Perifoveal Exudative Vascular Anomalous complex

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Perifoveal exudative vascular anomalous complex (PEVAC) has been described by Querques et al. as a large isolated unilateral perifoveal aneurismal change [1]

Disease Entity

Disease

Patients with PEVAC typically have no relevant personal or family medical history.  Is typically not associated with hypertension, diabetes, blood dyscrasias, and retinal vascular occlusive or inflammatory diseases.  Very little is known about the prevalence, pathogenesis, course, and treatment of this condition.  There are no associated microaneurysms, exudative AMD and no signs of general or local inflammatory diseases which indicate the entity is different from diabetic maculopathy, sequellae from occlusive retinopathy, or associated with inflammatory diseases[2]

Etiology

The etiology is unknown.

Risk Factors

Unknown

Pathophysiology

Unknown perifoveal isolated aneurysm with dilation. Unclear pathophysiology remains could be along the spectrum of idiopathic retinal vascular abnormalities of the macula.[3][4]

Diagnosis

Clinical Diagnosis

Based on a case series by Saccon et. al. [5]i, appears as perifoveal isolated aneurysm, appearing similar to a large microaneurysm, associated with small retinal hemorrhages, intraretinal exudation, and in some cases hard exudates.  Can be seen in healthy subjects (47%), but also in patients with concomitant AMD (40%), or myopia (13%). Age of diagnosis variable (46-90), avg age 71 in one study. affecting both young and aged patients (more common in elder patients).

Diagnostic Procedures

On OCT, PEVAC appears as a round unilateral hyperreflective lesion with intraretinal cystic spaces without any indication of neovascularization .  In particular lesions illustrated a reflective wall surrounding a dark lumen containing variably reflective material. Typically surrounding by intraretinal cystic spaces. In most cases located between the outer plexiform layer (OPL) and the inner nuclear layer (INL), but may also be found in the inner plexiform later (IPL), without any signs of CNV (choroidal neovascularization).

FA demonstrates well-defined hyperfluorescent lesion with isolated aneurysmal dilation with variable leakage in the late frames.  ICGA had the same lesion noted in FA without any leakage noted.  Of note fluorescein angiography (FA) and indocyanine green angiography (ICG) demonstrates no retinal or choroidal abnormalities in patients with PEVAC.

OCT-A as been also shown to aid in the localization and visualization of retinal abnormalities, which confirmed that there was no sign of capillary remolding except for rarefaction of the capillary network around the lesion.[6][7]

Differential diagnosis

Type 1 macular telangiectasia (or a subtype)

Macroaneurysm associated with other disorders such as retinal vein occlusion, diabetic retinopathy, and or other systemic/inflammatory diseases

Neovasular AMD (deep retinal vascular anomalous complex, retinal angiomatous proliferation, or type 3 neovascularization)

Management

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General treatment

Demonstrates wide clinical spectrum. Visual decline occurring associated with CME (cystoid macular edema), however patient may be asymptomatic at baseline. No randomized control trials on the treatment of PEVAC.   Case series by Sacconi et. al., PEVAC was non-responsive to ranibizumab or aflibercept intravitreal injections.[8] However case report by Mrejen et. al. described a case of PEVAC with macular edema resolving within 8 months of treatment with anti-VEGF (total of 11 injections), and a case of PEVAC with macular edema resolving after 2 treatments of focal thermal laser photocoagulation of the aneurysm.

References

  1. Querques G, Kuhn D, Massamba N, Leveziel N, Querques L, Souied EH. Perifoveal exudative vascular anomalous complex. J Fr Ophtalmol. 2011 Oct;34(8):559.e1-4. doi: 10.1016/j.jfo.2011.03.002. Epub 2011 May 7. PMID: 21550688.
  2. Mrejen, Sarah MD, Le, Hoang Mai MD, Nghiem-Buffet, Sylvia MD, Tabary, Sandrine, Quentel, Gabriel MD, Cohen, Salomon Y. MD, PhD. INSIGHTS INTO PERIFOVEAL EXUDATIVE VASCULAR ANOMALOUS COMPLEX, Retina: January 2020 - Volume 40 - Issue 1 - p 80-86 doi: 10.1097/IAE.0000000000002435
  3. Mrejen, Sarah MD, Le, Hoang Mai MD, Nghiem-Buffet, Sylvia MD, Tabary, Sandrine, Quentel, Gabriel MD, Cohen, Salomon Y. MD, PhD. INSIGHTS INTO PERIFOVEAL EXUDATIVE VASCULAR ANOMALOUS COMPLEX, Retina: January 2020 - Volume 40 - Issue 1 - p 80-86 doi: 10.1097/IAE.0000000000002435
  4. Sacconi R, Freund KB, Yannuzzi LA, Dolz-Marco R, Souied E, Capuano V, Semoun O, Phasukkijwatana N, Sarraf D, Carnevali A, Querques L, Bandello F, Querques G. The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. Am J Ophthalmol. 2017 Dec;184:137-146. doi: 10.1016/j.ajo.2017.10.009. Epub 2017 Oct 27. PMID: 29079450
  5. Sacconi R, Freund KB, Yannuzzi LA, Dolz-Marco R, Souied E, Capuano V, Semoun O, Phasukkijwatana N, Sarraf D, Carnevali A, Querques L, Bandello F, Querques G. The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. Am J Ophthalmol. 2017 Dec;184:137-146. doi: 10.1016/j.ajo.2017.10.009. Epub 2017 Oct 27. PMID: 29079450
  6. Mrejen, Sarah MD, Le, Hoang Mai MD, Nghiem-Buffet, Sylvia MD, Tabary, Sandrine, Quentel, Gabriel MD, Cohen, Salomon Y. MD, PhD. INSIGHTS INTO PERIFOVEAL EXUDATIVE VASCULAR ANOMALOUS COMPLEX, Retina: January 2020 - Volume 40 - Issue 1 - p 80-86 doi: 10.1097/IAE.0000000000002435
  7. Sacconi R, Freund KB, Yannuzzi LA, Dolz-Marco R, Souied E, Capuano V, Semoun O, Phasukkijwatana N, Sarraf D, Carnevali A, Querques L, Bandello F, Querques G. The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. Am J Ophthalmol. 2017 Dec;184:137-146. doi: 10.1016/j.ajo.2017.10.009. Epub 2017 Oct 27. PMID: 29079450
  8. Sacconi R, Freund KB, Yannuzzi LA, Dolz-Marco R, Souied E, Capuano V, Semoun O, Phasukkijwatana N, Sarraf D, Carnevali A, Querques L, Bandello F, Querques G. The Expanded Spectrum of Perifoveal Exudative Vascular Anomalous Complex. Am J Ophthalmol. 2017 Dec;184:137-146. doi: 10.1016/j.ajo.2017.10.009. Epub 2017 Oct 27. PMID: 29079450
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