OBSTETRIC ASPECTS IN WOMEN WITH FACIOSCAPULOHUMERAL MUSCULAR-DYSTROPHY, LIMB-GIRDLE MUSCULAR-DYSTROPHY, AND CONGENITAL MYOPATHIES

Objective: To increase the knowledge about pregnancy and delivery in w omen with certain muscle diseases, which is important for obstetric ma nagement and family planning of affected women. Design: The obstetric histories of patients with facioscapulohumeral (FSH) muscular dystroph y, limb-girdle (LG) muscular dystrophy, and congenital myopathies (CM) were retrospectively evaluated using questionnaires and medical repor ts. 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 asc ertained from January 1, 1992, to December 31, 1994, through departmen ts of neurology and human genetics, and the German self-support group for muscle diseases. Fifty-eight gestations resulting in 52 live birth s were reviewed. Results: Miscarraiges were reported in 3 of 26 gestat ion 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 de liveries (vaginal operation or cesarean section) were performed in 6 o f 23 gestations in patients with FSH dystrophy (1 emergency section), in 5 of 12 patients with LC dystrophy (2 emergency sections), and in 3 of 17 deliveries in patients with CM. Patients with FSH dystrophy gen erally coped well with their muscle disease in pregnancy and after del ivery; however, 4 women were stated to have difficulties in caring for their families. The situation differed in LG dystrophy, where most wo men (5 of 9) experienced worsening of weakness in pregnancy and requir ed assistance after delivery. In the patients with CM, 3 women experie nced a deterioration during pregnancy, and 4 patients reported difficu lties after delivery, Conclusions: No deleterious outcome of pregnancy and labor was observed in this series of patients with muscular dystr ophy or CM, although operative deliveries were more frequent. A signif icant 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.