Myotonic dystrophy is a rare autosomal dominant degenerative neuromuscular
and neuroendocrine disease. Pregnancy can aggravate the maternal disease. O
bstetrical complications include stillbirth, premature labor, polyhydramnio
n, abnormal presentation, prolonged labor, increased operative delivery, po
stpartum hemorrhages and anesthetic accidents. If the fetus is affected sev
ere neonatal morbidity and mortality with arthrogryposis and mental retarda
tion is common. We present a case where the family chose continuation of pr
egnancy with a known diagnosis of maternal and severe fetal myotonic dystro
phy. A multidisciplinary team was used in the management of pregnancy and c
ounseling the patient. (C) 1999 Elsevier Science Ireland Ltd. All rights re
served.