- Email: [email protected]
Myotonic dystrophy in pregnancy
lstudmdlnl
GYNECOLOGY &OBSTETRICS International Journal of Gynecology & Obstetrics 50 (1995) 297-298
Letter to the editor
Myotonic dystrophy in pregnancy N. Vendola*, C. Matarazzo, S. Bennici Department of Obstetrics and Gynecology, ‘E Morelli’
Hospital, Sondalo. Italy
Received 7 February 1995;accepted 14 June 1995
Keywordr:
Myotonic dystrophy; Pregnancy; Obstetric complications
Pregnancy associated with myotonic dystrophy (MD) may be hazardous both for the mother and the baby. A 24-year-old primigravida was referred to our department at 10 weeks’ gestation becauseof maternal congenital MD in pregnancy. The patient had been born healthy, but because of a walking disability thorough neurological examinations had been started at the age of 20 years. The final diagnosis was MD. There was no family history of neuromuscular disease. At the first antenatal visit she complained of generalizedweakness.Cardiovascular and respiratory systems were normal. Routine blood tests revealed normal levels; only creatinine phosphokinase was marginally elevated. Serial obstetric ultrasound examinations for fetal growth were satisfactory and there was no evidence of excessamniotic fluid. Pregnancy was complicated by worsening muscle weaknessand myotonia, while creatinine phosphokinase levels significantly increased. Electro* Corresponding author, via Frugoni, 26, Box 38, 20162 Milano, Italy, Tel.: +39 2 6432510;Fax: +39 2 6432510.
cardiography showed a first-degree heart block. It was decided to undertake cesarean section under epidural anesthesia at 36 weeks’ gestation. A viable 2200-g female infant was delivered with an Apgar score of 7/10. A densely adherent placenta was found in the posterior uterine wall, requiring manual extraction, which caused the loss of 1.5 1 blood, but hemostasis was achieved. The postoperative course was uneventful. The child has developed well over the course of the 1st year. MD is a multisystem disorder involving several organs besidesskeletal muscle. It presentsat about the age of 18, but may long go unrecognized because of its insidious nature. Complications of pregnancy and delivery may be the first symptoms of the disorder. In fact, sudden deterioration of muscular weaknessat about 28 weeks’ gestation is common and has been linked to an effect of increased levels of circulating progesterone on the cell membrane potential (11. Furthermore, the pregnancy itself may be complicated by fetal loss, premature delivery or abnormalities of labor [2]. BecauseMD affects smooth muscle as well as cardiac and skeletal muscle, it is
0020-7292/95/$09.50 0 1995 International Federation of Gynecology and Obstetrics SSDI 0020-7292(95)02468-R
298
N. Vendola et al. /International
Journal of Gynecology & Obstetrics SO (1995) 297-298
not unusual to find an increased incidence of uterine inertia, retained placenta and postpartum hemorrhage [3]. We report a successful pregnancy outcome in diagnosed maternal muscular dystrophy. Our key to prenatal managementincluded awarenessof the association between MD and complications in pregnancy and delivery, and a multidisciplinary approach to the disorder. Decisions regarding delivery and its timing must be made in collaboration between the obstetrician, neonatologist and neurologist. An experienced anesthesiologist should be available for safe anesthesia,since there are associatedcardiovascular and respiratory risks [41.
References 111 Samat HB, O’Connor T, Byrne PA. Clinical effects of
myotonic dystrophy on pregnancy and neonate. Arch Neurol 1976;33: 459-465. PI Shore RN, MacLachlan TB. Pregnancy with myotonic dystrophy: course, complications and management. Obstet Gynecol 1971;38: 448-454. i31 Harvey JC, Sherboume DH, Siegel CI. Smooth muscle involvement in myotonic dystrophy. Am J Med 1965; 39: 81-90. I41 Miller J, Lee C. Muscle diseases.In: Katz J, Benumof J, Kadis LB, editors. Anesthesia and uncommon diseases. Philadelphia, PA: WB Saunders, 1981: 530-561.
GYNECOLOGY &OBSTETRICS International Journal of Gynecology & Obstetrics 50 (1995) 297-298
Letter to the editor
Myotonic dystrophy in pregnancy N. Vendola*, C. Matarazzo, S. Bennici Department of Obstetrics and Gynecology, ‘E Morelli’
Hospital, Sondalo. Italy
Received 7 February 1995;accepted 14 June 1995
Keywordr:
Myotonic dystrophy; Pregnancy; Obstetric complications
Pregnancy associated with myotonic dystrophy (MD) may be hazardous both for the mother and the baby. A 24-year-old primigravida was referred to our department at 10 weeks’ gestation becauseof maternal congenital MD in pregnancy. The patient had been born healthy, but because of a walking disability thorough neurological examinations had been started at the age of 20 years. The final diagnosis was MD. There was no family history of neuromuscular disease. At the first antenatal visit she complained of generalizedweakness.Cardiovascular and respiratory systems were normal. Routine blood tests revealed normal levels; only creatinine phosphokinase was marginally elevated. Serial obstetric ultrasound examinations for fetal growth were satisfactory and there was no evidence of excessamniotic fluid. Pregnancy was complicated by worsening muscle weaknessand myotonia, while creatinine phosphokinase levels significantly increased. Electro* Corresponding author, via Frugoni, 26, Box 38, 20162 Milano, Italy, Tel.: +39 2 6432510;Fax: +39 2 6432510.
cardiography showed a first-degree heart block. It was decided to undertake cesarean section under epidural anesthesia at 36 weeks’ gestation. A viable 2200-g female infant was delivered with an Apgar score of 7/10. A densely adherent placenta was found in the posterior uterine wall, requiring manual extraction, which caused the loss of 1.5 1 blood, but hemostasis was achieved. The postoperative course was uneventful. The child has developed well over the course of the 1st year. MD is a multisystem disorder involving several organs besidesskeletal muscle. It presentsat about the age of 18, but may long go unrecognized because of its insidious nature. Complications of pregnancy and delivery may be the first symptoms of the disorder. In fact, sudden deterioration of muscular weaknessat about 28 weeks’ gestation is common and has been linked to an effect of increased levels of circulating progesterone on the cell membrane potential (11. Furthermore, the pregnancy itself may be complicated by fetal loss, premature delivery or abnormalities of labor [2]. BecauseMD affects smooth muscle as well as cardiac and skeletal muscle, it is
0020-7292/95/$09.50 0 1995 International Federation of Gynecology and Obstetrics SSDI 0020-7292(95)02468-R
298
N. Vendola et al. /International
Journal of Gynecology & Obstetrics SO (1995) 297-298
not unusual to find an increased incidence of uterine inertia, retained placenta and postpartum hemorrhage [3]. We report a successful pregnancy outcome in diagnosed maternal muscular dystrophy. Our key to prenatal managementincluded awarenessof the association between MD and complications in pregnancy and delivery, and a multidisciplinary approach to the disorder. Decisions regarding delivery and its timing must be made in collaboration between the obstetrician, neonatologist and neurologist. An experienced anesthesiologist should be available for safe anesthesia,since there are associatedcardiovascular and respiratory risks [41.
References 111 Samat HB, O’Connor T, Byrne PA. Clinical effects of
myotonic dystrophy on pregnancy and neonate. Arch Neurol 1976;33: 459-465. PI Shore RN, MacLachlan TB. Pregnancy with myotonic dystrophy: course, complications and management. Obstet Gynecol 1971;38: 448-454. i31 Harvey JC, Sherboume DH, Siegel CI. Smooth muscle involvement in myotonic dystrophy. Am J Med 1965; 39: 81-90. I41 Miller J, Lee C. Muscle diseases.In: Katz J, Benumof J, Kadis LB, editors. Anesthesia and uncommon diseases. Philadelphia, PA: WB Saunders, 1981: 530-561.