RESUMEN
A significant percentage of children with hemangiomas may have PHACES syndrome which refers to the association of posterior fossa malformations, facial hemangiomas, arterial cerebrovascular abnormalities, cardiovascular anomalies, eye abnormalities and ventral defects like sternal clefting or supraumbilical raphe. A variety of factors have led to under diagnosis of PHACES syndrome in the past including lack of awareness and limited imaging modalities. Also, patients with PHACES syndrome with arterial cerebrovascular abnormalities can present with acute ischemic stroke. However, these patients usually present before one year of age. We describe a 29-year-old woman with no history of cerebrovascular disease who initially presented with symptoms of a stroke and was subsequently diagnosed to have PHACES syndrome exhibiting an array of multiple unusual imaging findings. We also discuss the current literature and recommendations about PHACES syndrome.
Asunto(s)
Coartación Aórtica/patología , Trastornos Cerebrovasculares/patología , Fosa Craneal Posterior/anomalías , Anomalías del Ojo/patología , Hemangioma/patología , Síndromes Neurocutáneos/patología , Adulto , Coartación Aórtica/diagnóstico por imagen , Angiografía Cerebral , Trastornos Cerebrovasculares/diagnóstico por imagen , Fosa Craneal Posterior/diagnóstico por imagen , Imagen de Difusión por Resonancia Magnética , Anomalías del Ojo/diagnóstico por imagen , Femenino , Humanos , Síndromes Neurocutáneos/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
SUMMARY: We describe a 65-year-old woman with an asymptomatic idiopathic lingual artery aneurysm which is suspected to be congenital. We review the literature on external carotid artery branch aneurysms, diagnostic evaluation and discuss treatment options for the various types and the specific chosen in the case presented.
Asunto(s)
Aneurisma/diagnóstico por imagen , Aneurisma/cirugía , Embolectomía/métodos , Tomografía Computarizada por Rayos X/métodos , Lengua/irrigación sanguínea , Lengua/cirugía , Anciano , Embolectomía/instrumentación , Femenino , Humanos , Lengua/diagnóstico por imagen , Resultado del TratamientoRESUMEN
A persistent question in the field of antibody imaging and therapy is whether increased affinity is advantageous for the targeting of tumors. We have addressed this issue by using a manipulatable model system to investigate the impact of affinity and antigen density on antibody localization. In vitro enzyme-linked immunosorbent assays and bead-binding assays were carried out using BSA conjugated with high and low densities (HD and LD, respectively) of the chemical hapten rho-azophenyl-arsonate as an antigen. Isotype-matched monoclonal antibodies (mAbs) 36-65 and 36-71, with identical epitope specificity but 200-fold differences in affinity, were chosen as targeting agents. The relative in vitro binding of 36-65 and 36-71 was compared with an artificial "tumor" model in vivo using antigen-substituted beads s.c. implanted into SCID mice. Nonsubstituted BSA beads were implanted in the contralateral groin as a nonspecific control. The efficacy of the targeting of [125I]-labeled antibodies was assessed by the imaging of animals on a gamma-scintillation camera using quantitative region-of-interest image analysis over the course of 2 weeks and by postmortem tissue counting. In vitro, both antibodies bound well to the HD antigen, whereas only the high-affinity mAb 36-71 bound effectively to the LD antigen. In vivo, high-affinity mAb 36-71 bound appreciably to both LD and HD beads. In contrast, there was no specific localization of low-affinity mAb 36-65 to LD antigen beads, although the antibody did bind to the beads with the HD antigen. Whereas the high-affinity mAb 36-71 increased its binding to HD beads throughout the 14 days of observation, binding of the high affinity antibody to LD beads and of the low affinity antibody to HD beads plateaued between 10-14 days. These in vitro and in vivo findings demonstrate that the need for a high-affinity antibody is dependent on the density of the target antigen. High-affinity antibodies bind effectively even with a single antigen-Fab interaction, irrespective of the antigen density. In contrast, low-affinity antibodies, because of weak individual antigen-Fab interactions, require the avidity conferred by divalent binding for effective attachment, which can only occur if antigen density is above a certain threshold. An understanding of the differential behavior of high- and low-affinity antibodies and the impact of avidity is useful in predicting the binding of monovalent antibody fragments and engineered antibody constructs and underlies the trend toward development of multivalent immunological moieties. Consideration of the relative density of the antigen on the tumor and the background tissues may enable and even favor targeting with low-affinity antibodies in selected situations.