The overall incidence of neonate malformations for offspring of mothers treated with antiseizure medications is approximately 10%, or 2 to 3 fold. Pdf fetal hydantoin syndrome fhs is a spectrum of defects caused to. Fetal hydantoin syndrome with rheumatic valvular heart. Fetal hydantoin syndrome the journal of pediatrics. Fetal hydantoin syndrome, also called fetal dilantin syndrome is a group of defects caused to the developing fetus by exposure to teratogenic effects of phenytoin. Dilantin is the brand name of the drug phenytoin sodium in the united states, commonly used in.
An anticonvulsant drug, c15h12n2o2, used to treat epilepsy, often in the form of its sodium salt. The discussion aims to bring to attention thepotential hazard of the use of hydantoin drug during reproductive age to all medical practitioners. Fetal hydantoin syndrome synonyms, fetal hydantoin syndrome pronunciation, fetal hydantoin syndrome translation, english dictionary definition of fetal hydantoin syndrome. Hanson jw, myrianthopoulos nc, harvey ma, smith dw j pediatr 1976 oct. Though fetal hydantoin syndrome fhs was first reported by langhman and others, wide phenotypic variability of this syndrome has lead many clinicians to question its very existence.
Statistics of fetal hydantoin syndrome 0 people with fetal hydantoin syndrome have taken the sf36 survey. Less frequently observed anomalies include cleft lip and palate. Fetal hydantoin syndrome genetic and rare diseases information. Fetal hydantoin syndrome pictures, causes, symptoms. Some of the most frequently seen physical deformities include a. The suspected teratogenicity of diphenylhydantoin dph in man is important, especially to the 0. The infant had hypoplastic distal phalanges and hypoplastic fingernails, which are common manifestations of the fetal hydantoin syndrome. Fetal hydantoin syndrome dr rohit sharma and radswiki et al. But the patient also had an imperforate anus, a rectovaginal fistula, renal agenesis, a dislocated hip, multiple rib and. The trip database provides clinical publications about. Article pdf available in journal of nepal paediatric society.
Fetal hydantoin syndrome characteristics jama pediatrics. His mother had been receiving oral phenytoin 300 mg daily for the past 3 years, including the entire period of gestation, for treatment of her. Fetal hydantoin syndrome and its anaesthetic implications. Some of the principal syndromes of this disorder include microcephaly or developing an unusually small head, brain malfunctions, developmental delays, growth deficiencies. Fetal hydantoin syndrome is a rare disorder that is believed to be caused by exposure of a fetus to the anticonvulsant drug phenytoin. Understand clinical significance toward the maternal and fetal effects of each medical. Women with epilepsy who are pregnant and worried about their medication. Fetal hydantoin syndrome or fetal dilantin syndrome is a rare disease that is caused when a fetus is exposed to phenytoin, an anticonvulsant drug in the form of dilantin. Risks to the offspring of women treated with hydantoin anticonvulsants, with emphasis on the fetal hydantoin syndrome. We report a twelve year old girl with fhs with rheumatic valvular heart disease. Eleven per cent of infants exposed to hydantoins in utero have a recogniz able pattern of abnormalities with serious clinical consequences.
Drug is thought to deplete already low levels of fetal vitamin k therefore suppressing the vitamin kdependent coagulation factors ii, vii, ix, and x. The fetal hydantoin syndrome is a pattern of congenital anomalies including craniofacial anomalies, nail and digital hypoplasia, prenatalonset growth deficiency, and neurodevelopmental deficiencies. Fetal hydantoin syndrome radiology reference article. The antiepileptic drugs are prescribed to mother due to controlling the seizure attack.
Abstract in a prospective study of 19 pregnancies monitored by amniocentesis at the center for human genetics and the meyer rehabilitation institute, university of nebraska, omaha, an adverse outcome was predicted for four fetuses on the basis of low epoxide hydrolase activity 30% of standard. Letters must be signed by au authors, typewrittes double spaced, and most not exceed two pages of text including references. Sc, universit of oregony, 1975 thesis submitted i partian l fulfillment the requirements for the degree of master of science in the faculty of graduate studies in the genetics program we accep thit thesis ass conforming to the required standard. Not all infants exposed to phenytoin will be affected with the disorder. Risks to the offspring of women treated with hydantoin anticonvulsants with emphasis on the fetal hydantoin syndrome. Handbook of genetic counselingfetal hydantoin syndrome. An additional 30% of children who are exposed during embryonic development show lesser degrees of alteration. Sibs exposed to similar amount have been affected to different degrees. There is a wide range in the nature and severity of characteristics associated with fetal hydantoin syndrome. Physical findings included gum hypertrophy, digitaiization of the thumbs. However, very few studies were able to account for the etiological and phenotypic heterogeneity of these conditions. Physical deformities involving the face and skull are quite common and can take on a wide variety of forms. This syndrome has several similarities to fetal hydantoin syndrome, including hypoplastic fingernails and toenails, especially of the fifth digits. Fetal hydantoin syndrome is a disorder that is caused by exposure of a fetus to phenytoin, a drug commonly prescribed for epilepsy.
Letters must be signed by au authors, typewrittes double spaced, and most. The increased incidence of fetal malformation after gestational exposure to hydantoin has been reported in pediatric, 1 obstetric, 1 and general medical 3 journals but not previously, to my knowledge, in the dermatologic literature. Fetal hydantoin syndrome jama dermatology jama network. Fetal hydantoin syndrome is a drugrelated embryofetopathy that can occur when an embryofetus is exposed to the anticonvulsant drug. Of infants born to women who used phenytoin during pregnancy, 1030% are reported to show some of the characteristics associated with this syndrome. Fetal hydantoin syndrome is a characteristic pattern of mental and physical birth. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for fetal hydantoin syndrome. Over the years, great efforts have been made to record the frequency of orofacial clefts in different populations.
Fetal hydantoin syndrome genetic and rare diseases. Fetal hydantoin syndrome, also called fetal dilantin syndrome is a group of defects caused to the developing fetus by exposure to the teratogenic effects of phenytoin or carbamazepine. Patient did not respond well to the commands during the treatment too. Total score ranges from 0 to 3,600 being 0 the worst and 3,600 the best. Dilantin is the brand name of the drug phenytoin sodium in the united states, commonly used in the treatment of epilepsy it may also be called congenital hydantoin syndrome, fetal hydantoin syndrome, dilantin embryopathy, or. But most antiepileptic drugs, such as phenytoin has a teratogenic effect, means these drugs can cross placental barrier of the pregnant women and reach to the fetus or embryo and provide multiple harmful effects like fetal hydantoin syndrome. An increased risk of heart defects, facial clefts, and digital hypoplasia has been reported. Fetal hydantoin syndrome is a characteristic pattern of mental and physical birth defects that results from maternal use of the antiseizure anticonvulsant drug phenytoin dilantin during pregnancy. Fetal hydantoin syndrome, phenytoin, congenital anomalies, epilepsycase reporta term baby 41wks was born to a g p l mother with polyhydraminos with seizure disorder. One entity that can be confused with the fetal hydantoin syndrome is the coffinsiris syndrome. The risk that an infant exposed to hydantoin in utero will have the clinical phenotype of the fullblown fetal hydantoin syndrome is approximately 5 to 10 percent, whereas the risk of an infants. A drugrelated embryofetopathy that can occur when an embryofetus is exposed to the anticonvulsant drug.
This report of a child born to a mother taking diphenylhydantoin emphasizes the ocular find ings. Research has shown that anticonvulsants are teratogens and pose a risk for fetal malformations. The fetal hydantoin syndrome refers to a spectrum of features that can develop in a small proportion 10% 5 of women taking the antiepileptic drug phenytoin dilantin tm during pregnancy. Hydantoin definition of hydantoin by the free dictionary. If you have problems viewing pdf files, download the latest version of adobe. Meadow was the first to note a possible link between congenital abnormalities and maternal use of anticonvulsive drug in 1968. The distinctive cutaneous features of the fetal hydantoin syndrome may bring the disorder to the attention of the dermatologist. Prenatal prediction of risk of the fetal hydantoin syndrome. Effects due to maternal exposure to hydantoin anticonvulsant. Cleft lip and palate broad nasal bridge ocular hypertelorism abnormal ears congenital heart disease limb malformation mental and growth retardation 10. The range and severity of associated abnormalities will vary greatly from one infant to another.
Fetal hydantoin syndrome definition of fetal hydantoin. The increased incidence of fetal malformation after gestational exposure to hydantoin has been reported in pediatric,1 obstetric,1 and general medical3 journals. The abnormalities found in children with fetal hydantoin syndrome. Fetal hydantoin syndrome is a disorder that is caused by exposure of a fetus.
Fetal hydantoin syndrome symptoms,causes,treatment and. If you have problems viewing pdf files, download the latest version of adobe reader. Fetal anticonvulsant syndrome facs also known as fetal valproate syndrome and fetal hydantoin syndrome is a group of malformations that can affect some babies if they are exposed to certain medicines known as antiepileptic drugs aeds while in the womb most women with epilepsy will have a healthy child. Fetal hydantoin syndrome an overview sciencedirect topics. Fetal hydantoin syndrome medical condition youtube. The fetal hydantoin syndrome, a recurrent pattern of minor anomalies that occurs in about 10% of infants born to epileptic women treated with phenytoin during pregnancy, has been recognized for more than 35 years 233. Treatment is directed towards the symptoms that are present in an individual. A case of prenatal diagnosis of fetal hydantoin syndrome by ultrasound. Birth defects associated with phenytoin and phenobarbital taken during pregnancy include craniofacial and limb deformities. Fetal hydantoin syndrome nord national organization for rare.
An infant with features of fetal hydantoin syndrome, born to an epileptic mother, was foilowed from birth to 20 months of age. A case of prenatal diagnosis of fetal hydantoin syndrome. An increased risk of congenital abnormalities fetal hydantoin syndrome has been associated with the use of phenytoin the active metabolite of this drug in epileptic women during pregnancy. Fetal hydantoin syndrome nord national organization for. Ideal sources for wikipedia s health content are defined in the guideline wikipedia. Symptoms in affected individuals may include abnormalities of the skull and facial features, growth deficiencies, underdeveloped nails of the fingers and toes, andor mild developmental delays. Fetal hydantoin syndrome how is fetal hydantoin syndrome. Ocular manifestations of the fetal hydantoin syndrome g. Bhakoo, omkar n kapoor, madan m kanwar, amrinder j. Pdf letters to the editor are wdcomed for publication subject to editing. Dilantin, phenytoin and a few other similar anticonvulsants are also included. Thus, data of cases with syndromic orofacial clefts from large populationbased studies are infrequent. The classic features of fetal hydantoin syndrome include craniofacial anomalies, prenatal and postnatal growth deficiencies, underdeveloped nails of the fingers and toes, and mental retardation. Medical complications of pregnancy serena wu, md maternalfetal medicine department of obgyn.
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