Fetal hydantoin syndrome is a rare disorder that is believed to be caused by exposure of a fetus to the anticonvulsant drug phenytoin. 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. Less frequently observed anomalies include cleft lip and palate, microcephaly, ocular defects, cardiovascular anomalies, hypospadias, umbilical and inguinal hernias, and significant developmental delays. Anaesthesia for incidental surgery in such a patient poses unique challenges for the anesthesiologist. We report the successful management of a 4-year-old male child with fetal hydantoin syndrome, cleft palate, spina bifida, atrial septal defect, and dextrocardia for tibialis anterior lengthening under subarachnoid block.
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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Less commonly, microcephaly, ocular defects, oral clefts, umbilical and inguinal hernias, hypospadias and cardiac anomalies have also been reported. Other search option s Alphabetical list. Suggest an update.
Summary and related texts. Related genes. Clinical signs. Check this box if you wish to receive a copy of your message. Additional information Further information on this disease Classification s 6 Gene s 0 Clinical signs and symptoms Publications in PubMed Other website s 3. Health care resources for this disease Expert centres Diagnostic tests 0 Patient organisations 19 Orphan designation s and orphan drug s 0.
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Fetal hydantoin syndrome
Its clinical manifestations include limb abnormalities, ocular defects, central nervous system anomalies, intrauterine growth restriction, and hand and phalangeal anomalies. This case report presents an 8-year-old child born to an epileptic mother with a history of AED therapy, with features suggestive of FHS. The epidemiology of epilepsy in Rochester, Minnesota, through Epilepsia ;
Fetal Hydantoin Syndrome and Its Anaesthetic Implications: A Case Report
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services.
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 the treatment of epilepsy. It may also be called congenital hydantoin syndrome ,  fetal hydantoin syndrome , dilantin embryopathy , or phenytoin embryopathy. Association with EPHX1 has been suggested. About one third of children whose mothers are taking this drug during pregnancy typically have intrauterine growth restriction with a small head and develop minor dysmorphic craniofacial features microcephaly and mental retardation and limb defects including hypoplastic nails and distal phalanges birth defects.