Omphalocele in fetus at 13 weeks' gestation exposed to sodium valproate in utero

Abstract

Omphalocele occurs in 1/3200 to 1/10 000 livebirths. It is a defect in the anterior abdominal wall with extrusion of abdominal contents into the base of the umblical cord. In nearly 50 % of cases, the omphalocele is associated with other malformations. Also it may be a manifestation of multiple anomaly syndromes including Beckwith-Wiedemann and CHARGE, trisomies 13, 18, 21 and triploidy. Valproic acid and valproic acid sodium are anticonvulsive drugs used in the treatment of epileptic disorders. Drug passes fetus through plasenta rapidly. It causes major and minor congenital anomalies, intrauterine growth restriction, hyperbiluribunemia, hepatotoxicity, transient hyperglisinemia, fetal and neonatal distress when used in pregnancy. In the present article, a fetus with omphalocele diagnosed at 13 weeks gestation exposed to sodium valproate in utero are presented and the subject is discussed in the light of contemporary literature.

Keywords

• Valproic Acid,Hernia, Umbilical,Pregnancy Complications,Adverse effects,Abortion, Induced

İn utero sodyum valproata maruz kalan 13-14 haftalık. omfalosel olgusu

Abstract

Omfalosel 1/3200 ile 1/10 000 canlı doğum arasında görülen nadir bir durumdur. Abdominal içeriğin orta hattan herniye olması olarak tanımlanabilir. Vakaların yaklaşık yarısı diğer malformasyonlarla ilişkilidir. Ayrıca multipl anomali sendromları (Beckwith-Wiedemann ve CHARGE gibi), trizomi 13, 18, 21 ve triploidiler de eşlik edebilmektedir. Valproik asid ve tuz formu sodyum valproate konvülsif hastalıkların tedavisinde kullanılan antikonvüzanlardır. İlaç, plasentadan fetüse hızla geçmektedir. Gebelikte valproik asid ve sodyum valproate kullanımında görülen fetal ve neonatal sorunlar arasında majör ve minör konjenital anomaliler, intrauterin gelişme geriliği, hiperbiluribinemi, hepatotoksisite, geçici hiperglisinemi, fetal ve neonatal distres sayılabilmektedir. Makalede, gebeliği esnasında valproik asid kullanan ve 13-14 hafialılf iken yapılan ultrasonografide omfalosel tespit edilen olgu sunularak, konu güncel literatürün ışığında tartışılmıştır.

Keywords

• Valproik asit,Fıtık, göbek,Gebelik komplikasyonları,Yan etkiler,Düşük, istemli

References

1. 1. Callen WP. Ultrasonography in Obstetrics and Gynecology. 4th ed, 2000: 498. WB Saunders
2. 2. Günalp S, Tuncer S. Kadın Hastalıkları ve Doğum, Tanı ve Tedavi. 1. baskı, 2004: 48. Pelikan Yayıncılık
3. 3. Chen CP, Chang TY, Lin HH, Chern SR, Wang W. Concomitant omphalocele and anencephaly associated with trisomy 18 and arthrogryposis diagnosed in early pregnancy. Taiwan J Obstet Gynecol. 2008 Mar;47(l):93-94
4. 4. Stoll C, Alembik Y, DottB, Roth MP. Omphalocele and gastroschisis and associated malformations
5. Am J Med Genet A. 2008 May 15;146A(10):1280-5
6. 5. Chen CP. Syndromes and disorders associated with omphalocele (III): single gene disorders, neural tube defects, diaphragmatic defects and others. 2007 Jun;46(2):111-20
7. 6. Brown JK. Valproate toxicity. Dev Med Child Neurology 1988; 80: 115-125 7. Clayton Smith J, Donnai D. Fetal valproate syndrome. J Med Genet 1995; 32: 724-727
8. 8. Jager-Roman E, Deichl A, Jakob S, Koch S, Rating D, Steldinger R et al. Fetal growth, major malformation and minor anomalies in infants born to women receiving valproic acid. JPediatr 1986; 108: 997-1004

9. 9. Dalens B, Raynaud E-J, Gaulme J. Teratogenicty of valproic acid. J Pediatr 1980;97:332-3 10. Gomez MR. Possible teratogenicty of valproic acid. J Pediatr 1981;98:508-9 11. Thomas D, Buchanan N. Teratogenic effects of anticonvulsants. J Pediatr 1981;99:163 12. Clay SA, McVie R, Chen H. Possible teratogenic effect of valproic acid. J Pediatr 1981 ;99:828 13. Koch S, Jager-Roman E, Rating D, Helge H
10. Possible teratogenic effect of valproate during pregnancy. J Pediatr 1983; 103:1007-8 14. Robert E, GuilbaudP. (1982) Maternal valproic acid and congenital neural tube defects. Lancet 11:1142 15. Dilberti JH, Farndon PA, Dennis NR, Curry CJR. The fetal valproate syndrome. Am J Med Genet 1984; 19 : 473-381
11. 16. Winter RM, Donnai D, Burn J, Tucker SM
12. Fetal valproate syndrome : is there a recognisable phenotype? J Med Genet 1987; 24: 692-695
13. 17. Jones KL(ed). Smith's recognizable patterns of human malformation, fifth edition, Philadelphia; WB Saunders Company 1997; 566-567
14. 18. Kozma C. Valproic acid embryopathy : report of two siblings with further expansion of the phenotypic abnormalities and review of the literature. Am J Med Genet 2001; 98: 168-175
15. 19. Bantz E (1983) Valproic acid and congenital malformations, a case report. Clin Pediatr 23:352-353 20. Ong LL, Schardein JL, Petrere JA, Sakowski R, Jordan H, Humhrey RR, Fitzgerald JE, de la Iglesia FA. Teratogenesis of calcium valproate in rats. 1983 Mar-Apr;3(2): 121-6 21. Winter RM, Donnai D, Burn J, Tuckers SM (1987) Fetal valproate syndrome: is there a recognisable phenotype? J Med Genet 29:692-695 22. Boussemart T, Bonneau D, Levard G, Berthier M, Oriot D. Omphalocele in a newborn baby exposed to sodium valproate in utero. 1995 Mar; 154(3) :220-l