Optical Coherence Tomography Angiography in patients with Neurofibromatosis type 1: a quantitative vascular prospective study

2021 ◽  
Author(s):  
Gilda Cennamo ◽  
Daniela Montorio ◽  
Maria Paola Laezza ◽  
Piera Giunta ◽  
Pasquale Fabio Barra ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Prospective Study ◽  
Neurofibromatosis Type 1 ◽  
Optical Coherence Tomography Angiography ◽  
Neurofibromatosis Type ◽  
Optical Coherence

Optical coherence tomography angiography of choroidal nodules in neurofibromatosis type-1: A case series

2021 ◽  
pp. 112067212110307
Author(s):  
Francisco Javier Moreno-Morillo ◽  
José Ignacio Fernández-Vigo ◽  
Bárbara Burgos-Blasco ◽  
Carlos Llorente-La Orden ◽  
Beatriz Vidal-Villegas ◽  
...  
Keyword(s):  
Optical Coherence Tomography ◽  
Neurofibromatosis Type 1 ◽  
Optical Coherence Tomography Angiography ◽  
Case Series ◽  
Neurofibromatosis Type ◽  
Vessel Density ◽  
Enhanced Depth Imaging ◽  
Optical Coherence ◽  
Near Infrared Reflectance

Purpose: To describe optical coherence tomography angiography (OCTA) findings in the retina and choroid of patients with neurofibromatosis type 1 (NF1). Methods: We describe a series of four patients diagnosed with NF1 and choroidal nodules who underwent a comprehensive ophthalmic evaluation including a retinal multimodal imaging study based on retinography, near-infrared reflectance imaging (NIR), enhanced depth imaging (EDI) optical coherence tomography (OCT) and OCTA. Results: Patients were three women and one man aged 36–47 years. In all patients, the choroidal nodules were not visible in retinographies but easily detectable with NIR, appearing as multiple bright patches. On OCTA, we observed reduced vessel density in the choriocapillaris in zones where choroidal nodules appeared in OCT images. In one patient, a corkscrew vessel was visible in the superficial capillary plexus. Conclusion: Choriocapillaris vessel density was reduced in zones where choroidal nodules occur in NF1 patients. Further work is needed to clarify the clinical relevance of this finding.

scholarly journals Optical Coherence Tomography Angiography of Retinal Microvascular Changes Overlying Choroidal Nodules in Neurofibromatosis Type 1

2017 ◽  
Vol 8 (1) ◽  
pp. 214-220 ◽  
Author(s):  
Catherine Cassiman ◽  
Ingele Casteels ◽  
Peter Stalmans ◽  
Eric Legius ◽  
Julie Jacob
Keyword(s):  
Optical Coherence Tomography ◽  
Neurofibromatosis Type 1 ◽  
Optical Coherence Tomography Angiography ◽  
Neurofibromatosis Type ◽  
Retinal Vasculature ◽  
Retinal Vessels ◽  
Microvascular Changes ◽  
Capillary Plexus ◽  
Superficial Capillary Plexus

Purpose: To report 3 cases of neurofibromatosis type 1 (NF1) with choroidal nodules associated with retinal microvascular changes imaged with optical coherence tomography angiography (OCTA). Methods: Small case series in 3 NF1 patients. OCTA examinations were performed by a trained examiner (J.J.) after pupillary dilation. A standard scan, centered over the macula measuring 6 × 6 mm and 3 × 3 mm was obtained according to the findings on standard color photography. Additional scans were obtained in the zones with microvascular abnormalities. The segmentation provided by the machine software was used. Results: Corkscrew retinal vessels were observed in association with “placoid”-type choroidal nodules as shown by near-infrared reflectance imaging. In all cases, multiple lesions were found. They were second- or third-order tortuous vessels originating from the superior or inferior temporal veins. OCTA demonstrated that the tortuous venules were located in the superficial capillary plexus, and no abnormalities were found in the deep capillary plexus. Discussion: Corkscrew retinal vessels are part of a spectrum of retinal microvascular alterations seen in association, sometimes overlying choroidal nodules in patients with NF1 and are visualized in the superficial capillary plexus on OCTA. We demonstrated with OCTA that they are not associated with flow loss or ischemia in the superficial and deep capillary plexus. The link between the underlying nodule remains unclear. Since neovascularization was described in choroidal ganglioneuroma, we hypothesize that corresponding secretory substances from Schwann cells, ganglion cells, or melanocytes in choroidal nodules might alter the retinal vasculature. Conclusion: We report on 3 cases of NF1 with choroidal nodules in association with retinal microvascular changes imaged with OCTA. OCTA demonstrated preservation of the blood flow in the deep and superficial capillary plexus of the retina. We hypothesize that angiogenic factors secreted by the underlying choroidal nodules could have an effect on the retinal vasculature. Further immunohistological studies in NF1 patients with choroidal nodules to detect angiogenic factors (such as VEGF) are necessary to confirm this hypothesis.

scholarly journals Optical Coherence Tomography Imaging of Choroidal Abnormalities in Neurofibromatosis Type 1

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Shinji Makino ◽  
Hironobu Tampo
Keyword(s):  
Optical Coherence Tomography ◽  
Neurofibromatosis Type 1 ◽  
Fundus Autofluorescence ◽  
Neurofibromatosis Type ◽  
Posterior Pole ◽  
Optical Coherence ◽  
Tomography Imaging ◽  
Optical Coherence Tomography Imaging

We report a case of neurofibromatosis type 1 (NF1) examined by infrared fundus autofluorescence (IR-FAF) and optical coherence tomography (OCT) to characterize the associated choroidal abnormalities. The conventional ophthalmoscopic findings were unremarkable. However, IR-FAF revealed multiple bright patchy lesions in the choroid of the posterior pole, in both eyes. OCT demonstrated irregular hyperreflectivity at the sites of these lesions. Patients with NF1 may have typical choroidal lesions that are visible on IR-FAF, which can be confirmed through OCT.

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