Jump to comment:

• Pachychoroid and vitelliform deposits
  Salomon Y Cohen, Alain Gaudric and Sarah Mrejen
  Published on: 21 May 2024
• Rapid response to "Pachyvitelliform maculopathy: optical coherence tomography analysis of a new entity"
  Beatrice Tombolini
  Published on: 21 May 2024

• Published on: 21 May 2024

  Pachychoroid and vitelliform deposits

  • Salomon Y Cohen, Ophthalmologist Centre Ophtalmologique d'Imagerie et de Laser, Paris, France
  • Other Contributors:
    • Alain Gaudric, Ophthalmologist
    • Sarah Mrejen, Ophthalmologist

  Dear Editor, we read with interest the study of Hilely and coworkers, describing a new condition which they referred to as pachyvitelliform maculopathy. Their study clearly demonstrates the association between pachychoroid features and adult vitelliform lesions (AVLs).1 Two previous reports by our teams may stregthen their hypothesis of a relationship between the two conditions. In cuticular drusen, we have shown that vitelliform detachments occur significantly more often in eyes with a thick choroid, and we suggested that the choroidal vasculature could play a role in the occurrence of macular detachments in patients with cuticular drusen, leading to accumulation of photreceptors debris and deposits.2 More recently, we report a series of 49 eyes with hyperreflective vertical lines diagnosed on B-scans of spectral-domain optical coherence tomography. Twenty-four eyes presented with AVLs related to adult vitelliform dystrophy or pattern dystrophy. We were surprised that, in 14 cases out of 24, signs of pachychoroid were present. We suggested that further studies were needed to better understand this relationship.3 So we fully agree in that the pachychoroid syndrome may interfere with the metabolism of the retinal pigment epithelium and the phagocytosis process, leading to accumulation of photoreceptors debris. We really thank Hilely and coworkers for their convincing step towards a better recognition of this curious association.

  Salomon Y Cohen, A Gaudric, Sarah Mrej...

  Show More

  Dear Editor, we read with interest the study of Hilely and coworkers, describing a new condition which they referred to as pachyvitelliform maculopathy. Their study clearly demonstrates the association between pachychoroid features and adult vitelliform lesions (AVLs).1 Two previous reports by our teams may stregthen their hypothesis of a relationship between the two conditions. In cuticular drusen, we have shown that vitelliform detachments occur significantly more often in eyes with a thick choroid, and we suggested that the choroidal vasculature could play a role in the occurrence of macular detachments in patients with cuticular drusen, leading to accumulation of photreceptors debris and deposits.2 More recently, we report a series of 49 eyes with hyperreflective vertical lines diagnosed on B-scans of spectral-domain optical coherence tomography. Twenty-four eyes presented with AVLs related to adult vitelliform dystrophy or pattern dystrophy. We were surprised that, in 14 cases out of 24, signs of pachychoroid were present. We suggested that further studies were needed to better understand this relationship.3 So we fully agree in that the pachychoroid syndrome may interfere with the metabolism of the retinal pigment epithelium and the phagocytosis process, leading to accumulation of photoreceptors debris. We really thank Hilely and coworkers for their convincing step towards a better recognition of this curious association.

  Salomon Y Cohen, A Gaudric, Sarah Mrejen
  Centre Ophtalmologique d’Imagerie et de Laser, Paris, France

  References
  1. Hilely A, Au A, Lee WK, Fogel Levin M, et al. Pachyvitelliform maculopathy: an optical coherence tomography analysis of a novel entity. Br J Ophthalmol. 2023 Jul 14:bjo-2022-322553.
  2. Mrejen-Uretsky S, Ayrault S, Nghiem-Buffet S, et al. Choroidal thickening in patients with cuticular drusen combined with vitelliform macular detachment. Retina. 2016;36(6):1111-8.
  3. Amoroso F, Mrejen S, Pedinielli A, et al. Intraretinal hyperreflective lines. Retina. 2021;41(1):82-92.

  Show Less

  Conflict of Interest:
  None declared.

  • Back to top
• Published on: 21 May 2024

  Rapid response to "Pachyvitelliform maculopathy: optical coherence tomography analysis of a new entity"

  • Beatrice Tombolini, Opthalmology Resident Vita-Salute San Raffaele University, Milan (Italy)

  We read with considerable attention the manuscript by Hilely et al. entitled “Pachyvitelliform maculopathy: an optical coherence tomography analysis of a novel entity.”1 We would like to compliment the Authors on their great effort and excellent figures.
  Hilely and associated introduced a novel clinical entity named pachyvitelliform maculopathy (PVM). It consisted of the association between acquired vitelliform lesion (AVL) and pachychoroid disease spectrum (PDS) features, including pachychoroid (choroidal thickness > 250 μm), pachyvessels (dilated Haller vessels), and inner choroid thinning. In detail, a yellowish hyperreflective lesion overlayed a thick choroid and pachyvessels. In contrast to other forms PDS,2 PVM displayed no subretinal (SRF) or intraretinal fluid (IRF) and neovascularization. Furthermore, cases of pachychoroid and drusen (medium, large, cuticular, pseudo reticular, and subretinal drusenoid deposits) were excluded. This condition was described in 60 years older subjects with electro-oculogram within normal range and with a negative genetic test, although performed in a minority of the cohort.
  We would like to have the thoughts about the following issue that Hilely and colleagues ought to address. Although the Authors stated that this was the first description of PVM, previous papers discussed the aforementioned association between PDS and vitelliform lesions. In detail, Spaide described three cases (two monoliteral, and one bilateral) o...

  Show More

  We read with considerable attention the manuscript by Hilely et al. entitled “Pachyvitelliform maculopathy: an optical coherence tomography analysis of a novel entity.”1 We would like to compliment the Authors on their great effort and excellent figures.
  Hilely and associated introduced a novel clinical entity named pachyvitelliform maculopathy (PVM). It consisted of the association between acquired vitelliform lesion (AVL) and pachychoroid disease spectrum (PDS) features, including pachychoroid (choroidal thickness > 250 μm), pachyvessels (dilated Haller vessels), and inner choroid thinning. In detail, a yellowish hyperreflective lesion overlayed a thick choroid and pachyvessels. In contrast to other forms PDS,2 PVM displayed no subretinal (SRF) or intraretinal fluid (IRF) and neovascularization. Furthermore, cases of pachychoroid and drusen (medium, large, cuticular, pseudo reticular, and subretinal drusenoid deposits) were excluded. This condition was described in 60 years older subjects with electro-oculogram within normal range and with a negative genetic test, although performed in a minority of the cohort.
  We would like to have the thoughts about the following issue that Hilely and colleagues ought to address. Although the Authors stated that this was the first description of PVM, previous papers discussed the aforementioned association between PDS and vitelliform lesions. In detail, Spaide described three cases (two monoliteral, and one bilateral) of central serous chorioretinopathy (before the introduction of PDS definition) that slowly (range: 6 months - 5.5 years) developed sub-retinal yellowish material similar to that seen in adult-onset foveal vitelliform dystrophy (AOFVD) in absence of exudation.3 Furthermore, also Hilely et al. described this lesion appeared hyperfluorescent on fundus autofluorescence, suggesting that its content was made of lipofuscin-like AOFVD.4 Spaide hypothesized that the development of the yellowish deposit in CSC eyes and AOFVD occurred after resorption of the subretinal fluid as a late step of a process not completely understood. However, this study lacked optical coherence tomography (OCT) analysis, so it’s not possible to exclude with certainty that these cases did not present any signs of SRF, as declared by Spaide et al.3 Freund and colleagues then examined the multimodal imaging appearance of AVL, including structural OCT. The group reported two cases of CSC (again before the introduction of the PDS definition) and AVL, with one of them showing no SRF. Interestingly, no early or unilateral AVL showed SRF also in the fellow eye, indicating that the formation of the vitelliform lesion comes first before the occurrence of SRF.5 Subsequently, Soman et al.6 described as first a case of bilateral AVL and pachychoroid, that in one eye was complicated by macular neovascularization (MNV). A noteworthy aspect of this case of pachychoroid neovasculopathy was the initial description of bilateral vitelliform lesions without concomitant retinal fluid, followed in the left eye by the progression from a nonexudative to an exudative MNV combined with reabsorption, and subsequent deposition of AVL in a different location.6 Recently, the association between AVL and a form of PDS (i.e. peripapillary pachychoroid syndrome) was reported. However, all these cases showed IRF, and in a minority of cases also SRF, as these are typical features of this condition. 7
  Given all this evidence, it would be difficult to conclude that the Authors reported a new clinical entity. The association between different forms of PDS and AVL was yet reported, even if in sporadic series. The Authors had the merit of having better characterized this peculiar form of PDS associated with AVL in the new classifications of PDS subtypes. However, it cannot be excluded with certainty that the Authors reported cases of CSC after the reabsorption of leakage fluid, similar to the aforementioned previous evidence. We are interested in the Authors' thoughts on the matter.

  References
  1. Hilely A, Au A, Lee WK, et al. Pachyvitelliform maculopathy : an optical coherence tomography analysis of a novel entity. Br J Ophthalmol. 2023;0:1-7. doi:10.1136/bjo-2022-322553
  2. Van Dijk EHC, Boon CJF. Serous business: Delineating the broad spectrum of diseases with subretinal fluid in the macula. Prog Retin Eye Res. 2021;84:100955. doi:10.1016/j.preteyeres.2021.100955
  3. Spaide RF. Deposition of yellow submacular material in central serous chorioretinopathy resembling adult-onset foveomacular vitelliform dystrophy. Retina. 2004;24(2):301-304. doi:10.1097/00006982-200404000-00019
  4. Spaide RF, Noble K, Morgan A, Freund KB. Vitelliform Macular Dystrophy. 2006;2. doi:10.1016/j.ophtha.2006.03.023
  5. Freund KB, Laud K, Lima LH, Spaide RF, Zweifel S, Yannuzzi LA. Acquired Vitelliform Lesions. Retina. 2011;31(1):13-25. doi:10.1097/iae.0b013e3181ea48ba
  6. Soman M, Iqbal S, Sheth JU, Meleth P, Nair U. Progression of Subclinical Pachychoroid Neovasculopathy to an Active Neovascularization in the Presence of Acquired Vitelliform Lesions. Case Rep Ophthalmol Med. 2021;2021(c):1-8. doi:10.1155/2021/3098420
  7. Barequet D, Iglicki M, Meshi A, Loewenstein A, Goldstein M, Zur D. ACQUIRED VITELLIFORM LESIONS: A Novel Finding in Eyes with Peripapillary Pachychoroid Syndrome. Retina. 2022;42(5):949-956. doi:10.1097/IAE.0000000000003403

  Show Less

  Conflict of Interest:
  None declared.

  • Back to top