European Journal of Pediatrics

, Volume 146, Issue 3, pp 301–302 | Cite as

Median cleft face syndrome in association with hydrocephalus, agenesis of the corpus callosum, holoprosencephaly and choanal atresia

  • T. Bömelburg
  • W. Lenz
  • T. Eusterbrock
Case Reports


A premature female infant had the characteristic features of the median cleft face syndrome in association with holoprosencephaly, agenesis of the corpus callosum, hydrocephalus and choanal atresia. Whereas the nervous system is rarely affected in patients with the median cleft face syndrome, severe mental retardation has been reported in three previously published cases and is suggested in our own patient. All had an associated agenesis of the corpus callosum.

Key words

Median cleft face syndrome Frontonasal dysplasia Holoprosencephaly Agenesis of the corpus callosum 



median cleft face syndrome


frontonasal dysplasia


cerebrospinal fluid


inner canthal distance


outer canthal distance


canthal index


inter-pupillary distance


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Copyright information

© Springer-Verlag 1987

Authors and Affiliations

  • T. Bömelburg
    • 1
  • W. Lenz
    • 2
  • T. Eusterbrock
    • 1
  1. 1.Department of PediatricsUniversity of MünsterMünsterFederal Republic of Germany
  2. 2.Department of Human GeneticsUniversity of MünsterMünsterFederal Republic of Germany

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