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.
AbbottU.K., PisentiJ.M.Making the connection: exploring classical concepts in normal and abnormal limb development using contemporary approaches.Prog Clin Biol Res1993; 383: 99–112.
2.
BagherianV., GrahamM., GersonL.P., ArmstrongD.L.Double pituitary glands with partial duplication of facial and fore brain structures with hydrocephalus.Comput Radiol1984; 8: 203–210.
3.
ChoK.W., De RobertisE.M.Differential activation of Xenopus homeobox genes by mesoderm inducing growth factor and retinoic acid.Genes Dev1990; 4: 1910–1916.
4.
CowanW.M.Anterograde and retrograde transneuronal degeneration in the central and peripheral nervous system. In: NautaW.J.H., EbessonS.O.E. eds. Contemporary research methods in neuroanatomy.New York: Springer, 1970; 217–251.
5.
DuesterG.A hypothetical mechanism for fetal alcohol syndrome involving ethanol inhibition of retinoic acid synthesis at the alcohol dehydrogenase step.Alcohol Clin Exp Res1991; 15: 568–572.
6.
FawcettJ.W., O'LearyD.D.M.The role of electrical activity in the formation of topographic maps in the nervous system.Trends Neurosci1985; 8: 201–206.
7.
GillandE., BakerR.Conservation of neuroepithelial and mesodermal segments in the embryonic vertebrate head.Acta Anat (Basel)1993; 148: 110–123.
8.
HarveyS.C., BurrH.S.The development of the meninges.Arch Neurol Psychiat1926; 15: 545–567.
9.
JooreJ., van der LansG.B., LanserP.H., VervaartJ.M., ZivkovicD.Speksnijder JE, Kruijer W. Effects of retinoic acid on the expression of retinoic acid receptors during zebrafish embryogenesis.Mech Dev1994; 46: 137–150.
10.
KeibelF.The development of the sense organs. In: Keibel F, Mall FP. Manual of human embryology. Vol. II.Philadelphia: JB Lippincott,1912; 180–264.
11.
LawM.I., Constantine PatonM.Anatomy and physiology of experimentally produced striped tecta.J Neurosci1981; 7: 741–759.
12.
MadenM., HolderN.Retinoic acid and development of the central nervous system.Bioessays1992; 14: 431–438.
13.
MannI.Developmental abnormalities of the eye.Philadelphia: Lippincott,1957; 42–59.
14.
MarshallH., StuderM., PopperlH., AparicioS., KuroiwaA., BrennerS., KrumlaufR.A conserved retinoic acid response element required for early expression of the homeobox gene Hoxb-1.Nature1994; 370: 567–571.
15.
MettlerF.A.Congenital malformation of the brain.J Neuropath Exp Neurol1947; 6: 98–110.
16.
MinkoffR.Cell proliferation and migration during primary palate development. In: PrattR.M., ChristiansenR.L. eds. Current research trends in prenatal craniofacial development.New York: Elsevier, 1980; 119–132.
17.
Morriss-KayG.Retinoic acid and craniofacial development: molecules and morphogenesis.Bioessays1993; 15: 9–15.
18.
Morriss-KayG., WoodH., ChenW.H.Normal neurulation in mammals.Ciba Found Symp1994; 181: 51–69.
19.
MortonW.R.M.Duplication of the pituitary and the stomatodaeal structures in a 38 week male infant.Arch Dis Child1956; 31: 135–142.
20.
PadgetD.H.The development of the cranial arteries in the human embryo.Carnegie Inst Wash Pub 212, Contrib Embryol1948; 32: 205–261.
21.
PuellesL., RubensteinJ.L.Expression patterns of homeobox and other putative regulatory genes in the embryonic mouse forebrain suggest a neuromeric organization.Trends Neurosci1993; 16: 472–479.
22.
SiebertJ.R., AstleyS.J., ClarrenS.K.Holoprosencephaly in a fetal macaque (Macaca nemestrina) following weekly exposure to ethanol.Teratology1991; 44: 29–36.
23.
StrickerM., van der MeulenJ.C., RaphaelB., MazzolaR., TolhurstD.E.Craniofacial malformations.Edinburgh: Churchill Livingstone,1990; 1–11. (History; see especially Fig 1.12, second row right).
24.
TessierP.Anatomical classification of facial, cranio-facial, and latero-facial clefts.J Maxillofac Surg1976; 4: 69–92.
25.
ThorogoodP., FerrettiP.Heads and tales: recent advances in craniofacial development.Br Dent J1992; 173: 301–306.
26.
VoneschJ.L., NakshatriH., PhilippeM., ChambonP., DolleP.Stage and tissue-specific expression of the alcohol dehydrogenase 1(Adh-1) gene during mouse development.Dev Dyn1994; 199: 199–213.
27.
WalshF.B., HoytW.F.Clinical neuro ophthalmology.Baltimore: Williams and Wilkins,1969; 648–655.
28.
WarburgM.Update of sporadic microphthalmos and coloboma. non-inherited anomalies.Ophthalmic Paediatr Genet1992; 13: 111–122.
29.
YajimaH.Induction of longitudinal double malformations by centrifugalion or by partial UV-irradiation of eggs in the chironomid species, Chironomus samoensis (diptera: chironomidae).Entomol Genet1983; 8: 171–191.
30.
YakovlevP.I.Pathoarchitectonic studies of cerebral malformations. III. Arrhinencephalies (holotelencephalies).J Neuropath Exp Neurol1959; 18: 22–55.
