[A rare cause of polyhydramnios: Steinert's syndrome. A clinical case report].
Steinert's syndrome is a systemic disease with autosome mother-to-child transmission, characterized by myotonia and muscular dystrophia. The syndrome's clinical characteristics include: respiratory and alimentation diseases, facial diplegia, generalized hypotonia, areflexia, atrophy, arthrogryposis, hydramnios, retard in psychomotor development, cataract and genital disorders. A case of pregnancy occurs in Steinert syndrome's patient with hydramnios as a predominant symptom, is presented. At birth, in the congenital neonatal form, there is grave, generalized hypotonia which causes a very quick death of the newborn. There are not many characters which can be found out by ultrasound: hydramnios, reduction of fetal tone and active movements, micrognathia. Consequently it is of the utmost importance in those cases an adequate prenatal genetic counseling and a correct obstetrical management.
Palmerio
G
G
Divisione di Ginecologia e Ostetricia, Ospedale Bolognini, Seriate, Bergamo.
Rosaschino
P
P
Castelli
G
G
Zambetti
E
E
Bianchi
P
P
Martinelli
D
D
D000015:Abnormalities, Multiple / Q000209:etiology / Q000473:pathology; D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009223:Myotonic Dystrophy / Q000150:complications* / Q000473:pathology; D006831:Polyhydramnios / Q000209:etiology* / Q000473:pathology; D011247:Pregnancy; D011263:Pregnancy Trimester, Third
Una causa rara di polidramnios: la sindrome di Steinert. Descrizione di un caso clinico.