Segmented swept source optical coherence tomography angiography assessment of the perifoveal vasculature in patients with X-linked juvenile retinoschisis: a serial case report

Abstract

PurposeTo describe perifoveal microvascular changes occurring in X-linked juvenile retinoschisis (XLRS) using swept source optical coherence tomography angiography (SS OCTA).Patients and methodsThis is a serial case report of three patients. Retrospective data of patients affected by XLRS were collected. Structural optical coherence tomography (OCT) and color fundus photography (CFPh) were carried out with Topcon® OCT 2000 3D OCT as part of the standard care. Two patients were imaged on Topcon Atlantis® SS OCTA and one on Topcon Triton® SS OCTA. SS OCTA images were acquired using the 3 × 3 mm fovea-centered cubes scanning protocol. Analysis of both perifoveal superficial vascular plexus (pSVP) and perifoveal deep vascular plexus (pDVP) was performed by two observers after automated segmentation.ResultsFour eyes of three males (mean age 14 ± 3.8 years) were analyzed. All eyes showed foveoschisis on CFPh images. OCT B-scans of three eyes showed schistic cysts in the ganglion cell layer, inner nuclear layer (INL) and outer nuclear layer (ONL); in one eye, cysts were depicted in INL and ONL only. In two eyes, SS OCTA showed abnormal foveal avascular zone (FAZ) shape in the pSVP, and in the other two, FAZ shape was abnormal in both plexuses. In all eyes, retinal vascular abnormalities (ie, microvascular protrusions) were present in pDVP.ConclusionSS OCTA can depict perifoveal microvascular changes in young patients affected by XLRS. In this study, the structural and vascular changes seem to be more evident in the pDVP and may represent a useful biomarker of prognosis.

Multimodal imaging in retinoschisis X-linked

“…In addition, central macular and choroidal thickness have increased (Figure 1C). In agreement with other authors, OCTA (Angiovue System, Optovue Inc. Fremont, CA, USA) shows an increase of the foveal avascular zone in both superficial and deep vascular plexus and the presence of teleangectasias (Figure 2A and 2B) [4][5][6][7][8]. In Face images reveal the presence of reflective areas that appear like a petaoloid or coral shape without coriocapillaris alteration (Figure 2A-2C).…”

“…Clinically, the patients show typically a cystic-like stellate maculopathy or a foveal schisis and peripheral retinoschisis of various degrees of severity on fundoscopy [3]. Other authors described this condition in Optical Coherence Tomography (OCT) and OCT angiography (OCTA) as splitting of inner retinal layers and perifoveal microvascular changes, more evident in the deep vascular plexus [4]. The splitting of the inner retinal layers, mainly involving Inner Nuclear Layer (INL) followed by Outer Plexiform Layer (OPL) and ganglion cell layer (GCL), determines the formation of foveal schisis that leads to early bilateral visual loss [5].…”

Clinical and Genetic Study of X-Linked Juvenile Retinoschisis in the Czech Population

“…OCTA imaging, which is a relatively new method, was similar to previously described cases and documented perifoveal microvascular changes. The usefulness of this method for monitoring disease progression or treatment response is yet to be established [39][40][41][42].…”

Optical Coherence Tomography Angiography: Practical Usefulness

“…OCTA has been shown to detect foveoschisis and vascular changes associated with it. Stringa and colleagues 27 evaluated this in patients with X-linked retinoschisis. OCT B-scan demonstrated schisis of the ganglion cell layer, inner nuclear layer, and outer nuclear layer.…”