Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study

Abstract Von Hippel–Lindau (VHL) disease is an autosomal dominant genetic disease caused by VHL gene mutation. Retinal hemangioblastomas (RH) are vascularized tumors and represent the main ocular manifestation of the disease. Histopathologically, RH are composed of capillary vessels and stromal cell...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Elisabetta Pilotto, Elisabetta Beatrice Nacci, Gilda De Mojà, Alfonso Massimiliano Ferrara, Raffaele Parrozzani, Davide Londei, Stefania Zovato, Edoardo Midena
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/d8ff259dfa9a49869002046f785b0e17
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:d8ff259dfa9a49869002046f785b0e17
record_format	dspace
spelling	oai:doaj.org-article:d8ff259dfa9a49869002046f785b0e172021-12-02T15:08:22ZStructural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study10.1038/s41598-020-79652-w2045-2322https://doaj.org/article/d8ff259dfa9a49869002046f785b0e172021-01-01T00:00:00Zhttps://doi.org/10.1038/s41598-020-79652-whttps://doaj.org/toc/2045-2322Abstract Von Hippel–Lindau (VHL) disease is an autosomal dominant genetic disease caused by VHL gene mutation. Retinal hemangioblastomas (RH) are vascularized tumors and represent the main ocular manifestation of the disease. Histopathologically, RH are composed of capillary vessels and stromal cells, the neoplastic population of the lesion. The origin of these stromal cells remains controversial, even if they are hypothesized to be glial cells. The aim of the present study was to investigate neuronal and microvascular changes of the peripapillary retinal nerve fiber layer, in which glial cells, neurons and capillaries (the radial peripapillary capillary plexus) interact. VHL patients with or without peripheral RH were enrolled and compared to healthy controls. Mean peripapillary retinal nerve fiber layer (pRNFL) thickness was measured by means of optical coherence tomography (OCT). The following vascular parameters of the radial peripapillary capillary plexus were quantified using OCT angiography: Vessel Area Density,Vessel Length Fraction, Vessel Diameter Index and Fractal Dimension. One hundred and nine eyes of 61 patients, and 56 eyes of 28 controls were consecutively studied. Mean pRNFL was significantly thinner in VHL eyes without RH versus eyes with RH and controls. Mean pRNFL thickness did not differ between VHL eyes with RH and controls. All OCTA vascular parameters were reduced in VHL eyes with or without RH versus controls, with significative difference for Vessel Diameter Index. The same OCTA parameters did not significantly differ between VHL eyes with or without RH. In VHL eyes without RH, pRNFL thinning may be the consequence of impaired perfusion of the radial peripapillary capillary plexus, while the increase of pRNFL thickness in VHL eyes with RH may depend on possible activation and proliferation of the other RNFL resident cells, the glial cells.Elisabetta PilottoElisabetta Beatrice NacciGilda De MojàAlfonso Massimiliano FerraraRaffaele ParrozzaniDavide LondeiStefania ZovatoEdoardo MidenaNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-6 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Elisabetta Pilotto Elisabetta Beatrice Nacci Gilda De Mojà Alfonso Massimiliano Ferrara Raffaele Parrozzani Davide Londei Stefania Zovato Edoardo Midena Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study
description	Abstract Von Hippel–Lindau (VHL) disease is an autosomal dominant genetic disease caused by VHL gene mutation. Retinal hemangioblastomas (RH) are vascularized tumors and represent the main ocular manifestation of the disease. Histopathologically, RH are composed of capillary vessels and stromal cells, the neoplastic population of the lesion. The origin of these stromal cells remains controversial, even if they are hypothesized to be glial cells. The aim of the present study was to investigate neuronal and microvascular changes of the peripapillary retinal nerve fiber layer, in which glial cells, neurons and capillaries (the radial peripapillary capillary plexus) interact. VHL patients with or without peripheral RH were enrolled and compared to healthy controls. Mean peripapillary retinal nerve fiber layer (pRNFL) thickness was measured by means of optical coherence tomography (OCT). The following vascular parameters of the radial peripapillary capillary plexus were quantified using OCT angiography: Vessel Area Density,Vessel Length Fraction, Vessel Diameter Index and Fractal Dimension. One hundred and nine eyes of 61 patients, and 56 eyes of 28 controls were consecutively studied. Mean pRNFL was significantly thinner in VHL eyes without RH versus eyes with RH and controls. Mean pRNFL thickness did not differ between VHL eyes with RH and controls. All OCTA vascular parameters were reduced in VHL eyes with or without RH versus controls, with significative difference for Vessel Diameter Index. The same OCTA parameters did not significantly differ between VHL eyes with or without RH. In VHL eyes without RH, pRNFL thinning may be the consequence of impaired perfusion of the radial peripapillary capillary plexus, while the increase of pRNFL thickness in VHL eyes with RH may depend on possible activation and proliferation of the other RNFL resident cells, the glial cells.
format	article
author	Elisabetta Pilotto Elisabetta Beatrice Nacci Gilda De Mojà Alfonso Massimiliano Ferrara Raffaele Parrozzani Davide Londei Stefania Zovato Edoardo Midena
author_facet	Elisabetta Pilotto Elisabetta Beatrice Nacci Gilda De Mojà Alfonso Massimiliano Ferrara Raffaele Parrozzani Davide Londei Stefania Zovato Edoardo Midena
author_sort	Elisabetta Pilotto
title	Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study
title_short	Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study
title_full	Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study
title_fullStr	Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study
title_full_unstemmed	Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study
title_sort	structural and microvascular changes of the peripapillary retinal nerve fiber layer in von hippel–lindau disease: an oct and oct angiography study
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/d8ff259dfa9a49869002046f785b0e17
work_keys_str_mv	AT elisabettapilotto structuralandmicrovascularchangesoftheperipapillaryretinalnervefiberlayerinvonhippellindaudiseaseanoctandoctangiographystudy AT elisabettabeatricenacci structuralandmicrovascularchangesoftheperipapillaryretinalnervefiberlayerinvonhippellindaudiseaseanoctandoctangiographystudy AT gildademoja structuralandmicrovascularchangesoftheperipapillaryretinalnervefiberlayerinvonhippellindaudiseaseanoctandoctangiographystudy AT alfonsomassimilianoferrara structuralandmicrovascularchangesoftheperipapillaryretinalnervefiberlayerinvonhippellindaudiseaseanoctandoctangiographystudy AT raffaeleparrozzani structuralandmicrovascularchangesoftheperipapillaryretinalnervefiberlayerinvonhippellindaudiseaseanoctandoctangiographystudy AT davidelondei structuralandmicrovascularchangesoftheperipapillaryretinalnervefiberlayerinvonhippellindaudiseaseanoctandoctangiographystudy AT stefaniazovato structuralandmicrovascularchangesoftheperipapillaryretinalnervefiberlayerinvonhippellindaudiseaseanoctandoctangiographystudy AT edoardomidena structuralandmicrovascularchangesoftheperipapillaryretinalnervefiberlayerinvonhippellindaudiseaseanoctandoctangiographystudy
_version_	1718388217262112768

Structural and microvascular changes of the peripapillary retinal nerve fiber layer in Von Hippel–Lindau disease: an OCT and OCT angiography study

Ejemplares similares