van de Biezenbos J B, Nijhuis J G, Brunner H G
Martini Ziekenhuis, locatie Van Swieten, afd. Neurologie, Groningen.
Ned Tijdschr Geneeskd. 1992 Dec 12;136(50):2480-2.
We describe the maternal and neonatal complications of pregnancy in two patients with myotonic dystrophy. The disease leads to an increased spontaneous abortion rate, hydramnios, prolonged first and second stages of labour, retained placenta, postpartum haemorrhages and anaesthetic sensitivity in the mother. The neonatal problems are caused by the congenital form of the disease. The major clinical features of congenital myotonic dystrophy are bilateral facial weakness, hypotonia, neonatal distress, feeding difficulties, talipes, tent-shaped mouth, mental retardation and delayed motor development. Relatives of a known myotonic dystrophy patient should be advised to let themselves be examined for this disease. If the disease is diagnosed, information should be given regarding possibilities for prenatal diagnosis. Pregnancy in myotonic dystrophy patients should be monitored by a gynaecologist. Labour has to take place in a hospital with intensive care facilities for mother and child.
我们描述了两名患强直性肌营养不良症患者妊娠时的孕产妇及新生儿并发症情况。该疾病导致母亲自然流产率增加、羊水过多、第一产程和第二产程延长、胎盘滞留、产后出血以及对麻醉敏感。新生儿问题由该疾病的先天性形式引起。先天性强直性肌营养不良症的主要临床特征为双侧面部无力、肌张力减退、新生儿窘迫、喂养困难、畸形足、嘴呈帐篷状、智力发育迟缓以及运动发育延迟。已知强直性肌营养不良症患者的亲属应被告知接受该疾病的检查。若确诊该疾病,应告知其产前诊断的可能性。强直性肌营养不良症患者的妊娠应由妇科医生进行监测。分娩必须在具备母婴重症监护设施的医院进行。