Coats disease associated with vasoproliferative tumor treated with cryotherapy and intravitreal injections of triamcinolone / Doença de Coats associada a tumor vasoproliferativo tratada com crioterapia e injeções intravítreas de triancinolona

Abstract Report of a case of Coats disease associated with retinal vasoproliferative tumor in a young female patient with two peripheral vascularized tumors and lipid exudation involving the macula and peripapillary region with serous retinal detachment areas and pre-papillary fibrous proliferation. The proposed and performed treatment was the intravitreal injection of triamcinolone acetonide to decrease the tumor exudation, followed by photocoagulation of the peripheral areas of telangiectasia without subretinal fluid and cryotherapy of the tumors. Despite that this is a rare and difficult to treat combination, in this case report, success was obtained in receding the tumor masses and reapplying the retina, leading to anatomic and visual stabilization.

Resumo Relato de um caso de Doença de Coats associada a tumor vasoproliferativo de retina em uma paciente jovem com duas tumorações vascularizadas periféricas e exsudação lipídica acometendo mácula e região peripapilar com áreas de descolamento de retina seroso e proliferação fibrosa pré-papilar. O tratamento proposto e realizado foi a injeção intra-vítrea de triancinolona para diminuir a exsudação do tumor, seguida de fotocoagulação periférica das áreas de telangiectasia sem fluido subretiniano e criocoagulação dos tumores. Apesar de se tratar de uma associação rara e de difícil tratamento, neste relato de caso, obteve-se êxito em regredir as massas tumorais e reaplicar a retina, levando à estabilização anatômica e visual.

Retinopathy of Prematurity-assist: Novel Software for Detecting Plus Disease.

PURPOSE: To design software with a novel algorithm, which analyzes the tortuosity and vascular dilatation in fundal images of retinopathy of prematurity (ROP) patients with an acceptable accuracy for detecting plus disease. METHODS: Eighty-seven well-focused fundal images taken with RetCam were classified to three groups of plus, non-plus, and pre-plus by agreement between three ROP experts. Automated algorithms in this study were designed based on two methods: the curvature measure and distance transform for assessment of tortuosity and vascular dilatation, respectively as two major parameters of plus disease detection. RESULTS: Thirty-eight plus, 12 pre-plus, and 37 non-plus images, which were classified by three experts, were tested by an automated algorithm and software evaluated the correct grouping of images in comparison to expert voting with three different classifiers, k-nearest neighbor, support vector machine and multilayer perceptron network. The plus, pre-plus, and non-plus images were analyzed with 72.3%, 83.7%, and 84.4% accuracy, respectively. CONCLUSIONS: The new automated algorithm used in this pilot scheme for diagnosis and screening of patients with plus ROP has acceptable accuracy. With more improvements, it may become particularly useful, especially in centers without a skilled person in the ROP field.

Signos vasculares retinianos en recién nacidos con cardiopatía congénita crítica: serie de casos / Retinovascular findings in newborns with critical congenital heart disease: A case series

La cardiopatia congènita crítica (CCC) podría provocar cambios en la vasculatura de la retina. Sin embargo, no se dispone de suficientes datos sobre este problema en los recién nacidos. Se evaluaron los cambios en la vasculatura retiniana en una serie de 43 recién nacidos con CCC. Se los dividió en dos grupos según el tipo de CCC; grupo 1 (n= 18): CCC obstructiva izquierda y grupo 2 (n= 25): CCC obstructiva derecha. Se detectaron enfermedades vasculares retinianas en 21 pacientes (48,8%); estas fueron más frecuentes en el grupo 1 (p= 0,04). La patología más común fue la tortuosidad vascular retiniana, observada en seis pacientes (33,3%) del grupo 1 y en 4 (16,0%) del grupo 2. Ninguno de los 21 pacientes con cambios en la vasculatura de la retina requirió tratamiento durante el seguimiento. En un análisis multivariado, solo la CCC obstructiva izquierda estuvo asociada con el desarrollo de enfermedad vascular retiniana (P= 0,03, razón de probabilidades --#91;OR--#93;: 2,8, intervalo de confianza --#91;IC--#93; del 95%: 1,1-7,4). Los cambios vasculares retinianos son frecuentes en los recién nacidos con CCC.

Critical congenital heart disease (CCHD) may cause changes in retinal vasculature. However, there is lack of data in this issue in newborns. We evaluated retinovascular changes in a series of 43 newborn with CCHD. They were divided into 2 groups according to the type of CCHD; group 1 (n= 18): left obstructive CCHD and group 2 (n= 25): right obstructive CCHD. Retinovascular pathologies were detected in 21 patients (48.8%); it was more frequent in group 1 (p= 0.04). The most common pathology was retinal vascular tortuosity in 6 patients (33.3%) of group 1, and 4 (16.0%) in group 2. None of 21 patients with retinovascular changes required any therapy at follow-up. In multivariate analysis, only having a left obstructive CCHD was associated with the development of any retinovascular pathology (P= 0.03, OR: 2.8, CI95%: 1.1-7.4). Retinovascular changes are frequent in newborn patients with CCHD.

Retinovascular findings in newborns with critical congenital heart disease: A case series. / Signos vasculares retinianos en recién nacidos con cardiopatía congénita crítica: serie de casos.

Critical congenital heart disease (CCHD) may cause changes in retinal vasculature. However, there is lack of data in this issue in newborns. We evaluated retinovascular changes in a series of 43 newborn with CCHD. They were divided into 2 groups according to the type of CCHD; group 1 (n= 18): left obstructive CCHD and group 2 (n= 25): right obstructive CCHD. Retinovascular pathologies were detected in 21 patients (48.8%); it was more frequent in group 1 (p= 0.04). The most common pathology was retinal vascular tortuosity in 6 patients (33.3%) of group 1, and 4 (16.0%) in group 2. None of 21 patients with retinovascular changes required any therapy at follow-up. In multivariate analysis, only having a left obstructive CCHD was associated with the development of any retinovascular pathology (P= 0.03, OR: 2.8, CI95%: 1.1-7.4). Retinovascular changes are frequent in newborn patients with CCHD.

La cardiopatia congènita crítica (CCC) podría provocar cambios en la vasculatura de la retina. Sin embargo, no se dispone de suficientes datos sobre este problema en los recién nacidos. Se evaluaron los cambios en la vasculatura retiniana en una serie de 43 recién nacidos con CCC. Se los dividió en dos grupos según el tipo de CCC; grupo 1 (n= 18): CCC obstructiva izquierda y grupo 2 (n= 25): CCC obstructiva derecha. Se detectaron enfermedades vasculares retinianas en 21 pacientes (48,8%); estas fueron más frecuentes en el grupo 1 (p= 0,04). La patología más común fue la tortuosidad vascular retiniana, observada en seis pacientes (33,3%) del grupo 1 y en 4 (16,0%) del grupo 2. Ninguno de los 21 pacientes con cambios en la vasculatura de la retina requirió tratamiento durante el seguimiento. En un análisis multivariado, solo la CCC obstructiva izquierda estuvo asociada con el desarrollo de enfermedad vascular retiniana (P= 0,03, razón de probabilidades [OR]: 2,8, intervalo de confianza [IC] del 95%: 1,1-7,4). Los cambios vasculares retinianos son frecuentes en los recién nacidos con CCC.

Retinopathy of Prematurity-assist: Novel Software for Detecting Plus Disease

PURPOSE: To design software with a novel algorithm, which analyzes the tortuosity and vascular dilatation in fundal images of retinopathy of prematurity (ROP) patients with an acceptable accuracy for detecting plus disease. METHODS: Eighty-seven well-focused fundal images taken with RetCam were classified to three groups of plus, non-plus, and pre-plus by agreement between three ROP experts. Automated algorithms in this study were designed based on two methods: the curvature measure and distance transform for assessment of tortuosity and vascular dilatation, respectively as two major parameters of plus disease detection. RESULTS: Thirty-eight plus, 12 pre-plus, and 37 non-plus images, which were classified by three experts, were tested by an automated algorithm and software evaluated the correct grouping of images in comparison to expert voting with three different classifiers, k-nearest neighbor, support vector machine and multilayer perceptron network. The plus, pre-plus, and non-plus images were analyzed with 72.3%, 83.7%, and 84.4% accuracy, respectively. CONCLUSIONS: The new automated algorithm used in this pilot scheme for diagnosis and screening of patients with plus ROP has acceptable accuracy. With more improvements, it may become particularly useful, especially in centers without a skilled person in the ROP field.

Aplasia of the optic nerve.

Aplasia of the optic nerve is an extraordinarily rare congenital anomaly that affects one or both optic nerves and is associated with the absence of the central retinal vessel and retinal ganglion cells. We report a case of unilateral optic nerve aplasia in a 4-month-old infant who was found to have left microphthalmos on routine postnatal checkup. Family history, antenatal history, and systemic evaluation were unremarkable. Magnetic resonance imaging showed absent left optic nerve with left microphthalmos. The optic chiasm was present and slightly deviated towards the right side. The remaining cerebral and ocular structures were normal.