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June 1968, Vol 115, No. 6 >
Article | June 1968

Median Facial Cleft With Hypotelorism FREE

Robert McDonald, MA, MD
[+] Author Affiliations

Received for publication Sept 18, 1967.

Reprint requests to Department of Child Health, University of Cape Town, Wernher and Beit Medical Laboratories Observatory, Cape, South Africa.


Am J Dis Child. 1968;115(6):728-731. doi:10.1001/archpedi.1968.02100010730017.
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THE central part of the face and the forebrain are closely connected in early development, and when midline facial defects occur, there is often concomitant maldevelopment of the anterior part of the brain.1 It was at first thought that the cerebral anomaly consisted of a lack of development of the olfactory bulb, tract, and cortex (arhinencephaly); but the abnormality is now known to be more extensive, with failure of division of the forebrain into lobes and ventricles. For this condition the terms holotelencephaly and alobar holoprosencephaly have been used. Brucker et al1 did not think that these terms adequately covered all the possible varieties of lesion which may occur and suggested as a descriptive title median cerebrofacial dysgenesis. DeMyer and co-workers2 pointed out that if the median facial abnormalities were associated with hypotelorism, a holopresencephalic brain was an invariable accompaniment. We feel that the name given to

REFERENCES

1 +
Brucker, P.A.; Hoyt, C.J.; and Trusler, H.M.:  Severe Cleft Lip With Arrhinencephaly , Plast Reconstr Surg 32:527, 1963;.
2 +
DeMyer, W.; Zeman, W.; and Palmer, C.G.:  The Face Predicts the Brain: Diagnostic Significance of Median Facial Anomalies for Holoprosencephaly (Arhinencephaly) , Pediatrics 34:256, 1964;.
3 +
Currarino, G., and Silverman, F.N.:  Orbital Hypotelorism, Arhinencephaly and Trigonocephaly , Radiology 74:206, 1960;.
4 +
DeMyer, W.; Zeman, W.; and Palmer, C.G.:  Familial Alobar Holopresencephaly (Arhinencephaly) With Median Cleft Lip and Palate , Neurology 13:913, 1963;.
5 +
Bishop, K., et al:  Holoprosencephaly , J Pediat 65:406, 1964;.
6 +
Landau, J.W.; Barry, J.M.; and Koch, R.:  Arhinencephaly , J Pediat 62:895, 1963;.
7 +
Haworth, J.C.; Medovy, H.; and Lewis, A.J.:  Cebocephaly With Endocrine Dysgenesis , J Pediat 59:726, 1961;.
8 +
Miller, J.Q., et al:  A Specific Congenital Brain Defect (Arhinencephaly) in 13-15 Trisomy , New Eng J Med 268:120, 1963;.
9 +
Conen, P.E.; Phillips, K.G.; and Mauntner, L.S.:  Multiple Developmental Anomalies and Trisomy of a Group 13-15 Chromosome ("D" Syndrome) , Canad Med Assoc J 87:709, 1962;.
10 +
Bourne, G.L., and Benirschke, K.:  Absent Umbilical Artery , Arch Dis Child 35:534, 1960;.

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References

1 +
Brucker, P.A.; Hoyt, C.J.; and Trusler, H.M.:  Severe Cleft Lip With Arrhinencephaly , Plast Reconstr Surg 32:527, 1963;.
2 +
DeMyer, W.; Zeman, W.; and Palmer, C.G.:  The Face Predicts the Brain: Diagnostic Significance of Median Facial Anomalies for Holoprosencephaly (Arhinencephaly) , Pediatrics 34:256, 1964;.
3 +
Currarino, G., and Silverman, F.N.:  Orbital Hypotelorism, Arhinencephaly and Trigonocephaly , Radiology 74:206, 1960;.
4 +
DeMyer, W.; Zeman, W.; and Palmer, C.G.:  Familial Alobar Holopresencephaly (Arhinencephaly) With Median Cleft Lip and Palate , Neurology 13:913, 1963;.
5 +
Bishop, K., et al:  Holoprosencephaly , J Pediat 65:406, 1964;.
6 +
Landau, J.W.; Barry, J.M.; and Koch, R.:  Arhinencephaly , J Pediat 62:895, 1963;.
7 +
Haworth, J.C.; Medovy, H.; and Lewis, A.J.:  Cebocephaly With Endocrine Dysgenesis , J Pediat 59:726, 1961;.
8 +
Miller, J.Q., et al:  A Specific Congenital Brain Defect (Arhinencephaly) in 13-15 Trisomy , New Eng J Med 268:120, 1963;.
9 +
Conen, P.E.; Phillips, K.G.; and Mauntner, L.S.:  Multiple Developmental Anomalies and Trisomy of a Group 13-15 Chromosome ("D" Syndrome) , Canad Med Assoc J 87:709, 1962;.
10 +
Bourne, G.L., and Benirschke, K.:  Absent Umbilical Artery , Arch Dis Child 35:534, 1960;.

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