Intravitreal bevacizumab versus ranibizumab for the treatment of retinal angiomatous proliferation.

PURPOSE: To evaluate the effects of intravitreal bevacizumab and ranibizumab treatments in retinal angiomatous proliferation (RAP). METHODS: Fifty patients affected by RAP were randomly assigned either to intravitreal bevacizumab injection (IVBI) or intravitreal ranibizumab injection (IVRI). After a loading phase including three consecutive monthly injections, the retreatment was administered in cases of persistent RAP. The primary outcome measures were the mean changes in BCVA between the two treatment groups, and the proportion of eyes gaining 1 and 3 lines at the end of the follow-up. Secondary outcomes included central macular thickness (CMT) changes and progression to more advanced stages of RAP. RESULTS: Fifty patients affected by stage 1 and 2 RAP were recruited. Twenty-six and 24 patients received IVBI and IVRI, respectively. At the baseline, mean best corrected visual acuity (BCVA) values were 0.59 ± 0.21 (LogMAR ± SD, approximately corresponding to 20/80 Snellen Equivalent-SE) in IVBI group and 0.66 ± 0.33 (approximately 20/90 SE) in IVRI group with no statistical difference. At 12-month examination, both groups showed a statistically significant improvement in the BCVA, with a final mean value of 0.43 ± 0.24 (approximately 20/54 SE) in IVBI group and 0.50 ± 0.32 (approximately 20/63 SE) in the IVRI group. A BCVA gain of 1 and 3 lines was registered in 20 and 8 eyes, respectively, in the IVBI group. Similarly, 17 and 7 eyes showed an improvement of 1 or 3 lines, respectively, in the IVRI group. The CMT reduced significantly from baseline to 12-month examination in both groups. A lower proportion of eyes with complete pigment epithelium detachment resolution was noted in the IVBI group than in the IVRI group (40% versus 90%). CONCLUSIONS: Our study shows that both IVBI and IVRI are equally effective in improving the BCVA over a 1-year follow-up in eyes affected by stage 1 and 2 RAP.

Angiographic and OCT features of retinal angiomatous proliferation.

BACKGROUND: To propose a classification system for retinal angiomatous proliferation (RAP) on the basis of the indocyanine green angiography (ICG). METHODS: Retrospective chart review of 55 eyes of 55 patients presenting with RAP. Fluorescein angiography (FA), ICG and optical coherence tomography (OCT) were used to evaluate the patients. RESULTS: All RAP lesions appeared as occult or minimally classic CNV on FA without clear evidence of pigment epithelium detachment (PED). We were able to identify five different patterns of RAP on the basis of ICG. These were focal (27.2%), irregular (21.8%), circular (21.8%), multifocal (18.2%), and combined (10.9%) hyperfluorescence. The sudden termination of retinal vessel course sign was observed in 14 of 55 eyes (25.4%), which had a circular or irregular pattern on the ICG. Only the circular RAP exhibited a late hypofluorescence ('wash out') with staining of the surrounding tissue on the ICG. Forty eight of 55 eyes (86%) had PED according to the OCT. Out of these 48 eyes, 19 had intraretinal fluid (IRF) alone, and the rest had IRF and subretinal fluid. The eight eyes (14%) without PED belonged to the focal hyperfluorescence group and the fluid was located intraretinally in cystic spaces. In addition, in four eyes (7%) with coexisting CNV a band of tissue beneath the RAP protruding in the PED was observed. CONCLUSION: We propose a classification system for RAP on the basis of ICG and present the angiographic and OCT findings of these lesions. These data may further aid in the early diagnosis of RAP and can be also used for prognosis and clinical course documentation.

Retinal angiomatous proliferation: morphological changes assessed by Stratus and Cirrus OCT.

BACKGROUND AND OBJECTIVE: Spectral domain optical coherence tomography (SD-OCT) should be used to examine lesions containing retinal angiomatous proliferation (RAP) to achieve a better understanding of the origin of this pathology. PATIENTS AND METHODS: In this prospective, observational case series, patients with RAP underwent retinal thickness imaging with Stratus OCT and cube 200 X 200 imaging with Cirrus OCT. RESULTS: A total of 12 eyes from 11 patients were included in the study. Of these, eight eyes had stage III RAP and four eyes had stage II RAP. Cirrus OCT detected the hyperreflective area corresponding to the neovascularization in all 12 eyes and Stratus OCT detected this pathology in 10 of the 12 eyes. All stage III lesions contained a retinal pigment epithelial break, which was more visible in the 200 x 200 scans obtained from Cirrus OCT compared with six radial scan lines obtained from Stratus OCT. CONCLUSION: Cirrus OCT had considerable advantages compared with Stratus OCT, particularly in investigation of juxtafoveal pathology.

Efficacy of intravitreal bevacizumab to treat retinal angiomatous proliferation stage II and III.

PURPOSE: To evaluate the efficacy of intravitreal bevacizumab to treat retinal angiomatous proliferation (RAP) stages II and III. METHODS: A retrospective, interventional, nonrandomized multicentric study was performed. The files, optical coherence tomography (OCT) scans, indocyanine green, and fluorescein angiograms of patients with RAP stages II and III who had been treated by intravitreal bevacizumab were retrospectively examined. Final visual acuity, number of injections, and appearance of adverse events were considered as main outcome indicators. RESULTS: Twenty-six eyes from 24 patients (9 male and 15 female) were treated by intravitreal bevacizumab. Fourteen eyes presented RAP stage II and 12 eyes presented RAP stage III. Mean age was 76+/-9 and 79+/-6 years, respectively. Mean initial best-corrected visual acuity (BCVA) was logMAR 0.60+/-0.24 and 1.13+/-0.37, respectively. Mean BCVA was 0.62+/-0.26 and 1.06+/-0.37, respectively, at 6 months (p=0.96 and 0.10, respectively, Student t test for paired data) and 0.63+/-0.26 and 1.04+/-0.37, respectively, at 12 months (p=0.82 and p=0.06, respectively, Student t test for paired data). The average number of injections during the first year was 3.4 and 3.2, respectively. CONCLUSIONS: Intravitreal bevacizumab may stabilize visual acuity during the first year in RAP lesion stage II and III. Visual prognosis seems to be better in RAP II lesions.

[Vascular malformations (I). Concept, classification, pathogenesis and clinical features]. / Malformaciones vasculares (I). Concepto, clasificación, fisiopatogenia y manifestaciones clínicas.

Vascular malformations are anomalies always present at birth that, contrary to hemangiomas, never regress and may grow during lifetime. Clinical presentation of vascular malformations is extremely variable and ranges from asymptomatic spots of mere aesthetic concern to lesions with high blood flow or located in critical sites that may be life-threatening. Given the low incidence of these disorders it is difficult to establish therapeutic guidelines. In addition to a correct classification of vascular anomalies, it is necessary a multidisciplinary approach for the follow-up and management of these patients. The first part of this review focuses on the different classifications of vascular anomalies, maintaining as reference the one proposed by the International Society for the Study of Vascular Anomalies (ISSVA). Additionally, clinical features of the different subtypes of vascular anomalies as well as their association in certain syndromes are reviewed.

Endothelial lesions of soft tissues: a review of reactive and neoplastic entities with emphasis on low-grade malignant ("borderline") vascular tumors.

Soft tissue proliferations composed of endothelial cells are a heterogeneous group of lesions that can cause diagnostic difficulty. Further complicating the issue is the constantly changing nomenclature of some entities, as well as reclassification of some vascular tumors from the high-grade malignant category to the low-grade malignant or borderline category. Modern ancillary techniques such as immunohistochemistry and cytogenetics have done little to advance our knowledge of these lesions. This review article outlines the most recent classification of endothelial lesions of the skin and soft tissues, with emphasis on the low-grade malignant (borderline) category. In addition, many tumor-like lesions containing an endothelial component are also discussed.

[Not all vascular birthmarks are angiomas]. / No todas las manchas vasculares son angiomas.

Vascular anomalies are extremely common but for many years have been poorly understood, partly due to the complex and colorful terminology used, which is full of descriptive terms tinged with folklore. While many lesions look the same, they have a very different prognosis and require very different treatment. Therefore, it is essential to be precise when using the nomenclature. This article reviews the current classification of vascular anomalies and describes recent advances in their diagnosis and management. Our aim is to replace the old confusing terminology and contribute to the unification of concepts and terms. At the same time we stress the importance of interdisciplinary collaboration in order to offer patients accessible and integral treatment.

Angiomatosis retinae: (Enfermedad de Von Hippel) / Angiomatosis of the retina (Von Hippel Disease)

La angiomatosis de la retina ó Enfermedad de Von Hippel, es una patología que consiste en la presencia de un hamartoma angiomatoso que afecta al nervio óptico, retina o ambos, además de la posibilidad de afectación del Sistema Nervioso Central (SNC) y otros órganos, por lo cual entra dentro del grupo de las facomatosis. El siguiente es un reporte de un caso evaluado multidisciplinariamente en el Hospital Militar "Dr Carlos Arvelo" para descartar compromiso de un caso de Angiomatosis Retiniana referido a nuestro servicio

Hamartoma melanocítico dérmico y facomatosis pigmento-vascular / Dermal melanocytic hamartona and pigmentary-vascular phacomatosis

El Hamartoma Melanocítico Dérmico es una displasia névica, caracterizada por la ubicación ectópica de melanocitos en dermis, en forma extensiva en napa y persistente. Es un cuadro de rara observación. Se comunican dos casos, uno de ellos asociado con una angiomatosis profusa, constituyendo lo que se conoce como Facomatosis Pigmento-vascular. Esta es una entidad malformativa variada donde, además de nevos melanocíticos, pigmentarios y vasculares, pueden existir malformaciones diversas

Hamartoma melanocítico dérmico y facomatosis pigmento-vascular / Dermal melanocytic hamartona and pigmentary-vascular phacomatosis

El Hamartoma Melanocítico Dérmico es una displasia névica, caracterizada por la ubicación ectópica de melanocitos en dermis, en forma extensiva en napa y persistente. Es un cuadro de rara observación. Se comunican dos casos, uno de ellos asociado con una angiomatosis profusa, constituyendo lo que se conoce como Facomatosis Pigmento-vascular. Esta es una entidad malformativa variada donde, además de nevos melanocíticos, pigmentarios y vasculares, pueden existir malformaciones diversas

Reassessment of malignant "angioendotheliomatosis". Evidence in favor of its reclassification as "intravascular lymphomatosis".

Malignant angioendotheliomatosis (MAE) is a lethal intravascular proliferation which has been thought to be of endothelial origin. In order to characterize its cellular nature, we studied 15 cases of MAE immunocytochemically, using antisera for factor VIII-related antigen, cytokeratin, epithelial membrane antigen, vimentin, blood group isoantigens, thoracic duct lining cell antigens (TDLCA), common leukocyte antigen, and Ulex europaeus I lectin. In 14 of 15 cases, common leukocyte antigen was observed in malignant intravascular cells. Similar reactivity for factor VIII-related antigen was present in 14 cases, but was largely restricted to cells enmeshed in fibrin-platelet thrombi, and probably represents adsorption of platelet-derived factor VIII by tumor cells. All cases failed to bind Ulex europaeus lectin and lacked immunoreactivity for TDLCA, cytokeratin, epithelial membrane antigen, and blood group isoantigens; two manifested positivity for vimentin. Immunofluorescent microscopy of frozen tissue in one case showed monoclonal IgM-kappa immunoglobulin on the surfaces of tumor cells. Electron-microscopic study of three cases disclosed a predominant cell type lacking features of epithelial or endothelial differentiation; a minor cell population displayed endothelial characteristics and was thought to be reactive. Four patients with typical MAE also had extravascular large-cell lymphoma in lymph nodes, spleen, adrenal glands, stomach, or soft tissues. Six patients showed clinical evidence of autoimmune disease. These results suggest that MAE displays lymphoid rather than endothelial differentiation.

[Classification of angiodysplasias and vascular tumors]. / Classification des angiodysplasies et tumeurs vasculaires.

Numerous vascular dysplasiae belong to the group of genodysplasiae. Most arteriovenous dysplasiae (cirsoid, racemosum aneurysms,...) are the substratum of various regional "angiomatosis". Stenotic or ectatic arterial dysplasiae can be associated with genodysplasiae. In addition, lymphatic dysplasiae (congenital elephantiasis, etc...) also exist. Among benign tumours, angiomas are sometimes hardly distinguishable from dysplasiae. Some tumors with intermediate malignancy have an uncertain prognosis: chemodectoma, hemangiopericytoma. As for malignant vascular tumours, they fall into 3 varieties: angiosarcoma, Kaposi's sarcoma and sarcoma of vascular walls.

[Phacomatoses. Pathogenesis - Classification - Vascular aspects]. / Les phacomatoses. Pathogénie - Classification - Aspects vasculaires.

The pathogenesis of the phacomatoses, developmental diseases of the embryonic plates, permits an understanding of the different manifestations which characterize these disorders. This pathogenesis also constitutes the best basis for a rational classification. The author sets out the main features of this pathogenesis and its practical applications, and then considers the principal vascular aspects of the phacomatoses, especially in Osler-Rendu disease, Blue Rubber Bleb Naevi, Mafussi's syndrome, the haemangioblastomatoses, Bailey's glomangiomatosis, the Louis-Bar syndrome, Struge-Weber angiomatosis, the syndrome of Bonnet-Dechaume and Blanc, Cobb's syndrome, the angio-osteo-hypertrophic syndromes, von Recklinghausen's neurofibromatosis, Bourneville's tuberous sclerosis, and the melanic phacomatoses.

[General classification and limited nosology of the phacomatoses]. / Classification générale et limites nosologiques des phacomatoses

The authors suggest a new classification of phacomatoses based on the dysembryoplastic concept of these disorders, which are blastomas of the germ layers. Three main groups are described : ectoblastic, mesoblastic and entoblastic phacomatoses. The diffuse forms are often familial and very evolutive and are contrasted with the regional forms which are commonly sporadic and not very evolutive. The limits of the phacomatoses are finally specified.