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Tumor vasoproliferativo da retina
ORPHA:353356CID-10 · D31.2CID-11 · 2F36.YDOENÇA RARA
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Introdução

O que você precisa saber de cara

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A síndrome de Goldmann-Favre é um distúrbio genético raro caracterizado por nictalopia de início precoce, diminuição da acuidade visual e achados anormais no fundo do olho. É um tipo de degeneração vitreotapetorretiniana progressiva.

Publicações científicas
14 artigos
Último publicado: 2025 Jan 1
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SUS: Sem cobertura SUSScore: 0%
CID-10: D31.2
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Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico14PubMed
Últimos 10 anos6publicações
Pico20222 papers
Linha do tempo
2025Hoje · 2026
Publicações por ano (últimos 10 anos)

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Publicações mais relevantes

Timeline de publicações
6 papers (10 anos)
#1

VASCULAR CHANGES AND IRREVERSIBLE COMPLICATIONS IN 120° FUNDUS USING WIDEFIELD SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN VOGT-KOYANAGI-HARADA DISEASE.

Retina (Philadelphia, Pa.)2025 Jan 01

To characterize the changes in fundus corresponding to 120° field of view in chronic Vogt-Koyanagi-Harada disease in the quiescent phase and explore the associations with irreversible complications in the fundus using widefield swept-source optical coherence tomography angiography. Prospective cross-sectional study. Sixty-nine patients with chronic Vogt-Koyanagi-Harada disease (115 eyes) and 55 healthy control subjects (110 eyes) were included and underwent widefield swept-source optical coherence tomography angiography. Univariate analyses of variations in retinal and choroidal vessel density (VD), choroidal volume, and choroidal vascularity index in patients with Vogt-Koyanagi-Harada with different disease durations and the control subjects were conducted. Logistic regression analysis was used to identify the associations with irreversible complications, including choroidal neovascularization, vasoproliferative tumor of the retina, and chorioretinal atrophy. The Welch analysis of variance showed lower VD of superficial retina, deep retina, choriocapillaris, and large-sized and medium-sized vessels of the choroid, and choroidal volume and choroidal vascularity index in the patients with disease duration of >24 months compared with those with disease duration of ≤24 months (all P ≤ 0.011). The regression analysis revealed that the disease duration ( P = 0.008; OR = 1.02, 95% CI, 1.005-1.035) and VD of large-sized and medium-sized vessels of the choroid ( P = 0.001; OR = 0.707, 95% CI, 0.575-0.87) were significantly correlated with the irreversible complications. Patients with chronic Vogt-Koyanagi-Harada in the quiescent phase with disease duration of >24 months exhibit more severe decreased VD in each layer of the retina and choroid, reduced choroidal volume, and sparse choroidal vascularity compared with those with disease duration ≤24 months. Prolonged duration and decreased VD of large-sized and medium-sized vessels of the choroid were associated with irreversible complications in the fundus.

#2

[Morphological features of vasoproliferative tumor of the retina].

Arkhiv patologii2024

Vasoproliferative retinal tumor (VPT) is a term proposed by ophthalmologists in relation to the totality of manifestations of an intraocular volumetric process with involvement of the inner lining of the eye, an integral part of which is the active growth of blood vessels. The available literature data on the morphology of this process are very contradictory and ambiguous. The article presents two clinical cases of vasoproliferative retinal tumor with own illustration of morphological studies. «Вазопролиферативная опухоль сетчатки» — термин, предложенный офтальмологами в отношении совокупности проявлений внутриглазного объемного процесса с вовлечением внутренней оболочки глаза, неотъемлемой частью которого является активный рост сосудов. Имеющиеся данные литературы о морфологии этого процесса весьма противоречивы и неоднозначны. В статье представлено два наблюдения вазопролиферативной опухоли сетчатки с иллюстрацией собственных морфологических исследований.

#3

SURGICAL OUTCOMES OF PARS PLANA VITRECTOMY FOR INTRAOCULAR COMPLICATIONS RELATED TO VASOPROLIFERATIVE TUMOR OF THE RETINA.

Retina (Philadelphia, Pa.)2023 Nov 01

To investigate the safety and efficacy of vitrectomy pars plana vitrectomy in managing intraocular complications relating to vasoproliferative tumors of the retina (VPL). Retrospective study. 17 patients with VPL who underwent vitrectomy at Sheffield Teaching Hospital NHS Trust from 2005 to 2020 were included. Patient demographics, clinical characteristics, intraoperative data, and surgical outcomes were collected and evaluated. The mean age was 52 years. Indications for pars plana vitrectomy included epiretinal membrane (n = 7), vitreous hemorrhage (n = 5), retinal detachment (n = 3), diagnostic (n = 1), and others (n = 1). After pars plana vitrectomy, 14/17 (82.4%) have stabilized vision and 3/17 (17.6%) deteriorated. Subgroup analysis of epiretinal membrane peel had good outcomes with 6/7 (85.7%) noticing improvement or stabilization of symptoms and mean logarithm of minimal angle of resolution visual acuity improved from 0.719 [6/30] ± 0.267 [6/12] to 0.476 [6/19] ± 0.271 [6/12]. Patients undergoing surgery for vitreous hemorrhage also had good outcomes with resolution (and no recurrence) of the hemorrhage in 3/5 (60%) and 5/5 (100%) with one or more surgeries, respectively. Outcomes for retinal detachment surgery were logarithm of minimal angle of resolution 2.126 [HM] ± 0.301 [6/12] preoperatively and 1.185 [6/95] ± 0.522 [6/19] postoperatively, with one recurrence of retinal detachment. In the epiretinal membrane group, three patients had adjunctive treatment for VPL intraoperatively and four patients had none, and no difference found between the two groups in outcome or complications. Tumor with thickness of ≥2 mm exhibited inferior visual outcomes compared with <2 mm ( P < 0.05). This is one of the largest data sets in looking at outcomes of vitrectomy for complications of VPL. Pars plana vitrectomy is effective and safe in managing VPL-related intraocular complications with good outcomes and a low rate of complications, especially for patients with epiretinal membrane and vitreous hemorrhage.

#4

Photocoagulation-associated spontaneous release of epiretinal membrane secondary to retinal vascular tumor: case series of 8 cases.

Lasers in medical science2022 Mar

To evaluate the secondary epiretinal membrane (ERM) response after photocoagulation in retinal vascular tumor. This retrospective interventional case series included 8 patients (8 eyes) who were diagnosed with retinal vascular tumor and secondary ERM. All eyes were treated with photocoagulation and underwent comprehensive ophthalmologic examinations at baseline and at each follow-up. Of the 8 eyes with retinal vascular tumor and associated ERM, 4 eyes (50%) were von Hippel and 4 eyes (50%) were vasoproliferative tumor of the retina. The mean follow-up time was 12.63 ± 14.64 (range, 4-51) months. The BCVA in the eyes at baseline was 1.16 ± 1.10 logMAR (range, HM to 20/40). ERM located in the macular region in 100% of the eyes and led to CME with a mean central foveal thickness of 497.6 ± 147.7 μm (range, 294-736 μm) at presentation. After photocoagulation, the ERM spontaneously peeled in 7 of 8 eyes (87.5%), among which one case required surgical treatment due to complicating tractional retinal detachment. After ERM peeling without complications, 6 eyes recovered normal macular structure, with an improved BCVA in 5 eyes and a stable BCVA in 1 eye. Laser photocoagulation is necessary and effective treatment for retinal vascular tumor. After laser photocoagulation, retinal vascular tumor-related ERM spontaneously released in 75% of the cases, without complication and surgical intervention.

#5

Case Report: Exacerbation and Spontaneous Separation of the Epiretinal Membrane Following Laser Photocoagulation of a Vasoproliferative Tumor of the Retina.

Frontiers in medicine2022

The present report concerns a rare vasoproliferative tumor of the retina (VPTR) combined with a severe case of secondary epiretinal membrane (ERM). A 56-year-old male patient was diagnosed with VPTR and secondary ERM of the left eye. The patient underwent two rounds of laser photocoagulation (LP) of the tumor. The exacerbation of the ERM was observed after the first round of LP, while spontaneous separation over the five-month follow-up period was noted after the second round of LP. Thus, LP may represent a viable alternative treatment approach for VPTR combined with severe ERM.

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. VASCULAR CHANGES AND IRREVERSIBLE COMPLICATIONS IN 120&#xb0; FUNDUS USING WIDEFIELD SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN VOGT-KOYANAGI-HARADA DISEASE.
    Retina (Philadelphia, Pa.)· 2025· PMID 39173134mais citado
  2. [Morphological features of vasoproliferative tumor of the retina].
    Arkhiv patologii· 2024· PMID 38591906mais citado
  3. SURGICAL OUTCOMES OF PARS PLANA VITRECTOMY FOR INTRAOCULAR COMPLICATIONS RELATED TO VASOPROLIFERATIVE TUMOR OF THE RETINA.
    Retina (Philadelphia, Pa.)· 2023· PMID 37418640mais citado
  4. Photocoagulation-associated spontaneous release of epiretinal membrane secondary to retinal vascular tumor: case series of 8 cases.
    Lasers in medical science· 2022· PMID 34138385mais citado
  5. Case Report: Exacerbation and Spontaneous Separation of the Epiretinal Membrane Following Laser Photocoagulation of a Vasoproliferative Tumor of the Retina.
    Frontiers in medicine· 2022· PMID 35280881mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:353356(Orphanet)
  2. MONDO:0018148(MONDO)
  3. GARD:21533(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55787766(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Compêndio · Raras BR

Tumor vasoproliferativo da retina

ORPHA:353356 · MONDO:0018148
CID-10
D31.2 · Neoplasia benigna da retina
CID-11
MedGen
UMLS
C4749792
EuropePMC
Wikidata
Papers 10a
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