RESUMEN
A woman developed headaches, transient visual obscurations, anosmia, and decreased visual acuity. Ocular examination showed bilateral pulsatile proptosis and disc edema with choroidal folds. Standardized ophthalmic echography showed absence of bony orbital roofs, prominent dural pulsations, direct apposition of brain parenchyma and orbital tissues, and echographic signs suggesting bilateral optic nerve compression. CT and MRI showed a large defect in the floor of the anterior cranial fossa. The cribriform plate, both orbital roofs, and sphenoid bones were displaced by a large basal encephalocele. Clinical improvement followed reconstruction of the anterior cranial fossa and decompression of both optic nerves.
Asunto(s)
Encéfalo/patología , Encefalocele/complicaciones , Síndromes de Compresión Nerviosa/etiología , Nervio Óptico/patología , Adulto , Encéfalo/cirugía , Enfermedades de la Coroides/diagnóstico , Enfermedades de la Coroides/etiología , Encefalocele/diagnóstico , Encefalocele/cirugía , Exoftalmia/diagnóstico , Exoftalmia/etiología , Femenino , Humanos , Imagen por Resonancia Magnética , Síndromes de Compresión Nerviosa/diagnóstico por imagen , Trastornos del Olfato/diagnóstico , Trastornos del Olfato/etiología , Nervio Óptico/diagnóstico por imagen , Papiledema/diagnóstico , Papiledema/etiología , Tomografía Computarizada por Rayos X , Ultrasonografía , Trastornos de la Visión/diagnóstico , Trastornos de la Visión/etiología , Agudeza VisualRESUMEN
A case of Möbius syndrome was associated with transposition of the aorta and pulmonary artery, as well as acheiria. This combination of anomalies supports the hypothesis that Möbius syndrome is caused by an intrapartum insult during the fourth to seventh week of gestation and is consistent with the vascular theory of embryopathogenesis.
