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. 2023 Sep 1;17(5):595-599.
doi: 10.1097/ICB.0000000000001264.

MACULAR NEUROVASCULAR ABNORMALITIES IN A CHILD WITH INCONTINENTIA PIGMENTI ON HANDHELD OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY

Pujan R Patel  1   2 , Vincent Tai  1 , Ryan Imperio  1 , Christian Viehland  3 , Joseph A Izatt  1   3 , Cynthia A Toth  1   3 , Xi Chen  1
Affiliations

MACULAR NEUROVASCULAR ABNORMALITIES IN A CHILD WITH INCONTINENTIA PIGMENTI ON HANDHELD OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY

Pujan R Patel et al. Retin Cases Brief Rep. .

Abstract

Purpose: To report macular neurovascular abnormalities in a child with incontinentia pigmenti using handheld optical coherence tomography (OCT) and OCT angiography (OCT-A).

Methods: An eye of a child with incontinentia pigmenti enrolled in BabySTEPS was imaged using an investigational noncontact, handheld swept-source OCT device during examination under anesthesia. Custom MATLAB scripts were used to generate depth-resolved vascular slabs, B-scans with flow overlay, and retinal thickness maps.

Results: Depth-resolved OCT and OCT-A imaging demonstrated focal areas of decreased capillary flow that corresponded to areas of both inner retinal and outer retinal thinning on retinal thickness maps. Atypical diving of superficial retinal vessels occurred as they traversed from thin retina to normal-thickness retina.

Conclusion: Depth-resolved OCT and OCT-A identified retinal vascular abnormalities that were not evident on fundus photography or fluorescein angiography. This case depicted concurrent, localized abnormalities in retinal thickness and microvasculature in an eye with incontinentia pigmenti.

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Conflict of interest statement

J. A. Izatt and C. A. Toth have a segmentation related patent (US10366492B2). C. Viehland, J. A. Izatt, and C. A. Toth and Duke University have a patent application pending on the novel handheld probe described in this manuscript. C. A. Toth receives royalties through her university, from Alcon. C. A. Toth consults for EMMES Inc. C. Viehland and C. A. Toth have owner equity in Theia Imaging, LLC. J. A. Izatt receives royalties through Duke University from Leica Microsystems, Inc. No other authors have related conflict of interest.

Figures

Figure 1.
Figure 1.
Fundus photography (A) and fluorescein angiography (B) of the right eye of a 19-month old girl with incontinentia pigmenti. A large area of temporal avascular retina was observed (arrows).
Figure 2.
Figure 2.
Superficial vascular complex (SVC) (A) and deep vascular complex (DVC) (B) slabs of the macula of the right eye demonstrated decreased capillary flow in the perifoveal area of retinal thinning (A, B, dashed lines). Three medium-sized retinal vessels abruptly ended in the SVC slab (A, circles) and continued in the DVC slab (B, downstream of the circles), consistent with vascular diving. The retinal thickness map (C) showed that vascular diving occurred at the location where thin retina transitioned to normal-thickness retina (C, circles). Cross-sectional B-scans with flow overlay (D, E) showed an irregular foveal contour and a medium-sized retinal vessel traversing from the SVC territory (above the cyan line) (D, arrow) to the DVC territory (below the cyan line) (E, arrow). The cyan lines (D, E) denoted the inner plexiform layer / inner nuclear layer border.
Figure 3.
Figure 3.
Superficial (inner limiting membrane to inner plexiform layer, (A)) and deep (inner nuclear layer to the inner retinal pigment epithelium, (B)) retinal thickness maps exhibited similar pattern of perifoveal thinning as what was observed on the full retinal thickness map in Figure 2. Cross-sectional B-scan with flow overlay (C) showed simultaneous thinning in the ganglion cell layer (above the cyan line), inner nuclear layer (immediately below the cyan line), and outer plexiform layer (also deviated upward below the cyan line), with all three occurring between the arrows. The cyan line (C) denoted the inner plexiform layer / inner nuclear layer border.
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