선천성 근긴장성 근이영양증과 연관된 재발성 양수과다증 1예

Abstract

Hydramnios is a serious complication of pregnancy associated with a marked increase in perinatal mortality. A variety causes of this condition have been described, the majority of which relate to abnormalities of the fetus. Myotonic dystrophy is a progressive degenerative disease of the neuromuscular system, usually inherited in an autosomal dominant fashion. The disease is variable onset and clinical manifestations and usually severe in the male. Especially congenital myotonic dystrophy is a disease characterized by profound hypotonia. Affected infants present with varying degrees of respiratory failure, often necessitating immediate and prolonged ventilatory assistance. These neonates also have feeding difficulties due to impaired sucking and swallowing. The diagnosis of myotonic dystrophy in pregnancy is made retrospectively, following the birth of a severely affected neonates. A documented case of congenital myotonic dystrophy associated with hydramnios in two successive pregnancies is presented. The evidence suggests that myotonic dystrophy should be considered as a rare cause of recurrent hydramnios. We report a case of recurrent hyramnios in association with congenital myotonic dystrophy with a brief review of literatures.