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Perinatal/Neonatal Case Book

Transposition of the Great Arteries and Hypocalcemia in a Patient With Fetal Hydantoin Syndrome


We report a patient with fetal hydantoin syndrome (FHS) with associated d-transposition of the great arteries (d-TGA) and persistent hypocalcemia. d-TGA and hypocalcemia have each been individually reported once in association with FHS, but these patients were also prenatally exposed to phenobarbital. To our knowledge, this is the first report of these problems occurring after prenatal exposure to hydantoin alone. The combination of congenital heart disease and hypocalcemia in our patient raises the possibility of a hydantoin effect on neural crest migration.

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  1. Meadow S . Anticonvulsant drugs and congenital abnormalities Lancet 1968 2: 1296

    CAS  Article  Google Scholar 

  2. Monson R, Rosenberg L, Hartz S et al. Diphenylhydantoin and selected congenital malformations N Engl J Med 1973 289: 1049–52

    CAS  Article  Google Scholar 

  3. Hanson J, Smith D . The fetal hydantoin syndrome J Pediatr 1975 87: 285–90

    CAS  Article  Google Scholar 

  4. Anderson R . Cardiac defects in children of mothers receiving anticonvulsant therapy during pregnancy J Pediatr 1976 89: 318–9

    CAS  Article  Google Scholar 

  5. Friis B, Sardemann H . Neonatal hypocalcemia after intrauterine exposure to anticonvulsant drugs Arch Dis Child 1977 52: 239–47

    CAS  Article  Google Scholar 

  6. Sabry M, Farag T . Hand anomalies in fetal-hydantoin syndrome: from nail/phalangeal hypoplasia to unilateral acheiria Am J Med Gen 1996 62: 410–2

    CAS  Article  Google Scholar 

  7. Johnston M . Understanding human embryonic development In: Stevenson R, Hall J, Goodman R, editors. Human Malformations and Related Anomalies Vol. I: New York: Oxford University Press 1993 31–63

  8. Kirby M, Waldo K . Role of the neural crest in congenital heart disease Circulation 1990 82: 332–40

    CAS  Article  Google Scholar 

  9. Danielson M, Danielsson BRG, Marchner H et al. Histopathological and hemodynamic studies supporting hypoxia and vascular disruption as explanation to phenytoin teratogenicity Teratology 1992 46: 485–97

    CAS  Article  Google Scholar 

  10. Danielsson BRG, Danielson M, Rundqvist E et al. Identical phalangeal defects induced by phenytoin and nifedipine suggest fetal hypoxia and vascular disruption behind phenytoin teratogenicity Teratology 1992 45: 247–58

    CAS  Article  Google Scholar 

  11. Strickler S, Dansky L, Miller M et al. Genetic predisposition to phenytoin induced birth defects Lancet 1985 II: 746–9

    Article  Google Scholar 

  12. Kirby M . Plasticity and predetermination of the mesencephalic and trunk neural crest transplanted into the region of the cardiac neural crest Dev Biol 1989 134: 402–12

    CAS  Article  Google Scholar 

  13. Kirby M, Hunt P, Wallis K et al. Abnormal patterning of the aortic arch arteries does not evoke cardiac malformations Dev Dyn 1997 208: 34–47

    CAS  Article  Google Scholar 

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Shah, M., Morava, E., Gill, W. et al. Transposition of the Great Arteries and Hypocalcemia in a Patient With Fetal Hydantoin Syndrome. J Perinatol 22, 89–90 (2002).

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