title: Severe twin-twin transfusion syndrome: current management concepts.
abstract: The presence of functioning vascular anastomoses within the monochorionic placentation of a monozygotic twin pregnancy is responsible for the twin-twin transfusion syndrome. This is a heterogeneous disorder with a wide spectrum of severity. In the most severe form it results in the oligohydramnios-polyhydramnios sequence with a marked disparity in intertwin biometry and amniotic fluid volume. Without therapy, the perinatal mortality of severe twin-twin transfusion syndrome is 60-100%. Several therapeutic interventions have been attempted with therapeutic amniocentesis producing the most consistent improvement in fetal survival rates. The outcome of 10 cases of severe twin-twin transfusion syndrome, presenting as the oligohydramnios-polyhydramnios sequence, is presented. Gestational age at presentation ranged from 20-34 weeks. Three fetuses were hydropic at initial presentation and of these only 1 survived. All cases were treated with serial aggressive therapeutic amniocentesis, the number of procedures ranging from 1-9/patient. The volume of amniotic fluid removed ranged from 3,200-14,000 mL. Gestation was prolonged a mean of 46 days (range 1-106 days). The perinatal survival rate was 65% (13/20 infants). Preterm premature rupture of membranes and secondary preterm birth complicated 1 pregnancy treated with therapeutic amniocentesis. Short-term morbidity in the liveborn infants was predominantly secondary to prematurity and renal cortical necrosis. Severe twin-twin transfusion syndrome is the most common aetiology of the oligohydramnios-polyhydramnios sequence. Therapeutic amniocentesis offers an intervention that may reduce the high perinatal loss rate previously observed with this disorder.
authors.affiliation: Department of Obstetrics, King Edward Memorial Hospital for Women, Perth.
authors.forename: J E
authors.initials: JE
authors.lastname: Dickinson
mesh_terms: D000328:Adult; D000649:Amniocentesis / Q000379:methods*; D005260:Female; D005330:Fetofetal Transfusion / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy*; D006801:Humans; D016104:Oligohydramnios / Q000209:etiology; D006831:Polyhydramnios / Q000209:etiology; D011247:Pregnancy; D011256:Pregnancy Outcome; D011379:Prognosis; D016216:Ultrasonography, Prenatal