Triopia: craniofacial malformation with prosencephalic duplication.
A case of ocular duplication with complex craniofacial and central nervous system anomalies is described. The anomaly is termed triopia because the child's most overt and distinguishing feature was three eyes: the left orbit contained two globes with independent ocular adnexa; the right orbit contained one normal appearing and functioning globe. Computer assisted medical imaging was used to define, in vivo, the intra- and extracranial soft and hard tissue anomalies: the cerebral hemisphere ipsilateral to the ocular duplication was also duplicated. Possible bases for this anomaly include duplication of primordia for the eye and secondary prosencephalon.
Stelnicki
E J
EJ
Department of Plastic and Reconstructive Surgery, Washington University School of Medicine, St. Louis, Missouri, USA.
Marsh
J L
JL
Woolsey
T A
TA
Lee
B C
BC
Noetzel
M J
MJ
D000015:Abnormalities, Multiple; D002648:Child; D002675:Child, Preschool; D002971:Cleft Lip / Q000473:pathology; D002972:Cleft Palate / Q000473:pathology; D005124:Eye Abnormalities / Q000473:pathology*; D005143:Eyelids / Q000002:abnormalities; D005147:Facial Bones / Q000002:abnormalities*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009915:Orbit / Q000002:abnormalities; D016548:Prosencephalon / Q000002:abnormalities*; D012886:Skull / Q000002:abnormalities*; D014057:Tomography, X-Ray Computed