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Multimodal imaging of sclerochoroidal calcification associated with choroidal neovascular membrane

Published:November 26, 2020DOI:https://doi.org/10.1016/j.jcjo.2020.11.007
      Sclerochoroidal calcification is a benign condition typically diagnosed in asymptomatic, older, white adults, which can simulate other more serious conditions such as choroidal melanoma, metastasis, or lymphoma.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      It is characterized as yellow or yellow-white single or multifocal lesions typically found in the superotemporal quadrant near the vascular arcades, which can be unilateral or bilateral.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      Although sclerochoroidal calcification is most often idiopathic, systemic testing at the time of diagnosis is required to exclude associated disorders of calcium-phosphorus metabolism.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      Sclerochoroidal calcification has rarely been associated with choroidal neovascularization (NV) with variable clinical courses documented in the literature thus far.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      • Bessette AP
      • Singh AD.
      Multimodal imaging of choroidal neovascularization associated with sclerochoroidal calcification.
      • Cohen SY
      • Guyot-Sionnest M
      • Puech M.
      Choroidal neovascularization as a late complication of hyperparathyroidism.
      • Leys A
      • Stalmans P
      • Blanckaert J.
      Sclerochoroidal calcification with choroidal neovascularization.
      Herein, we report a case of sclerochoroidal calcification and associated choroidal neovascular membrane (CNVM) characterized by multimodal imaging, including optical coherence tomography angiography (OCTA).
      An 87-year-old white man with a 15-year history of stable, bilateral idiopathic sclerochoroidal calcification and antiphospholipid antibody syndrome on chronic anticoagulation was referred to the retina service for evaluation of a subretinal hemorrhage adjacent to sclerochoroidal calcification in the right eye. Head computed tomography (CT) scan at the time of initial diagnosis in 2004 demonstrated bilateral scleral hyperdensities consistent with calcification (Supplementary Fig. 1A [available online]); however, B-scan ultrasonography was not performed at initial diagnosis. He also underwent systemic evaluation to exclude disorders of calcium-phosphorus metabolism, which was negative. At the time of referral, the patient was asymptomatic. Best-corrected visual acuity was 20/25 in both eyes, and intraocular pressures were normal. Fundus examination of both eyes demonstrated several, ill-defined yellow choroidal lesions with areas of discrete calcification and overlying Retinal pigment epithelium (RPE) atrophy along the superotemporal arcade. The right eye demonstrated a subretinal hemorrhage along the superior border of the sclerochoroidal calcification (Fig. 1A). The left eye was notable for a stable choroidal nevus along the superonasal arcade with overlying drusen (Fig. 1B). Fundus autofluorescence of both eyes revealed hyperautofluorescence of the sclerochoroidal calcification, hypoautofluorescence in adjacent areas of RPE atrophy, and blockage owing to the hemorrhage in the right eye (Supplementary Fig. 1B, C [available online]). Fluorescein angiography of the right eye revealed hyperfluorescent late staining of the bilateral calcific lesions associated with blockage from the associated subretinal hemorrhage and a focal area of leakage suggestive of secondary choroidal NV (Fig. 1C, D). Enhanced depth imaging optical coherence tomography (EDI-OCT)` of the right eye showed multiple pigment epithelial detachments (PEDs) associated with subretinal fluid and subretinal hyper-reflective material suggestive of an active CNVM (Fig. 2A). Other areas of sclerochoroidal calcification bilaterally demonstrated that the lesions arose from the sclera with overlying choroidal thinning (Fig. 2B, C). Choriocapillaris segmentation of en-face flow OCT angiography of the right eye demonstrated a shadow artefact from the PED and hemorrhage and a possible lacy vascular network of choroidal NV that co-localized with the area of leakage found on fluorescein angiography (Fig. 2D). Given the asymptomatic nature and peripheral location of the CNVM, close observation with follow-up every 3 months was advised.
      Fig 1
      Fig. 1An 87-year-old white man was referred for evaluation of a subretinal hemorrhage. Ultra-widefield pseudocolor fundus photograph of the (A) right and (B) left eye revealed several, bilateral, ill-defined yellow choroidal lesions with areas of discrete calcification and overlying RPE atrophy along the superotemporal vascular arcades, (A) a subretinal hemorrhage along the superior border of the sclerochoroidal calcification in the right eye, and (B) a stable choroidal nevus with overlying drusen along the superonasal arcade in the left eye. (C) Late venous phase fluorescein angiogram of the right eye demonstrated blockage superior to the sclerochoroidal calcification along with hyperfluorescence of other areas of sclerochoroidal calcification consistent with intrinsic staining. (D) Recirculation phase fluorescein angiogram revealed subtle leakage, difficult to distinguish from the characteristic late staining of sclerochoroidal calcification.
      Fig 2
      Fig. 2Optical coherence tomography (OCT) and en-face flow optical coherence tomography angiography (OCTA) of an 87-year-old man with a history of sclerochoroidal calcification referred for evaluation of a subretinal hemorrhage. (A) OCT of the right eye showed multiple pigment epithelial detachments (PEDs) with subretinal fluid and subretinal hyper-reflective material suggestive of an active choroidal neovascular membrane (CNVM). (B, C) Other areas of sclerochoroidal calcification in both eyes demonstrated that the lesions arose from the sclera with overlying choroidal thinning. (D) Choriocapillaris segmentation of en-face flow OCTA of the right eye demonstrated a shadow artefact from the PED and hemorrhage and a possible lacy vascular network of CNVM (white arrow).
      In the English literature, only 4 cases of CNVM associated with sclerochoroidal calcification have been documented with variability in clinical course.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      • Bessette AP
      • Singh AD.
      Multimodal imaging of choroidal neovascularization associated with sclerochoroidal calcification.
      • Cohen SY
      • Guyot-Sionnest M
      • Puech M.
      Choroidal neovascularization as a late complication of hyperparathyroidism.
      • Leys A
      • Stalmans P
      • Blanckaert J.
      Sclerochoroidal calcification with choroidal neovascularization.
      In the most recent case published by Bessette and Singh, the patient required a number of treatments, including bevacizumab injections, photodynamic therapy, and argon laser photocoagulation, to achieve resolution of active leakage.
      • Bessette AP
      • Singh AD.
      Multimodal imaging of choroidal neovascularization associated with sclerochoroidal calcification.
      Two other cases were also successfully treated with argon laser photocoagulation.
      • Cohen SY
      • Guyot-Sionnest M
      • Puech M.
      Choroidal neovascularization as a late complication of hyperparathyroidism.
      ,
      • Leys A
      • Stalmans P
      • Blanckaert J.
      Sclerochoroidal calcification with choroidal neovascularization.
      A consecutive case series investigating the clinical manifestations and systemic associations of 27 patients with sclerochoroidal calcification by Honavar et al. reported one case of choroidal NV that remained stable without treatment through 1 year of observation.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      Although sclerochoroidal calcification is most often idiopathic, systemic testing at the time of diagnosis is required to exclude associated disorders of calcium-phosphorus metabolism, such as Bartter syndrome, Gitelman syndrome, and primary hyperparathyroidism.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      In the series by Honavar et al., 31% of patients were diagnosed with Gitelman syndrome, which carries a risk for serious systemic complications, including a higher risk of cardiovascular instability with anaesthesia.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      In the case reported herein, the patient's laboratory testing demonstrated no underlying metabolic disorder, and considering his peripheral involvement and excellent visual acuity, he was closely observed to monitor for lesion enlargement or macula-threatening hemorrhage.
      Sclerochoroidal calcification can be well characterized by multimodal imaging. On fundus autofluorescence imaging, the hyperautofluorescent nature of the lesions can presumably be explained by overlying choroidal thinning, resulting in the unmasking of underlying scleral hyperautofluorescence as well as the intrinsic hyperautofluorescence of calcific tissue within sclerochoroidal calcification.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      This choroidal thinning can also explain hyperfluorescence with persistent late staining on fluorescein angiography, which can make subtle leakage from choroidal NV difficult to identify, as exemplified in this case.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      Therefore, a multimodal imaging approach, including OCTA, may improve detection and monitoring of associated CNVM via direct visualization of the neovascular network arising from the choriocapillaris.
      A study by Inoue et al. demonstrated a sensitivity of 85.7% for detecting type 1 choroidal NV with OCTA and structural OCT versus a sensitivity of 66.7% for fluorescein angiography alone, suggesting the importance of a multimodal imaging strategy to improve detection.
      • Inoue M
      • Jung JJ
      • Balaratnasingam C
      • et al.
      A comparison between optical coherence tomography angiography and fluorescein angiography for the imaging of type 1 neovascularization.
      Not only can OCTA improve detection of associated CNVM, but OCTA might also help elucidate the cause of RPE atrophy commonly associated with sclerochoroidal calcification. Previous studies evaluating choroidal thickness in sclerochoroidal calcification have demonstrated a reduction in overlying choroidal thickness with subsequent outer retinal layer disturbances.
      • Honavar SG
      • Shields CL
      • Demirci H
      • Shields JA
      Sclerochoroidal calcification: clinical manifestations and systemic associations.
      Future series of sclerochoroidal calcification with OCTA could provide additional pathophysiologic information regarding the evolution of retinal and RPE atrophy associated with these lesions.
      In summary, we presented a case of sclerochoroidal calcification and associated CNVM documented with multimodal imaging, demonstrating that OCTA can be a valuable test to detect CNVM that is masked by late staining of sclerochoroidal calcification on fluorescein angiography. OCTA should be further explored as a tool to better understand the pathophysiology of sclerochoroidal calcification and associated changes in the retina, RPE, and choriocapillaris.

      Footnotes and Disclosure:

      The authors have no proprietary or commercial interest in any materials discussed in this article.

      Appendix. Supplementary materials

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