Tala Dakkak

and 3 more

Introduction:Cyclopia (synophthalmia) is a fatal uncommon congenital abnormality.1-3 It is the severest case of holoprosencephaly (HPE).1,3-7 During the organogenesis, the embryonic prosencephalon is divided partially or never into the right and left cerebral hemispheres,2,3which leads to the pilgrims not being divided into double separate caves.1,4,7-9 This happens between the eighteenth and the twenty-eighth day of pregnancy.1,4,7,8 The incidence is 1.05 per 100,000 births including stillbirth1 with a tendency to occur in females8. Clinically, there are typical craniofacial features including a single or imperfectly cleft eye in one orbit, nasal agenesis or proboscis, which is a non-functional nose located above the central orbit and that is known as Rhinocephaly.1-3,6,9,10 Furthermore, the extracranial characteristics along with Cyclopia, for instance, polydactyly, renal dysplasia, Omphalacele1,2,6 also cardiac malformations such as ventricle septum defect (VSD).10 Synophthalmia is possible to develop for no specific reason7 or due to risk factors such as exposure to deformed substances during pregnancy10, diabetes, infections6, and genetic mutations6,10. This case is incompatible with life4,5,7 either abortion or stillbirth or dying several hours after birth.1,3,8,10 Diagnosis began from the 22nd week of pregnancy6 by ultrasound (US).4-7 The most common syndrome associated with Cyclopia is Patau (trisomy 13)3,10. Herein, we present a unique case of alobar holoprosencephaly with cyclopia which was diagnosed after miscarriage.