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.
![](images/child_3.jpg)
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.
Nabendu Chowdhury,
Professor, Department of
Pediatrics,
Burdwan Medical College and
Hospital,
Burdwan, West Bengal,
India.
Kaustav Nayek,
R.M.O. Cum Clinical Tutor,
Calcutta Medical College
and Hospital,
Kolkata, West Bengal,
India.
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