Obstetric aspects in women with facioscapulohumeral muscular dystrophy, limb-girdle muscular dystrophy, and congenital myopathies
S. Rudnik-Schoneborn, B. Glauner, D. Rohrig and K. Zerres
Institute for Human Genetics, University of Bonn, Germany.
OBJECTIVE: To increase the knowledge about pregnancy and delivery in women
with certain muscle diseases, which is important for obstetric management
and family planning of affected women. DESIGN: The obstetric histories of
patients with facioscapulohumeral (FSH) muscular dystrophy, limb-girdle
(LG) muscular dystrophy, and congenital myopathies (CM) were
retrospectively evaluated using questionnaires and medical reports.
PATIENTS: The condition of 27 patients with different myopathies (FSH
muscular dystrophy, n = 11; LG muscular dystrophy, n = 9; and CM, n = 7
[subdivided into 5 patients with central core disease, 1 patient with
cytoplasmic bodies, and 1 patient with unspecified myopathy]) were
ascertained from January 1, 1992, to December 31, 1994, through departments
of neurology and human genetics, and the German self-support group for
muscle diseases. Fifty-eight gestations resulting in 52 live births were
reviewed. RESULTS: Miscarriages were reported in 3 of 26 gestations in 11
patients with FSH dystrophy, whereas 3 of 15 pregnancies in patients with
LG dystrophy were terminated. Preterm births occurred in 2 patients with
FSH dystrophy and in 3 patients with CM. Operative deliveries (vaginal
operation or cesarean section) were performed in 6 of 23 gestations in
patients with FSH dystrophy (1 emergency section), in 5 of 12 patients with
LG dystrophy (2 emergency sections), and in 3 of 17 deliveries in patients
with CM. Patients with FSH dystrophy generally coped well with their muscle
disease in pregnancy and after delivery; however, 4 women were stated to
have difficulties in caring for their families. The situation differed in
LG dystrophy, where most women (5 of 9) experienced worsening of weakness
in pregnancy and required assistance after delivery. In the patients with
CM, 3 women experienced a deterioration during pregnancy, and 4 patients
reported difficulties after delivery. CONCLUSIONS: No deleterious outcome
of pregnancy and labor was observed in this series of patients with
muscular dystrophy or CM, although operative deliveries were more frequent.
A significant aggravation of symptoms in gestation is more likely to occur
in patients with early-onset and progressive myopathy than in those with
stable disease courses.
