Sandbox:kalpana
Overview
Fetal hydantoin syndrome (FHS), characterized by altered growth and development, has been well described in recent years in the fetus of epileptic mothers taking phenytoin or other hydantoin anticonvulsants during the gestational period.[1]
Historical Perspective
Fetal hydanoin syndrom was first discovered by Meadow et al. in 1968. Manson and Frederic clarified the teratogenic effects of hydantoin in their epidemiological studies in 1973.[2]
Classification
There is no established system for the classification of Fetal hydantoin syndrome.
Pathophysiology
It is understood that fetal hydantoin syndrome s the result of infants born to mothers with seizure disorders treated with anticonvulsant medications during pregnancy are at an increased risk for teratogenic effects.[3]
Epidemiology and Demographics
The risk of neurological impairment estimated to be 1% to 11% is 2 to 3 times higher than in the general population. The risk of oral clefts and cardiac anomalies is 5 times than others in hydantoin exposed infants. Less frequently observed abnormalities include microcephaly, ocular defects, hypospadias, umbilical and inguinal hernias.[3]
Diagnosis
Diagnostic Study of Choice
There are no established criteria for the diagnosis of fetal hydantoin syndrome.
History and Symptoms
The patient with fetal hydantoin syndrome has a positive history of exposure to phenytoin during pregnancy. Common symptoms of fetal hydantoin syndrome include microcephaly, mental retardation, limb defects including hypoplastic nails and distal phalanges, heart defects.
Physical Examination
Common physical examination findings of fetal hydantoin syndrome include
- microcephaly, distinctive facial and limb anomalies, ocular defects, growth deficiency, congenital heart defects, cardiac rhythm disturbances, and variable systemic abnormalities involving the nervous, renal, and gastrointestinal systems.
- Congenital heart diseases associated with fetal hydantoin syndrome include pulmonary or aortic valvular stenosis, coarctation of aorta, patent ductus arteriosus, and ventricular septal defects.[4]
Laboratory Findings
- ↑ Jimenez JF, Seibert RW, Char F, Brown RE, Seibert JJ (1981). "Melanotic neuroectodermal tumor of infancy and fetal hydantoin syndrome". Am J Pediatr Hematol Oncol. 3 (1): 9–15. PMID 6263127.
- ↑ Ozkinay F, Yenigün A, Kantar M, Ozkinay C, Avanoğlu A, Ulman I (1998). "Two siblings with fetal hydantoin syndrome". Turk J Pediatr. 40 (2): 273–8. PMID 9677735.
- ↑ 3.0 3.1 Singh R, Kumar N, Arora S, Bhandari R, Jain A (2012). "Fetal hydantoin syndrome and its anaesthetic implications: a case report". Case Rep Anesthesiol. 2012: 370412. doi:10.1155/2012/370412. PMC 3469078. PMID 23082254.
- ↑ Hegde A, Kaur A, Sood A, Dhanorkar M, Varma HT, Singh G; et al. (2017). "Fetal Hydantoin Syndrome". J Pediatr. 188: 304. doi:10.1016/j.jpeds.2017.05.018. PMID 28578158.