Pierre Robin is a "sequence" as opposed to a syndrome. It can be associated with
a genetic disorder/ chromosomal abnormality or it can be the result of
intrauterine positioning which inhibits growth of the jaw. In either case the
tongue lies posteriorly and the palate is high and rounded with a V-shaped
cleft. If the malformation occurs in an otherwise chromosomally  normal
individual there will be no associated mental retardation. If, on the other
hand, it occurs (as it often does) in conjunction with other trisomies.....it
will carry the mental retardation of those syndromes.

References:
Smith's "Recognizable Patterns of Human Malformation" and Knupper and Drukker's
"High Risk Pregnancy." Both are published by Saunders.

Susan




Kathy Dettwyler <[log in to unmask]> on 03/06/2000 04:57:41 PM

Please respond to Lactation Information and Discussion
      <[log in to unmask]>

To:   [log in to unmask]
cc:    (bcc: Susan J Keith-Hergert/MGCR/CHP)

Subject:  dumb question




Is Pierre Robin syndrome associated with mental retardation, or just a
seriously recessed lower jaw?

What do these kids look like when they grow up?

Kathy Dettwyler

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