Indian Pediatrics 2002; 39:973-974

Cyclopia

 

A full term female baby was born per vaginum to a non consanguineous couple with uneventful antenatal period with very unusual facial features. On examination we found one eye and there was a proboscis instead of nose coming from above the eye (Fig. 1). Mouth cavity was normal and head circumference was within normal limit. There were features of severe birth asphyxia. Resuscitation was done by endotracheal intubation and positive pressure ventilation but the baby died after a few minutes.

Fig. 1. Photograph shows single central eye with proboscis.

Cyclopia is mostly associated with defect in the division of telencephalic vesicle. There may be one large fused hemisphere with a single ventricle or fusion may be restricted to the frontal region so that brain assumes horse shoe appearance with a single central ventricle with fusion of thalami together (alobar holoprocencephaly). Severe cases with cyclopia have one central eye or two eyes obviously fused together. There may be a proboscis instead of nose coming from between the eyes or nose with a single nostril and in others there is cleft palate. Sometimes these cases may be associated with congenital cyanotic heart disease. Most cases are sporadic in origin. However, holoproc-encephaly and cyclopia may be a manifesta-tion of Trisomy 13.

Kolkata, West Bengal, India.