Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento / Update on orbital vascular anomalies in pediatrics: imaging studies and management

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Anastomosis between the posterior superior alveolar artery and the infra-orbital artery: a review of current terminology / Anastomosis entre la arteria alveolar superior posterior y la arteria infra-orbital: una revisión de la terminología actual

SUMMARY: The posterior superior alveolar artery (PSAA) and the infra-orbital artery (IOA) present intraosseous and extraosseous rami which form an anastomosis in the lateral wall of the maxillary sinus. This anastomosis is always present, however it has not yet been included in anatomical terminology (AT), and different terms are used in scientific communication to refer to it. The aim of this study was to carry out a review of the different terms used to name this vascular structure. A literature review was carried out on the terms used to name the anastomosis between the PSAA and IOA in imaging studies and human cadavers that assessed the presence/frequency of this anatomical structure. The search was carried out in the Medline, EMBASE and LILACS databases, in Portuguese, Spanish and English, with no date restrictions. Qualitative analysis was applied to the studies selected, analysing the terminology used to refer to the anastomosis between the PSAA and IOA. Of the 2108 original articles found, 60 were selected as potentially relevant and 54 studies were finally included for qualitative analysis. Sixteen terms were found to refer to the anastomosis between the PSAA and IOA, the most frequent being Posterior Superior Alveolar Artery (PSAA), followed by Alveolar Antral Artery (AAA). Many terms are used in the medical literature to designate the anastomosis between the PSAA and IOA, the most frequent being PSAA and AAA. There is a need to unify the terms used to designate this vascular structure, and to incorporate the selected term into anatomical terminology, in order to avoid confusion in scientific communication.

RESUMEN: La arteria alveolar superior posterior (AASP) y la arteria infra-orbital (AIO) tienen ramas intra y extra óseas que forman una anastomosis en la pared lateral del seno maxilar. Esta anastomosis está siempre presente, sin embargo, aún no ha sido incluida en la terminología anatómica (TA), por lo que en la comunicación científica se utilizan diferentes términos para referirse a ella. El objetivo de este estudio fue realizar una revisión sobre los diferentes términos utilizados para nombrar esta estructura vascular. Se realizó una revisión de la literatura sobre los términos utilizados para nombrar la anastomosis entre AASP y AIO en estudios imagenológicos y en cadáveres humanos que evaluaron la presencia/frecuencia de esta estructura anatómica. La búsqueda fue realizada en las bases de datosMedline, EMBASE y LILACS, en los idiomas portugués, español e inglés, sin restricción de fecha. Los estudios seleccionados fueron evaluados de forma cualitativa, analizando la terminología empleada para referirse a la anastomosis entre AASP y AIO. Fueron encontrados 2108 artículos originales, siendo seleccionados 60 artículos potencialmente relevantes y finalmente fueron incluidos 54 estudios para análisis cualitativo. Fueron encontrados 16 términos para referirse a la anastomosis entre AASP y AIO, siendo AASP el más frecuente seguido de arteria alveolo-antral (AAA). Son muchos los términos utilizados en la literatura médica para designar la anastomosis entre AASP y AIO, siendo AASP y AAA los más usados. La unificación de los términos utilizados para designar esta estructura vascular y su incorporación en la Terminología Anatómica contribuiría a evitar equívocos en la comunicación científica.

Radiological management of the exiting points of supraorbital region neurovascular bundles / Manejo radiológico de los puntos de salida de los haces neurovasculares de la región supraorbitaria

SUMMARY: A preoperative computed tomography scan is useful to determine neurovascular exit points from orbit to supraorbital region. Determining the structure of exiting points (absence or presence, if present, being in form of foramen or notch) is important to plan the surgical approach. The aim of the study was to provide the radiological data by multi-detector computed tomography for estimating exiting points of the neurovascular bundles of the supraorbital region whether through foramen or notch in living subjects related to side (right/left), sex and age. Computed tomography examinations of 214 (102 male and 112 female) adult patients, aged average 44.2 ± 14 years, were evaluated, retrospectively. Presence or absence, number and nature (foramen/notch) of exiting points of neurovascular bundles were noted in each side regarding sex and age groups. The distance of foramen/notch to the midline of the face was recorded. Single notch was seen on the right in 123 and in 134 on the left, single foramen was seen in 62 on the right and in 56 on the left side and double foramen was seen in 13 on the right and in 6 on the left. The absence was seen in 16 on the right and 18 on the left side. No significant difference was seen on frequency compared between the sexes and age groups. Foramen was seen in 58 sides unilaterally and in 39 sides bilaterally. Notch was unilateral in 75 sides and bilateral in 95 sides. It was shown that males had a wider distance between right side foramen and left side notch to midline. Age groups did not show a significant difference in terms of side. Absence and foramen presence made up about 30-40 % of cases. Notch was the most common form. Foramen/notch presence was statistically unaffected by the sex and age factors. In terms of surgery, preoperative assessment of orbital exit points with computed tomography is essential.

RESUMEN: Una tomografía computarizada preoperatoria es útil para determinar los puntos de salida neurovascular en la región supraorbitaria. Para la planificación del abordaje quirúrgico es importante determinar la estructura de los puntos de salida (ausencia o presencia en forma de foramen). El objetivo de este estudio fue proporcionar los datos radiológicos mediante tomografía computarizada de detectores múltiples, para estimar los puntos de salida de los haces neurovasculares de la región supraorbitaria, ya sea a través del foramen o incisura en sujetos vivos relacionados con lado (derecho/izquierdo), sexo y edad. Se evaluaron retrospectivamente los exámenes de tomografía computarizada de 214 adultos (102 hombres y 112 mujeres), edad 44,2 ± 14 años. Se observó, en cada lado, presencia o ausencia, número y naturaleza (foramen / incisura) de los puntos de salida de los haces neurovasculares en cuanto a sexo y grupos de edad. Se registró la distancia del foramen / incisura al plano mediano de la cara. Se observó un foramen a la derecha en 123 de las tomografìas y en 134 a la izquierda, se observó un foramen simple en 62 a la derecha y en 56 en el lado izquierdo y se visualizó forámenes doble en 13 tomografías a la derecha y en 6 a la izquierda. Se observó ausencia en 16 casos a la derecha y 18 casos a la izquierda. No existió diferencia significativa en la frecuencia comparada entre los sexos y los grupos etarios. El foramen se detectó en 58 lados unilateralmente y en 39 lados bilateralmente. Se demostró que los hombres tenían una distancia mayor entre el foramen del lado derecho y el foramen del lado izquierdo hasta el pno mediano. No se observó una diferencia significativa en los diferentes grupos etarios en términos de lado. La ausencia y la presencia de los forámenes constituían alrededor del 30-40 % de los casos. Los factores de sexo y edad no afectaron estadísticamente la presencia del foramen / incisura. En términos de cirugía, la evaluación preoperatoria de los puntos de salida orbitales con tomografía computarizada es esencial.

Enoftalmos unilateral por várice orbitaria / Enophthalmos secondary to an orbital varix

El enoftalmos es un motivo de consulta poco frecuente en la práctica médica. La mayor parte de los casos se debe a un aumento de la cavidad ósea orbitaria de origen traumático. Presentamos el caso de una mujer de 63 años que consultó por enoftalmos progresivo de su ojo izquierdo, de doce años de evolución. El examen oftalmológico reveló la presencia de un marcado enoftalmos del lado izquierdo, que mejoraba notablemente con maniobras de Valsalva. Una tomografía computarizada y una resonancia magnética de la órbita permitieron diagnosticar una várice orbitaria no complicada. Las várices orbitarias son infrecuentes y por lo general se manifiestan por episodios de exoftalmos intermitente. Sin embargo, en raras ocasiones, la distensión y colapso repetidos pueden llevar a un enoftalmos progresivo por atrofia de la grasa orbitaria.

The enophthalmos is a rare cause of consultation in medical practice. The majority of cases are due to orbital space enlargement of traumatic origin.We report the case of a 63 year old patient with progressive enophthalmos of the left eye, with twelve years of evolution. Ophthalmologic examination revealed the presence of a marked enophthalmos on the left side, which improved significantly with Valsalva maneuvers. A CT scan and an MRI of the orbit allowed diagnosing uncomplicated orbital varices. Orbital varices are rare and usually manifest by episodes of intermittent exophthalmos. However, on rare occasions, repeated distension and collapse can lead to progressive enophthalmos by orbital fat atrophy.

Centralização cerebral materna na pré-eclâmpsia: revisão sistemática / Maternal cerebral centralization in pre-eclampsia: systematic review

A pré-eclâmpsia é uma doença de alta morbimortalidade com grande impacto na saúde pública. Ainda não há métodos efetivos para seu rastreamento e também não foi elucidada a sua fisiopatologia. O objetivo do presente estudo foi analisar artigos na literatura que avaliaram a possibilidade do fenômeno de "centralização cerebral materna" por meio do Doppler da artéria oftálmica em gestantes normais e gestantes com pré-eclâmpsia. A revisão sistemática da literatura envolveu artigos indexados das bases de dados Medline via PubMed e Biblioteca Virtual em Saúde (BVS), publicados entre 1989 a 2011. Por meio da estratégia de busca, localizaram-se 540 artigos, dos quais 505 foram obtidos na base de dados Medline e 35 na BVS. Selecionaram-se 16 artigos, sendo que 5 deles fazem referência a gestantes normais, 1 a gestante com pré-eclâmpsia e 10 comparam gestantes normais com pré-eclâmpsia. Em conclusão, os estudos encontrados indicam que gestantes com pré-eclâmpsia apresentam vasodilatação com hiperfluxo da artéria oftálmica, o que pode caracterizar um fenômeno de centralização cerebral materna

Pre-eclampsia is a high morbidity disease, with a significant impact in public health. There are no effective methods, as yet, to track this disease, and the pathophysiology has not yet been made clear. The objective of this study was to analyze articles found in the literature, evaluating the possibility of pregnant women presenting with the maternal cerebral centralization phenomenon, through Doppler imaging of the ophthalmic artery in women with normal pregnancy and those presenting with signs of pre-eclampsia. A systematic review of the literature involved articles found in the Medline (PubMed) database and in the Biblioteca Virtual em Saúde (BVS - Virtual Library on Health), published between 1989 and 2011. The search strategy employed by the author yielded 540 articles, of which 505 came from the Medline database and 35 from the BVS. Therefore, 16 articles were selected for this study, being 5 of them refer to normal pregnancies, 1 to pregnancies in which the woman presented with pre-eclampsia and 10 compared normal pregnancies with pregnant women presenting with pre-eclampsia. To sum up, the studies on this topic indicate that pregnant women exhibiting pre-eclampsia symptoms present with vasodilation with hyperflow in the ophthalmic artery, which can be characterized a maternal cerebral centralization phenomenon

Orbital Doppler Evaluation of Blood Flow Velocities in Optic Neuritis

PURPOSE: To evaluate orbital blood flow velocities and optic nerve diameter with Doppler and gray-scale sonography in patients with acute unilateral optic neuritis (ON). METHODS: Orbital Doppler and gray-scale sonography was performed in 46 eyes of 23 patients aged 19- to 47-years with acute unilateral ON. ON was diagnosed by an ophthalmologist on the basis of clinical presentation, presence of decreased visual acuity and assessment of visual evoked potentials. The peak systolic velocity (PSV) and end-diastolic velocity (EDV), as well as the resistance index (RI) and pulsatile index (PI) of the ophthalmic artery (OA), central retinal artery (CRA), posterior ciliary arteries (PCAs) and optic nerve diameter were measured in both eyes. We compared results from affected and unaffected eyes using the paired t-test. The area under the receiver operating characteristic (ROC) curves was used to assess the diagnosis of ON based on measured blood flow parameters of the OA, CRA and PCAs and optic nerve diameter. RESULTS: The mean (standard deviation) optic nerve diameter in eyes with ON was 4.1 (0.8) mm, which was significantly larger than the 3.0 (0.4) mm diameter measured in unaffected control eyes (p 0.05). The mean RI in the PCAs was slightly lower in the eyes with ON than in the contralateral eyes (0.60 vs. 0.64, p < 0.05). The area under the ROC curves indicated that optic nerve diameter was the best parameter for the diagnosis of ON. CONCLUSIONS: Optic nerve diameter was related to ON, but orbital blood flow parameters were not.

Reversal in the Diameter of the Superior Ophthalmic Vein after an Epidural Blood Patch in a Case of Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) is caused by single or multiple cerebrospinal fluid (CSF) leaks in the spine with the prototypical symptom of postural headache. One of the characteristic MRI features in SIH is intracranial venous engorgement. This report presents a case of SIH with engorgement of the bilateral superior ophthalmic veins (SOVs) which resume their normal diameters by the third day of successful epidural blood patches (EBPs). We define this phenomenon as the "reversal of the SOV" sign.

Color Doppler imaging of the superior ophthalmic vein in patients with Graves' orbitopathy before and after treatment of congestive disease

OBJECTIVE: To compare superior ophthalmic vein blood flow parameters measured with color Doppler imaging in patients with congestive Graves' orbitopathy before and after treatment and in normal controls. METHODS: Twenty-two orbits from 12 patients with Graves' orbitopathy in the congestive stage and 32 orbits from 16 normal controls underwent color Doppler imaging studies. Color Doppler imaging was repeated after treatment in the group of patients with Graves' orbitopathy, which included orbital decompression in 16 orbits and corticosteroids in six orbits. The findings for each group were compared. RESULTS: In the group of orbits with congestive disease, superior ophthalmic vein flow was detected in 17 orbits (anteroposteriorally in 13 and in the opposite direction in four) and was undetectable in five. After treatment, superior ophthalmic vein flow was detected and anteroposterior in 21 and undetected in one orbit. In normals, superior ophthalmic vein flow was detected anteroposterior in 29 orbits and undetectable in three orbits, indicating a significant difference between groups. There was also a significant difference between controls and congestive Graves' orbits and between congestive orbits before and after treatment, but not between controls and patients after treatment. A comparison of superior ophthalmic vein flow parameters revealed a significant difference between the groups. The superior ophthalmic vein flow was significantly reduced in the congestive stage compared with the flow parameters following treatment and in the untreated controls. CONCLUSIONS: Superior ophthalmic vein flow was significantly reduced in the orbits affected with congestive Graves' orbitopathy and returned to normal following treatment. Congestion appears to be a contributing pathogenic factor in the active inflammatory stage of Graves' orbitopathy.

Pulsating enophthalmos in association with an orbital varix.

We report a case of pulsating enophthalmos secondary to orbital varix associated with orbital bony defects. A 64-year-old female with pulsating enophthalmos of the right eye was found to have a right orbital mass with bony defects of the orbit. Valsalva maneuver failed to induce proptosis. The diagnosis of orbital varix was confirmed by exploratory orbitotomy. During general anesthesia for orbitotomy, proptosis of the right eye was noted. Ophthalmologists should be aware of the association between orbital varices and cranial bony defects and encephaloceles. Proptosis induced by general anesthesia and positive pressure ventilation suggests an underlying distensible venous anomaly.

Avaliação da artéria etmoidal anterior pela tomografia computadorizada no plano coronal / Anterior ethmoidal artery evaluation on coronal CT scans

O conhecimento da localização da artéria etmoidal anterior (AEA) constitui etapa importante na cirurgia do recesso do seio frontal e do etmóide anterior. A tomografia computadorizada (TC), em especial no plano coronal pode fornecer reparos anatômicos que identificam o trajeto da AEA. Objetivo: Identificar os reparos anatômicos que caracterizamo trajeto da AEA na parede medial da órbita e na parede lateral da fossa olfatória. Verificar a correlação entre a presença de pneumatização supra-orbitária e a visualização do etmoidal anterior (canal da AEA). Casuística e Métodos: Estudo retrospectivo de 198 tomografias computadorizadas dos seios paranasais no plano coronal do período de agosto a dezembro de 2006. Resultados: Pneumatização supra-orbitária foi identificada em 35% (70 exames). O canal da AEA foi caracterizado em 41% (81 exames). O sulco etmoidal anterior foi visualizado em 98% (194 dos exames) e o forameetmoidal anterior foi identificado em todos os exames (100%). Conclusão: O forame etmoidal anterior e o sulco etmoidal anterior foram referências anatômicas presentes em quase 100% dos exames avaliados. Houve correlação entre a presença de pneumatização supra-orbitária e a caracterização do canal da AEA.

The anterior ethmoidal artery (AEA) is an important point for frontal and ethmoid sinuses surgery. CT scans can identify landmarks to help the surgeon find the AEA. Aim: To identify the landmarks of the AEA on the orbital medial wall and on the lateral wall of the olfactory fossa. and to correlate the presence of supraorbital ethmoidal cells with spotting the anterior ethmoidal artery canal. Materials and Methods: Retrospective review of 198 direct coronal paranasal sinuses computed tomography (CT) scans from August to December, 2006. Results: Supraorbital pneumatization was seen in 35% (70 scans). The AEA canal was seen in 41% (81 scans). The anterior ethmoidal sulcus was seen in 98% (194 scans) and the anterior ethmoidal foramen was seen in all the scans (100%). Conclusion: The anterior ethmoidal foramen and the anterior ethmoidal sulcus were anatomical landmarks present in almost 100% of the scans studied. There was a correlation between the presence of supraorbital pneumatization and AEA canal visualization.

Intraorbital arteriovenous malformation treated by transcatheter embolization; a case report

To report the clinical and radiological findings and management of a patient with intraorbital arteriovenous malformation [AVM] treated by transcatheter embolization of the feeding artery. A 15-year-old female patient was referred with a one year history of left eye proptosis without prior trauma. Orbital CT scan and MRI demonstrated a large intraconal mass lesion extending to the extraconal space in the inferior orbit; angiograms revealed an intraorbital AVM. Superselective catheterization of the feeding artery and embolization with absorbable gelatin particles [Gelfoam] and non-absorbable polyvinyl alcohol particles were performed in two separate sessions followed by surgical debulking. Signs and symptoms were diminished after 14 months. lntraorbital AVMs can be treated by embolization of the feeding artery followed by surgical removal of the AVM nidus when the lesion is accessible

Dopplervelocimetria colorida dos vasos orbitais: técnica de exame e anatomia vascular normal / Color dopplervelocimetry of orbital vessels: technique and normal vascular anatomy

A dopplervelocimetria colorida é uma modalidade do exame ultra-sonográfico que se expandiu rapidamente em todas as áreas da medicina, em virtude da sua riqueza de informações. A dopplervelocimetria colorida das artérias orbitais tem sido empregada no auxílio diagnóstico de doenças oftalmológicas, bem como para o estudo de doenças específicas como a pré-eclâmpsia. O objetivo deste estudo é descrever a anatomia normal da região orbital e detalhar a técnica de exame dopplervelocimétrico colorido, tendo em vista ser este um exame acessível e reprodutível. Acreditamos que surgirão novas aplicações deste método, que deverá ser inserido na prática diária do radiologista, visando à melhoria da qualidade do diagnóstico e seguimento das doenças oculares e sistêmicas.

Color dopplervelocimetry is an ultrasound modality that has rapidly expanded to all medical areas due to the large amount of information that can be obtained. Orbital color dopplervelocimetry has been used for the diagnosis of ophthalmic disorders and to study specific diseases such as preeclampsia. The aim of this study is to describe the normal anatomy of the orbit and the technical details of color dopplervelocimetry, since this is an accessible and reproducible technique. We believe that new applications for this technique will emerge and that it will be introduced in the daily practice of radiologists to improve the quality of diagnosis and follow-up of ocular and systemic diseases.

Effects of vascular endothelial cell growth factor on fibrovascular ingrowth into rabbits hydroxyapatite orbital implant / 华中科技大学学报（医学）（英德文版）

The effects of different concentrations of vascular endothelial cell growth factor (VEGF) on the fibrovascular ingrowth into rabbits hydroxyapatite orbital implant were investigated. Twelve New Zealand white rabbits were divided into 3 groups and received hydroxyapatite orbital implant surgery in their right eyes. Before and after the operation, the implants were treated with 10 ng/ml VEGF, 100 ng/ml VEGF, or normal saline as control group. The animals received technetium bones scan at 2, 4, and 6 weeks postoperatively. The mean radioactivity counts within region of interest (ROI) of the surgery eye (R) and the non-surgery eye (L) in the same animal were tested, and the R/L ratios were calculated. The implants were harvested at 6th weeks and examined histopathologically. The results showed that at second week, there was no significant difference in mean R/L ratios between VEGF group and control group (F=2.83, P=0.111); At 4th week (F=7.728, P=0.011) and 6th week (F=7.831, P=0.011) postoperatively, the mean ratios in VEGF groups were significantly higher than that in control group. At 6th week postoperatively, the fibrovascularization rates in VEGF groups were higher than in control group significantly (F=8.711, P=0.008). It was suggested that VEGF could promote the fibrovascular ingrowth into hydroxyapatite orbital implant, thus might shorten the time required for complete vascularization of the HA orbital implant.

Sinus pericranii in the frontal region: a case report.

Sinus pericranii is a rare vascular anomaly. A case of sinus pericranii at the nasion with an orbital extension is presented. The drainage was into the superior sagittal sinus. The pathogenesis is discussed and the literature is reviewed.

Eco doppler color en oftalmología / Color doppler echography in ophthalmology

Es posible obtener resultados reproducibles en varios vasos orbitarios con EDC. La interpretación de los resultados debe efectuarse en base al conocimiento del principio Doppler y bases hemodinámicas de la circulación orbitaria. Se han entregado las herramientas para un adecuado examen y así obtener resultados confiables. El método es aplicable a un sinnúmero de patologías oculares y se está transformando en una herramienta muy importante para el clínico e investigadores, y será posiblemente un examen de rutina en un futuro próximo para los oftalmólogos

Proptose intermitente associada a sinus pericranii: diagnóstico diferencial de varizes orbitrárias / Intermitent proptosis associated with sinus pericranii: diferential diagnosis of orbital varices

Um caso de Sinus Pericranii associado a uma malformação do seio sagital superior, na qual o lobo frontal apresenta sua drenagem venosa para o exterior do crânio através do próprio Sinus Pericranii, originando varizes orbitárias. O diagnóstico preciso foi obtido pelo estudo angiográfico e o tratamento foi conservador devido ao risco cirúrgico. Foi feita uma breve revisão da literatura sobre Sinus Pericranii.

Ocular toxicity of tamoxifen.

Tamoxifen is an antioestrogen drug used widely in the management of oestrogen-dependent metastatic breast carcinoma. A number of ocular complications have been described secondary to tamoxifen therapy. We report two patients, one of whom had superior ophthalmic vein thrombosis and the other who had painful proptosis and acute angle-closure glaucoma with choroidal detachment secondary to tamoxifen therapy, both of which have not been reported earlier. In both patients the signs and symptoms resolved rapidly after the discontinuation of tamoxifen therapy. Awareness of the ocular toxicity of tamoxifen is essential as prompt withdrawal can result in resolution of most of the complications.