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$Unique_ID{BRK03737}
$Pretitle{}
$Title{Fetal Hydantoin Syndrome}
$Subject{Fetal Hydantoin Syndrome Dilantin Fetal Effects Of Fetal Dilantin
Syndrome Fetal Hydantoin Effects Hydantoin Anticonvulsants Fetal Effects Of
Mephenytoin Fetal Effects Of Mesantoin Fetal Effects Of Phenytoin Fetal
Effects Of Aarskog Syndrome Noonan Syndrome}
$Volume{}
$Log{}
Copyright (C) 1993 National Organization for Rare Disorders, Inc.
948:
Fetal Hydantoin Syndrome
** IMPORTANT **
It is possible that the main title of the article (Fetal Hydantoin
Syndrome) is not the name you expected. Please check the SYNONYMS listing to
find the alternate name and disorder subdivisions covered by this article.
Synonyms
Dilantin, Fetal Effects Of
Fetal Dilantin Syndrome
Fetal Hydantoin Effects
Hydantoin Anticonvulsants, Fetal Effects Of
Mephenytoin, Fetal Effects Of
Mesantoin, Fetal Effects Of
Phenytoin, Fetal Effects Of
Information on the following diseases can be found in the Related
Disorders section of this report:
Aarskog Syndrome
Noonan Syndrome
General Discussion
** REMINDER **
The Information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Fetal Hydantoin Syndrome is a rare disorder that is caused by exposure of
a fetus to phenytoin (Dilantin) which is an anticonvulsant drug prescribed
for epilepsy. Major symptoms of this disorder may include abnormalities of
the skull and facial features, growth deficiencies, underdeveloped nails of
the fingers and toes, and/or developmental delays.
Symptoms
Fetal Hydantoin Syndrome is typically characterized by altered growth,
unusual facial features, underdeveloped fingers and toes, and mental
deficiencies.
The most consistent facial features found in affected infants have been a
flat bridge of the nose, and eyes that are down-slanted, widely spaced, and
crossed (strabismus). An underdeveloped vertical groove in the center of the
upper lip (philtrum), cleft lip and/or palate, drooping eyelids (ptosis), and
mild webbing of the neck have also been reported.
Growth deficiencies may include underdeveloped fingers and toes,
malformed nails as well as finger-like thumbs.
It has been estimated that approximately thirty percent of affected
children with this syndrome have mild to moderate mental retardation which
may be associated with an abnormally small brain (microencephaly).
Causes
Fetal Hydantoin Syndrome is a rare disorder that is caused by exposure of a
fetus to phenytoin (Dilantin) which is an anticonvulsant drugs prescribed for
epilepsy. Not all infants exposed to phenytoin will be affected with the
disorder. There have been documented cases in which affected and unaffected
siblings have been exposed to the same amount of this drug. Some scientists
believe that enzyme differences may determine which sibling will be affected
with the disorder.
Affected Population
Fetal Hydantoin Syndrome is a rare disorder that affects males and females in
equal numbers. Approximately eleven percent of infants exposed to hydantoin
in the womb are affected with the disorder.
Related Disorders
Symptoms of the following disorders can be similar to those of Fetal
Hydantoin Syndrome. Comparisons may be useful for a differential diagnosis:
Aarskog Syndrome is thought to be inherited as a a sex-linked dominant
trait. Males with Fetal Hydantoin Syndrome may have symptoms similar to
those of Aarskog Syndrome. Infants affected with Aarskog Syndrome may have
widely spaced eyes (hypertelorism) with drooping eyelids (ptosis), and a
short broad nasal bridge. Short broad fingers and toes as well as stunted
growth, genital abnormalities and mild mental retardation may also be
present. (For more information on this disorder, choose "Aarskog Syndrome"
as your search term in the Rare Disease Database).
Noonan Syndrome is a rare disorder that may be inherited as an autosomal
dominant or autosomal recessive genetic trait. Symptoms of this disorder may
also be similar to those of infants affected with Fetal Hydantoin Syndrome.
This disorder is mainly characterized by a lack of sexual development, short
stature, possible mental retardation, a webbed neck, widely spaced eyes with
drooping eyelids, and/or skeletal abnormalities. (For more information on
this disorder, choose "Noonan Syndrome" as your search term in the Rare
Disease Database).
Therapies: Standard
It has been suggested that mothers receiving phenytoin (Dilantin) who have
given birth to an infant affected with Fetal Hydantoin Syndrome be given a
different anticonvulsant drug during future pregnancies. Some physicians
change a woman's epilepsy drug during pregnancy from phenytoin to other
anticonvulsants in order to avoid the possibility of this syndrome in a
fetus. However, the effect of other seizure medications on a fetus are not
always well understood.
When cleft lip and/or palate are present the coordinated efforts of a
team of specialists such as pediatricians, dental specialists, surgeons,
speech pathologists, and psychologists may be used to plan the child's
treatment and rehabilitation. Cleft lip may be surgically corrected.
Generally surgeons repair the lip when the child is still an infant. A
second surgery is sometimes necessary for cosmetic purposes when the child is
older.
Cleft palate may be repaired by surgery or covered by an artificial
device (prosthesis) that closes or blocks the opening. Surgical repair can
be carried out in stages or in a single operation, according to the nature
and severity of the defect. The first palate surgery is usually scheduled
during the toddler period.
Special education and related services will be of benefit to children
with learning delays. Other treatment is symptomatic and supportive.
Therapies: Investigational
Research on birth defects and their causes is ongoing.
This disease entry is based upon medical information available through
April 1993. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Fetal Hydantoin Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Institute of Child Health & Human Development (NICHD)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5133
For children with clefts a group of specialists called a Cleft Palate
Team may be of benefit. For information on local teams contact:
American Cleft Palate Cranial Facial Association
1218 Granview Ave.
Pittsburgh, PA 15211
(412) 681-1376
(800) 24CLEFT
For genetic information and genetic counseling referrals, please contact:
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100
Alliance of Genetic Support Groups
35 Wisconsin Circle, Suite 440
Chevy Chase, MD 20815
(800) 336-GENE
(301) 652-5553
References
SMITH'S RECOGNIZABLE PATTERNS OF HUMAN MALFORMATION, 4th Ed.: Kenneth L.
Jones, M.D., Editor; W.B. Saunders Co., 1988. P. 495.
BIRTH DEFECTS ENCYCLOPEDIA, Mary Louise Buyse, M.D., Editor-In-Chief;
Blackwell Scientific Publications, 1992. Pp. 714-15.
FETAL HYDANTOIN SYNDROME IN TRIPLETS. A UNIQUE EXPERIMENT OF NATURE:
S.A. Bustamante, et al.; Am J Dis Child (October, 1978, issue 132(10)). Pp.
978-9.
RISKS TO THE OFFSPRING OF WOMEN TREATED WITH HYDANTOIN ANTICONVULSANTS,
WITH EMPHASIS ON THE FETAL HYDANTOIN SYNDROME: J.W. Hanson, et al.; J
Pediatr (October, 1976, issue 89(4)). Pp. 662-8.
PRENATAL PREDICTION OF RISK OF THE FETAL HYDANTOIN SYNDROME: B.A.
Buehler, et al.; N Engl J Med (may, 1990, issue 322(22)). Pp. 1567-72.