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Cyclopia is a congenital anomaly characterized by the presence of a unique eye in the medial part of the face, which combines the lack of separation of the eyes and the failure of prosencephalic division. In this strange anomaly there is a defective development of the anterior end of the neural tube, which is always associated with brain abnormalities. It is considered the most extreme variable in the holoprosencephaly that is frequently associated with anomalies of the mid line involving the facial region and other systems. There exist a wide variation, from complete Cyclopia, which presents a rudimentary unique eye in the mid line, up to the incomplete Cyclopia, where there are two well-formed ocular globes that are very near in a wide orbit. It arises from the action of different teratogenic factors altering the normal embryonic development and consequently distorting axes determination, and the ocular organogenesis. Among the teratogenic factors the environmental and genetic are cited. At the moment the Cyclopia diagnosis is achieved during intra-uterine life by the ultrasonographic study at precocious stages of the development, during the first trimester of pregnancy. However, the postmortem radiological study of anatomical fetal pieces is also important for the assesment of other malformations. The case of a feminine sex fetus Cyclops is presented, who exhibits in the ventral face region a unique eye, which is located in a rhombic orbital cavity. The lids are fused. Between the superior lip and the unique eye, a depression is observed that delineate the superior maxillary. Simple radiological, tomographic, and magnetic resonance cranio-facial studies were carried out confirming their importance for the detection of other associate anomalies, not only external, but also bony and visceral (Moreno et al., 2008).
Cyclopia is a congenital anomaly characterized by the presence of a unique eye in the medial part of the face, which combines the lack of separation of the eyes and the failure of prosencephalic division. In this strange anomaly there is a defective development of the anterior end of the neural tube, which is always associated with brain abnormalities. It is considered the most extreme variable in the holoprosencephaly that is frequently associated with anomalies of the mid line involving the facial region and other systems. There exist a wide variation, from complete Cyclopia, which presents a rudimentary unique eye in the mid line, up to the incomplete Cyclopia, where there are two well-formed ocular globes that are very near in a wide orbit. It arises from the action of different teratogenic factors altering the normal embryonic development and consequently distorting axes determination, and the ocular organogenesis. Among the teratogenic factors the environmental and genetic are cited. At the moment the Cyclopia diagnosis is achieved during intra-uterine life by the ultrasonographic study at precocious stages of the development, during the first trimester of pregnancy. However, the postmortem radiological study of anatomical fetal pieces is also important for the assesment of other malformations. The case of a feminine sex fetus Cyclops is presented, who exhibits in the ventral face region a unique eye, which is located in a rhombic orbital cavity. The lids are fused. Between the superior lip and the unique eye, a depression is observed that delineate the superior maxillary. Simple radiological, tomographic, and magnetic resonance cranio-facial studies were carried out confirming their importance for the detection of other associate anomalies, not only external, but also bony and visceral (Moreno et al., 2008).
La Ciclopía es una malformación congénita mayor, producto de una secuencia que se inicia con un defecto en la división del cerebro anterior que conlleva a holoprosencefalía alobar y la fusión de los ojos resultando en un ojo central. Se caracteriza por presentar una sola órbita que contiene la estructura ocular, además puede haber al mismo tiempo una estructura cilíndrica que se denomina Proboscis, y usualmente se ubica por encima de la órbita.
Las formas más severas de la holoprosencefalía resultan en severas deformaciones faciales. Estas anomalías incluyen la Ciclopía, la etmocefalia, cebocefalía y labio fisurado Central. La Ciclopía es el fenotipo más severo de la holoprosencefalía, la cual puede dividirse en alobar, donde se incluye la Ciclopía, en semilobar y lobar; nosotros reportamos un caso de holoprosencefalía con Ciclopía con sinoftalmía (Pachajoa y Isaza., 2010)
En la Ciclopía se ha encontrado que los ojos muestran un amplio rango de variación, desde la Ciclopía completa, la cual presenta un ojo único rudimentario en la línea media, con una córnea simple, una pupila y un cristalino sin rasgos de duplicación, hasta la Ciclopía incompleta, donde hay dos globos oculares bien formados, que se encuentran muy cercanos en una órbita amplia. La Ciclopía puede ocurrir por la acción de diferentes factores teratógenos que alteran el desarrollo embrionario normal y en consecuencia afectan los procesos de formación de los patrones para la determinación de los ejes y la organogénesis ocular (Moreno et al., 2008)
Malformación de la línea media con una órbita única o estructuras oculares fusionadas.
Ausencia congénita de estructuras nasales externas, cuya extensión interna debe determinarse anatómicamente.
Puede formar parte de una alteración grave de la organización del prosencéfalo y asociarse a anomalías nasales o mandibulares.
Puede asociarse a otras anomalías craneofaciales; el desarrollo de cavidades y vías aéreas debe evaluarse por separado.
Arrinia y agnatia son hallazgos asociados distintos; la exposición a teratógenos no se confirma por la morfología.
La repercusión respiratoria depende de la conformación real de la vía aérea, no solamente de la apariencia facial.