Congenital hydrocephalus: a review and protocol for perinatal management.

A review of the literature on congenital hydrocephalus was used to develop a protocol for management. Specific emphasis is given to early diagnosis, evaluation of associated anomalies, and prognosis based on etiology.

Spontaneous resolution of fetal cystic hygroma in Down's syndrome.

We describe a case of spontaneous resolution of a cystic hygroma between 14-16 weeks' gestation in a fetus with trisomy 21 who, at termination at 19 weeks, revealed only mild webbing of the neck. Redundant nuchal skin folds are among the most common features of trisomy 21. Our case supports the hypothesis that this redundant skin of the fetal neck represents early cystic hygromas, which resolve in utero before 16 weeks' gestation.

Ultrasonic diagnosis of discordant fetal growth in twin gestations.

Forty-three consecutive twin pregnancies were evaluated by ultrasound to establish criteria for antenatal detection of discordant fetal growth. For each fetus an attempt was made to measure the biparietal diameter (BPD), abdominal circumference, and femur length; the estimated fetal weight was also calculated based on published formulas. The intrapair differences in BPD, abdominal circumference, femur length, and estimated fetal weight were evaluated as predictors of discordant fetal growth. Although the intrapair difference in BPD measurement was not a statistically significant predictor, an intrapair difference in abdominal circumference of 20 mm or more was found to have sensitivity 80%, specificity 85%, positive predictive value 62%, and negative predictive value 93%. Intrapair difference in the estimated fetal weight was found to be the best predictor of discordant fetal growth (sensitivity 80%, specificity 93%, positive predictive value 80%, and negative predictive value 93%). These data suggest that the intrapair difference in abdominal circumference measurement could be effectively used as a screening test for the diagnosis of discordant fetal growth. If the intrapair difference in abdominal circumference is 20 mm or greater, further evaluation, by determining the intrapair difference in estimated fetal weight, is indicated.

Antenatal evaluation and management of ultrasonically detected fetal anomalies.

With the advent of improved ultrasound imaging, it is now possible to make an intrauterine diagnosis of many fetal anomalies. The key to an accurate antenatal diagnosis is careful scanning of the fetus and knowledge of the abnormalities that may be associated with a particular anomaly. In the presence of fetal anomalies known to be associated with increased frequency of chromosome abnormalities, fetal karyotyping is indicated, using either amniocentesis or fetal blood sampling, depending upon the urgency of the diagnosis. Fetal echocardiography is mandatory when the ultrasonically detected fetal anomaly is one that is known to be associated frequently with cardiac disease. Based on the antenatal findings, the parents should receive appropriate genetic and perinatal counseling. The management plan should always take into consideration the parents' wishes. Management plans for the most common ultrasonically detected fetal anomalies are presented, based upon review of the literature and authors' experience.

The relationship between fetal biophysical profile and cord pH in patients undergoing cesarean section before the onset of labor.

In a prospective study of 124 patients undergoing cesarean section before the onset of labor, the fetal biophysical profile was found to have a significant relationship with umbilical cord blood pH (artery and vein). Using cord arterial pH less than 7.20 as a standard for the diagnosis of fetal acidosis, the sensitivity, specificity, and positive and negative predictive values of the fetal biophysical profile score were 90, 96, 82, and 98%, respectively. When the combination of nonreactive nonstress test and absent fetal breathing was used as the "abnormal test," the sensitivity, specificity, and positive and negative predictive values were 100, 92, 71, and 100%, respectively. The first manifestations of fetal acidosis are nonreactive nonstress testing and loss of fetal breathing; in advanced acidemia, fetal movements and fetal tone are compromised. A protocol of antepartum fetal evaluation is suggested based upon the individual biophysical components rather than the score alone.

Perinatal management and outcome of fetal ventriculomegaly.

In 20 consecutive cases of fetal ventriculomegaly, diagnosed by antenatal ultrasound examination, hydrocephalus was isolated in six patients (30%) and was associated with other anomalies in 14 (70%). There were no false positive diagnoses of fetal ventriculomegaly in this series. Fetal structural and/or chromosome abnormalities were diagnosed antenatally in 11 of the 14 patients (78.5%) with postnatally documented anomalies. In ten patients (50%), isolated fetal ventriculomegaly or ventriculomegaly associated with spina bifida was diagnosed antenatally, and the perinatal management consisted of frequent ultrasound examinations, weekly fetal biophysical profiles, and delivery by cesarean section after documenting fetal lung maturity. Ventriculo-amniotic shunt placement was not part of the management. The outcomes were induced abortion, four patients (20%); intrapartum death, two patients (10%); postnatal death, five patients (25%); and currently alive, nine patients (45%).

The ultrasound femur length as a predictor of fetal length.

A linear relationship between the ultrasound fetal femur length and the crown-heel length of the fetus is described. The formula for calculating the fetal length in centimeters was found to be 6.18 + 0.59 x femur length in millimeters. The value and potential uses of the calculated length of the fetus are discussed.

Fetal atrial flutter and X-linked dominant vitamin D-resistant rickets.

A pregnancy, complicated by hypophosphatemic familial rickets (vitamin D-resistant rickets) of a female fetus, associated with atrial flutter and congestive heart failure is presented. Upon review of the literature, only 22 cases of fetal atrial flutter have been reported. The association between hypophosphatemic familial rickets and atrial flutter has not yet been described.

Degree of oligohydramnios and pregnancy outcome in patients with premature rupture of the membranes.

Amniotic fluid volume was serially assessed by real-time ultrasound in 90 patients who presented with premature rupture of the membranes (PROM) and not in labor. The degree of oligohydramnios was correlated to the outcome of pregnancy, as reflected by pregnancy prolongation, intrapartum fetal heart rate patterns consistent with umbilical cord compression, cesarean section rate, fetal distress, infection, and perinatal mortality rate. These data suggest that in patients with PROM the degree of oligohydramnios is positively correlated with unfavorable pregnancy outcome.

The nonstress test.

Antepartum fetal evaluation by heart rate monitoring was carried out on a clinical service during a 1-year period utilizing the nonstress test as the primary screening device. Nonreactive nonstress tests were supplemented by the oxytocin contraction stress test. In this study, the use of the nonstress test appropriately led to the identification of all fetuses who were in jeopardy and, in the majority of instances, identified fetuses in good condition who did not require the administration of oxytocin to determine fetal well-being. In our experience, the nonstress test is a reliable, inexpensive, convenient, time-saving screening procedure which can be made available to a greater number of patients than the contraction stress test alone.

Effects of ritodrine hydrochloride on uterine activity and the cardiovascular system in toxemic patients.

The effects of ritodrine hydrochloride were evaluated in 25 toxemic patients in active labor utilizing continuous electronic monitoring of fetal and maternal cardiovascular systems and uterine activity. Fetal scalp blood and free flowing maternal antecubital venous blood was obtained for pH, Po2, Pco2, base deficit and blood glucose determinations prior to and immediately following the study period. The initial ritodrine dose was 50 mug/min for 15 minutes. The dose was increased by 50 mug/min each 15 minutes until there was a clinically apparent reduction in uterine activity. Once this was accomplished, the infusion was maintained for 30 minutes. There was a consistent increase in the maternal heart rate (MHR) and a significant rise in fetal heart rate (FHR) late in the infusion and in the postinfusion period. There was a widening of the maternal pulse pressure mainly due to a reduction in diastolic pressure with little change in the mean blood pressure. Maternal and fetal pH decreased and base deficit increased during the study although the PO2 and PCO2 remained unchanged. Maternal and fetal blood glucose rose significantly following ritodrine infusion.

Fetal breathing as a predictor of infection in premature rupture of the membranes.

The value of the presence or absence of fetal breathing in predicting infection was determined by a retrospective analysis of 130 patients with premature rupture of the membranes and no clinical signs of infection or labor. The last ultrasound examination performed within 48 hours of delivery was used for comparison to infection outcome, as reflected by the development of clinical amnionitis, possible neonatal sepsis, and neonatal sepsis. The sensitivity and specificity of fetal breathing in predicting infection in patients with premature rupture of the membranes were 91.6 and 64.8%, respectively. These data suggest that the presence of fetal breathing is a good predictor of noninfection outcome (negative predictive value 95.3%), whereas its absence does not necessarily indicate impending infection (positive predictive value 50%).

Fetal biophysical profile in twin gestations.

The fetal biophysical profile (nonstress test, fetal breathing movements, fetal movements, fetal tone, amniotic fluid volume, placental grading) was assessed in 49 consecutive referred high-risk patients with twin gestations. The relationship between the last fetal biophysical profile score before delivery was compared with the pregnancy outcome--as reflected by the presence of fetal distress and perinatal death. These data suggest that the fetal biophysical profile is a useful tool for observing fetal status in patients with twin gestations, and could be reliably used as a means of follow-up of nonreactive nonstress testing in these patients.

Fetal malignant melanoma: ultrasound presentation and review of the literature.

Malignant melanoma is an infrequently described type of congenital neoplasm. We report a case of primary fetal malignant melanoma. A review of the literature yielded six previous cases, four of which were due to metastasis from advanced maternal disease, and two of which were fetal (primary) in origin. Recommendations are discussed for management of this rare fetal malignancy.

The fetal biophysical profile and its predictive value.

Six fetal biophysical variables--the nonstress test, fetal movements, fetal breathing movements, fetal tone, amniotic fluid volume, and placental grading (biophysical profile)--were assessed in 150 high-risk pregnancies during a 30-minute observation period. The predictive value of the nonstress test alone, biophysical scoring, and contraction stress test alone in the identification of the healthy fetus as well as the fetus in jeopardy are discussed. The relationships between individual variables and combinations of variables to the outcome of pregnancy, as reflected by abnormal intrapartum fetal heart rate patterns, meconium during labor, fetal distress, and perinatal mortality rate were determined. The biophysical profile of all hypoxic fetuses was analyzed. These data suggest that the biophysical profile is more accurate in the identification of the hypoxic fetus than any other single method; therefore, a new protocol including the biophysical profile for antepartum fetal evaluation is presented.

Single ventricle in pregnancy: case report and review of the literature.

A pregnancy complicated by cyanotic heart disease due to single ventricle was recently managed at the authors's institution. Review of the literature showed only five previous case reports. The management of this uncommon disorder is presented along with a review of the literature. The presence of pulmonary hypertension appears to be a major determinant in assessing maternal risk.

Qualitative amniotic fluid volume versus amniocentesis in predicting infection in preterm premature rupture of the membranes.

Qualitative amniotic fluid volume assessment and amniocentesis were performed on admission in 54 patients who presented with premature rupture of the membranes and no clinical signs of infection or labor. Comparison of these two methods in predicting infection outcome--as reflected by the development of clinical amnionitis and/or neonatal sepsis--suggests them to have the same efficacy in predicting infection outcome in patients with premature rupture of the membranes. Qualitative amniotic fluid volume was found to have sensitivity 50%, specificity 92.8%, positive predictive value 66.6%, and negative predictive value 86.6%; amniocentesis was found to have sensitivity 58.3%, specificity 88%, positive predictive value 58.3%, and negative predictive value 88%. The use of ultrasonically estimated amniotic fluid volume could replace or be used in addition to amniocentesis in identifying patients with ruptured membranes at particular risk for developing infection.

Value of fetal ponderal index in predicting growth retardation.

Fetal ponderal indexes were calculated by ultrasound examination and compared with the neonatal ponderal indexes in 113 pregnancies. The relationship between the fetal and neonatal ponderal indexes throughout gestation is described. The fetal ponderal index also was evaluated as a predictor of intrauterine growth retardation (IUGR) and was found to have sensitivity and specificity of 76.9 and 82%, respectively. These data suggest that the fetal ponderal index could be used to rule out the diagnosis of IUGR with reasonable accuracy (negative predictive value 96.4%).

Prenatal detection of a neural tube defect after fetal exposure to valproic acid.

Fetal exposure to valproic acid has recently been associated with an increased incidence of neural tube defects. The prenatal detection of a fetus with both hydrocephalus and meningomyelocele after valproic acid exposure is presented and specific recommendations made for antepartum evaluation in future pregnancies.

Fetal biophysical profile and the effect of premature rupture of the membranes.

A retrospective study of 1151 fetal biophysical profiles and scores associated with good pregnancy outcome was conducted over a three-year period in the author's institution. Normal fetal biophysical activities and scores were determined throughout gestation from 25 to 44 weeks in patients with intact membranes, and compared with profiles and scores of a group of patients with premature rupture of the membranes and good pregnancy outcome. These data suggest that although the biophysical scoring of the healthy fetus with intact membranes does not change significantly throughout gestation, some of the fetal biophysical variables (nonstress test, fetal breathing movements, amniotic fluid volume, and placental grading) do. The rupture of membranes was found to be associated with higher incidence of reactive nonstress testing, absence of fetal breathing, and reduced amniotic fluid volume in most gestational ages; however, the overall biophysical scoring of the healthy fetus was not altered throughout gestation by the presence of ruptured membranes.