FETAL HYDANTOIN SYNDROME

Öz

An association between hydantoin taken during pregnancy and congenital abnormalities was defined as “fetal hydantoin syndrome”(FHS). Associated minor congenital abnormalities include digit and nail hypoplasia, developmental delay, typical facial appearance, rib anomalies, abnormal palmar creases, hirsutism, and low hairlines. Serious defects such as cardiovascular malformations, cleft lip or palate, and inguinal hernia may also be associated with this syndrome. In this case report, a 13-month-old girl with fetal hydantoin syndrome whose mother had received phenytoin throughout pregnancy has been presented. Her physical examination revealed global developmental delay, craniofacial dysmorphism, and pes equino varus. The patient did not have the cardiovascular malformations associated with FHS.The aim of this case report is to promote awareness among pediatricians regarding the importance of recognizing FHS in infants with congenital abnormalities

Anahtar Kelimeler

• Anticonvulsants, infant

Fetal Hidantoin Sendromu

Öz

Gebelikte hidantoin kullanımı ve konjenital anomali birlikteliği “ Fetal hidantoin sendromu” olarak tanımlanmıştır. Eşlik eden minör konjenital anomaliler; parmak ve tırnak hipoplazisi, büyüme geriliği, tipik yüz görünümü, kosta anomalileri, anormal palmar çizgiler, hirşutizm ve saç çizgisinde düşüklük olabilir. Kardiyovasküler malformasyonlar, yarık damak veya dudak ve inguinal herni bu sendromla ilişkili olabilir.Bu olgu takdiminde annesi gebeliği süresince hidantoin kullanmış, 13 aylık fetal hidantoin sendromlu bir kız hasta sunulmaktadır. Hastanın fizik muayene bulguları; genel gelişme geriliği, kraniyofasiyal dismorfizm ve pes ekino varusu içermekteydi. Olgunun sendrom ile ilişkili bir bulgu olan kardiyovasküler anomalisi yoktu.Bu olgu sunumu ile, pediatri uzmanlarının konjenital anomalili bebeklerde fetal hidantoin sendromunu akla getirmesi gerektiğini vurgulamayı amaçladık.

Anahtar Kelimeler

• Antikonvülzanlar, bebek

Kaynakça

1. Nicolai J, Vles JS, Aldenkamp AP.Neurodevelopmental delay in children exposed to antiepileptic drugs in utero: a critical review directed at structural study-bias. J Neurol Sci 2008;271(1-2):1-14.
2. Oguni M, Osawa M. Epilepsy and pregnancy. Epilepsia 2004;8:37- 41.
3. Janz D. On major malformations and minor anomalies in the offs- pring of parents with epilepsy. In: Janz D, Dam M, Richens A, Boosi L, Helge HG, Schmidt D, eds. Epilepsy, pregnancy and the child. New York: Raven Press, 1982:211-22.
4. Oğuz A, Sezgin I, Gökalp A, Gültekin A.The fetal hydantoin syndrome. Mater Med Pol 1991;23(4):308-11.
5. Van Dyke DC, Hodge SE, Heide F, Hill LR. Family studies in fetal hydantoin exposure. J Pediatr 1988;113(2):301-6.
6. No authors listed. Teratogenic risks of antiepileptic drugs. Br Med J (Clin Res Ed) 1981;283(6290):515-6.
7. Tavlı V, Ağın H, Sarıtaş T, Çevik B. Fetal hidantoin sendromu. Tur- kiye Klinikleri J Gynecol Obst 2006;16(4):156-8.
8. Kelly TE, Edwards P, Rein M, Miller JQ, Dreifuss FE. Teratogeni- city of anticonvulsant drugs. II: A prospective study. Am J Med Ge- net 1984;19(3):435-43.

9. Azarbayjani F, Danielsson BR. Pharmacologically induced emb- ryonic dysrhythmia and episodes of hypoxia followed by reoxyge- nation: a common teratogenic mechanism for antiepileptic drugs? Teratology 1998;57(3):117-26.
10. D’Souza SW, Robertson IG, Donnai D, Mawer G. Fetal pheny- toin exposure, hypoplastic nails, and jitteriness. Arch Dis Child 1991;66(3):320-4.
11. Buehler BA, Bick D, Delimont D. Prenatal prediction of risk of the fetal hydantoin syndrome. N Engl J Med 1993;329(22):1660-1.
12. Buehler BA, Rao V, Finnell RH. Biochemical and molecular tera- tology of fetal hydantoin syndrome. Neurol Clin 1994;12(4):741-8.