Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach.

Two patients with incomplete pentalogy of Cantrell are described. The first was a girl with a large omphalocele with evisceration of the heart, liver and intestines with an intact sternum. Echocardiography showed profound intracardiac defects. The girl died 33 h after birth. The second patient was a female fetus with ectopia cordis (EC) without intracardiac anomalies; a large omphalocele with evisceration of the heart, stomach, spleen and liver; a hypoplastic sternum and rib cage; and a scoliosis. The pregnancy was terminated. A review of patients described in the literature is presented with the intention of finding prognostic factors for an optimal approach to patients with the pentalogy of Cantrell. In conclusion the prognosis seems to be poorer in patients with the complete form of pentalogy of Cantrell, EC, and patients with associated anomalies. Intracardial defects do not seem to be a prognostic factor.

Progesterone for the prevention of preterm birth in women with multiple pregnancies: the AMPHIA trial.

BACKGROUND: 15% of multiple pregnancies ends in a preterm delivery, which can lead to mortality and severe long term neonatal morbidity. At present, no generally accepted strategy for the prevention of preterm birth in multiple pregnancies exists. Prophylactic administration of 17-alpha hydroxyprogesterone caproate (17OHPC) has proven to be effective in the prevention of preterm birth in women with singleton pregnancies with a previous preterm delivery. At present, there are no data on the effectiveness of progesterone in the prevention of preterm birth in multiple pregnancies. METHODS/DESIGN: We aim to investigate the hypothesis that 17OHPC will reduce the incidence of the composite neonatal morbidity of neonates by reducing the early preterm birth rate in multiple pregnancies. Women with a multiple pregnancy at a gestational age between 15 and 20 weeks of gestation will be entered in a placebo-controlled, double blinded randomised study comparing weekly 250 mg 17OHPC intramuscular injections from 16-20 weeks up to 36 weeks of gestation versus placebo. At study entry, cervical length will be measured. The primary outcome is composite bad neonatal condition (perinatal death or severe morbidity). Secondary outcome measures are time to delivery, preterm birth rate before 32 and 37 weeks, days of admission in neonatal intensive care unit, maternal morbidity, maternal admission days for preterm labour and costs. We need to include 660 women to indicate a reduction in bad neonatal outcome from 15% to 8%. Analysis will be by intention to treat. We will also analyse whether the treatment effect is dependent on cervical length. DISCUSSION: This trial will provide evidence as to whether or not 17OHPC-treatment is an effective means of preventing bad neonatal outcome due to preterm birth in multiple pregnancies. TRIAL REGISTRATION: Current Controlled Trials ISRCTN40512715.

Psychological consequences of termination of pregnancy for fetal anomaly: similarities and differences between partners.

OBJECTIVE: We examined the psychological responses to termination of pregnancy (TOP) for fetal anomaly from both men and women. The aim was to find risk factors for poor psychological outcome both for the individuals and for the couple. METHODS: A cross-sectional study was performed in 151 couples 2-7 years after TOP. We used standardized and validated questionnaires to investigate grief, symptoms of posttraumatic stress, somatic complaints, anxiety, and depression. RESULTS: Most couples adapted well to their loss, although several patients had pathological scores on posttraumatic stress symptoms and depression. Differences between men and women were slight. Higher education, good partner support, earlier gestational age, and life-incompatibility of the disorder positively influenced the outcomes, more for women than for men. Men and women with pathological scores rarely had such scores simultaneously. CONCLUSION: We emphasize the importance of equally involving both parents in the counselling because the outcomes of grief and posttraumatic stress symptoms between men and women only moderately differ and post-TOP psychopathology occurs in men as well. Good adjustment to TOP in women seems dependent on the level of support that they perceive from their partners. The intracouple results of the study suggest a mutual influence in the process of grieving between the partners.

Fetal cardiovascular response to large placental chorioangiomas.

A large placental chorioangioma is a relatively rare condition, which in 50% of all cases will lead to maternal and fetal complications. Since chorioangiomas are often associated with significant arterio-venous shunting within the placenta, several fetal hemodynamic compensatory mechanisms are initiated. Ultrasound and color Doppler flow mapping are important for the prenatal diagnosis of chorioangiomas, as an early prenatal diagnosis is crucial to minimize the risks for fetal well-being. Close surveillance of pregnancy and pregnancy termination by cesarean section at the earliest signs of fetal cardiac decompensation are indicated to reduce fetal and neonatal complications. Novel intrauterine treatment options include intravascular transfusion, fetoscopic devascularization, microcoil embolization, and intravascular injection of absolute alcohol.