June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
The presence of the LAPPEL sign in multiple inherited and acquired retinal vascular diseases and temporal correlation with capillary dropout.
Author Affiliations & Notes
  • Prethy Rao
    Associated Retinal Consultants, Royal Oak, Michigan, United States
  • Aristomenis Thanos
    Associated Retinal Consultants, Royal Oak, Michigan, United States
  • Jeremy D Wolfe
    Associated Retinal Consultants, Royal Oak, Michigan, United States
  • Michael Thomas Trese
    Associated Retinal Consultants, Royal Oak, Michigan, United States
  • Footnotes
    Commercial Relationships   Prethy Rao, None; Aristomenis Thanos, None; Jeremy Wolfe, None; Michael Trese, None
  • Footnotes
    Support  none
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 1542. doi:
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      Prethy Rao, Aristomenis Thanos, Jeremy D Wolfe, Michael Thomas Trese; The presence of the LAPPEL sign in multiple inherited and acquired retinal vascular diseases and temporal correlation with capillary dropout.
      . Invest. Ophthalmol. Vis. Sci. 2017;58(8):1542.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : To demonstrate that the LAPPEL sign (Late phase fluorescein Angiographic Peripheral or PostErior Leakage) is present in many retinal vascular diseases. To demonstrate a temporal association between LAPPEL and development of subsequent capillary loss in retinal vein occlusions (RVO).

Methods : This is a retrospective review of consecutive widefield and ultra-wide field fluoroscein angiograms (FA) in multiple inherited and acquired retinal vascular diseases that were performed at a tertiary referral center. FAs were independently assessed for the presence of a LAPPEL lesion. Serial ultra-wide field angiograms were then examined in eyes with RVOs to specifically determine the onset of the LAPPEL lesion and the presence of subsequent capillary dropout between 2013-2016.

Results : We present examples of LAPPEL in eyes with familial exudative vitreoretinopathy (n=10), Coats’ Disease (n=2), sickle cell retinopathy (n=2), retinal vein occlusions (n=72), and diabetic retinopathy (n=2).

In the 1139 RVO patient cohort, 131 had ultra-widefield FA. Seventy-two eyes (55%) had LAPPEL. Of these, twenty-three RVO eyes (46%) had serial ultra-widefield angiograms (n=15 CRVO, n=3 BRVO, n=5 HRVO) with an average of 2.8 FAs (2-4). Mean follow up duration was 20.7 months (0.5-41.4). Twenty eyes (87%) developed capillary dropout in the areas corresponding to prior LAPPEL. Average time to dropout was 10.9 months (0.17-25.1).

Conclusions : The LAPPEL sign is seen in multiple diseases and may represent damaged retinal endothelial cells, which can progress to endothelial cell death and capillary loss as demonstrated in RVO eyes. In previous tissue cultures, we have shown that human retinal endotheial cells exposed to VEGF induce selective destruction of inter-endothelial cell adhesive proteins (Claudin 5 and VE cadherin), which promotes breakdown of blood-retina barrier and endothelial tight junctions. The activation of the Wnt signaling pathway by Norrin can repair these VEGF-mediated changes in subsequent cultures. Currently, there is no treatment until loss capillary loss occurs, and then destructive laser is done. We suggest that treatment of damaged retinal endothelial cells prior to capillary loss or extensive VEGF-driven exudation medically may lead to preservation of vision and visual field.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

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