thickness in Central Serous Chorioretinopathy. Ophthalmol Retina. 2021;5(3):285-291.

23. Gal-Or O, Dansingani KK, Sebrow D, et al. Inner choroidal flow signal attenuation in pachychoroid disease: Optical Coherence Tomography Angiography. Retina. 2018;38(10):1984-1992.

24. Lee M, Lee H, Kim HC, Chung H. Changes in Stromal and Luminal Areas of the Choroid in Pachychoroid Diseases: Insights Into the Pathophysiology of Pachychoroid Diseases. Invest Ophthalmol Vis Sci. 2018;59(12):4896-4908.

25. Saito M, Saito W, Hashimoto Y, Yoshizawa C, Fujiya A, Noda K, Ishida S. Macular choroidal blood flow velocity decreases with regression of acute central serous chorioretinopathy. Br J Ophthalmol. 2013;97(6):775-780.