Outcome in pregnancies complicated by myotonic dystrophy: a study of 31 patients and review of the literature.
OBJECTIVE
Myotonic dystrophy can be associated with increased obstetric risks, but the maternal contribution for gestational outcome is difficult to establish considering the varying degrees of severity and the influence of fetal factors.


METHODS
We analyzed the pregnancy course and outcome of 31 women with classic myotonic dystrophy, who delivered a total of 66 children. In addition, 93 gestations from the literature were reviewed.


RESULTS
As most patients were not aware of their diagnosis at reproductive age, often the first indication of the maternal disease was a severely affected child (39%). Miscarriages and pre-eclampsia did not increase. Ectopic pregnancies occurred in 4%, placenta previa in 9% of gestations, while postpartum hemorrhage due to uterine atonia was only reported twice. Severe urinary tract infections were reported for 19% of the patients, but were only rarely encountered in the literature. Preterm labor, before 34 weeks, occurred in 19% of gestations and was often, but not exclusively attributed to congenitally affected fetuses in contrast to polyhydramnios (17%). Labor abnormalities of all three stages were frequent, increasing the number of operative deliveries (cesarean section rate 36%). Perinatal mortality was 15% and mainly related to congenitally affected children.


CONCLUSIONS
The risk for obstetric complications and urinary tract infections increases for pregnant patients with myotonic dystrophy. They need constant obstetric monitoring. It is hoped that a better awareness of the clinical picture might help to improve gestational outcome in myotonic dystrophy.
Rudnik-Schöneborn
Sabine
S
Institute for Human Genetics, University of Technology, RWTH Aachen, Germany. srudnik-schoeneborn@ukaachen.de
Zerres
Klaus
K
D000328:Adult; D017668:Age of Onset; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D007231:Infant, Newborn; D009223:Myotonic Dystrophy / Q000453:epidemiology* / Q000209:etiology / Q000235:genetics; D007744:Obstetric Labor Complications; D007752:Obstetric Labor, Premature; D010375:Pedigree; D011247:Pregnancy; D011248:Pregnancy Complications / Q000453:epidemiology* / Q000209:etiology; D011256:Pregnancy Outcome; D011295:Prenatal Care