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Reproductive System Treatment

Reproductive Issues

Although there is often no effective treament to restore fertility, assisted reproductive technology with or without oocyte/sperm donation may be helpful. Prenatal genetic diagnosis may also be performed to identify whether an expanded myotoic dystrophy allele has been passed along to the embryo.

Pregnancy Complications

Due to the increased incidence of complications during pregnancy with a child with congenital myotonic dystrophy type 1 (DM1), intensive obstetric and perinatal care is recommended.

Neonatal complications

  • Polyhydramnios: Amniotic fluid volume reduction may be considered only if there is preterm labor or significant maternal discomfort. Methods for reducing excessive amniotic fluid volume include:
    • Amnioreduction: Amniotic fluid is suctioned to reduce the edema seen. While amnioreduction can be repeated if severe polyhydramnios recurs, this exposes the fetus to the risks of serial invasive procedures and should be done only where symptoms warrant. 
    • Maternal administration of prostaglandin synthetase inhibitors: These agents stimulate fetal secretion of arginine vasopressin, which reduces renal blood flow and fetal urine flow. This has been seen to impair production and/or enhance reabsorption of lung fluid. Fetal and maternal side effects of these drugs include constriction of the ductus arteriosus, esophageal reflux, gastritis, and emesis, which must be monitored. 
  • Fetal hydrops: During pregnancy, treatment of hydrops is limited. Management of hydrops in newborn babies may include:
    • Support for respiratory distress using supplemental oxygen or mechanical ventilation
    • Removal of excessive fluid from spaces around the lungs and abdomen
    • Medications to help the kidneys remove excess fluid

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