|IMAGES IN CLINICAL NEONATOLOGY
|Year : 2015 | Volume
| Issue : 1 | Page : 64-65
Manisha Goyal, Seema Kapoor
Department of Pediatrics, Division of Genetics and Metabolism, Maulana Azad Medical College, New Delhi, India
|Date of Web Publication||10-Feb-2015|
Department of Pediatrics, Division of Genetics and Metabolism, Maulana Azad Medical College, New Delhi
Source of Support: None, Conflict of Interest: None
True cyclopia is the alobar type of holoprosencephaly in which the organogenetic development of the anterior brain and the midline mesodermal structures is suppressed. We are presenting a case of true cyclopia.
Keywords: Brain, cyclopia, holoprosencephaly
|How to cite this article:|
Goyal M, Kapoor S. True cyclopia. J Clin Neonatol 2015;4:64-5
| Introduction|| |
Our case was a female born out of second degree consanguineous marriage. She was delivered at term by normal vaginal delivery with birth weight of 1805 grams. The neonate died immediately after birth. Mother had three previous normal pregnancies and deliveries. She had no history of any drug exposure or chronic disease in the pregnancy. Although she had no antenatal checkups, pregnancy progressed with over distension. Ultrasonography showed absence of skull vault with anencephaly, single eye, and replacement of nose with stamp like structure and polyhydramnios at 38 weeks of pregnancy. Mother went into spontaneous labor and delivered a cyclop baby. On examination, a proboscis measuring 22 mm in length and 10 mm in diameter was present at the superior aspect of the face. There was a single median orbit with a well-developed globe partially covered by eyelids present in between mouth and proboscis [Figure 1]. Skull vault was absent with posterior encephalocele and open spinal cord [Figure 2]. The nose was absent. Ears, mouth, palate, and tongue were normal. Hands, feet, and genitalia were normal. Fetal autopsy was not possible due to refusal by parents. Chromosomal studies of blood cell cultures from the cord showed a normal karyotype.
True cyclopia is the most severe alobar type of holoprosencephaly.  It results from arrest of development of the anterior end of the neural plate between the 18 th and the 28 th day of gestation. Alobar means the complete absence of division of the prosencephalon structures, resulting in absent interhemispheric fissure and corpus callosum, fused thalami, fused cerebral hemispheres with only one cerebral ventricle, and facial dysmorphism such as cyclopia, proboscis, ethmocephaly and cebocephaly.  The etiology of cyclopia includes environmental factors (maternal diabetes mellitus, history of maternal alcoholism, in utero infections with cytomegalovirus, rubella or toxoplasma, teratogenic drugs (retinoic acid, cholesterol synthesis inhibitors) and genetic factors (chromosomal, sonic hedgehog gene mutation and syndromic). 
| References|| |
Demyer W, Zeman W, Palmer CG. The face predicts the brain: Diagnostic significance of median facial anomalies for holoprosencephaly (arhinencephaly). Pediatrics 1964;34:256-63.
Poenaru MO, Vilcea ID, Marin A. Holoprosencephaly: Two case reports. Maedica (Buchar) 2012;7:58-62.
Garzozi HJ, Barkay S. Case of true cyclopia. Br J Ophthalmol 1985;69:307-11.
[Figure 1], [Figure 2]