Antenatal sonographic findings in a case of intra-abdominal pulmonary sequestration.

This report describes the antenatal sonographic identification of a hyperechoic intra-abdominal mass in a 30-week fetus. The mass was proved by autopsy to be an extralobar pulmonary sequestration. Although a rare type of congenital malformation, pulmonary sequestration should be included in the differential diagnosis of an echodense intra-abdominal mass seen during antenatal ultrasound examination.

Autosomal recessive Robinow-like syndrome with anterior chamber cleavage anomalies.

We describe 2 sisters with short stature, mesomelic brachymelia, macrocephaly, hypoplastic genitalia, and anterior chamber cleavage anomalies. Many of their manifestations have been described in individuals with Robinow syndrome; however, the anterior chamber cleavage anomalies seen in both girls, hydrocephalus seen in the younger sister, and apparent autosomal recessive inheritance do not characterize the Robinow syndrome. The syndrome present in these sisters most likely represents a previously undescribed autosomal recessive syndrome.

Cytogenetic evaluation of fetal death: the role of amniocentesis.

Fetal death can be associated with chromosomal abnormalities. Because of the degree of tissue maceration and autolysis seen in stillborn fetuses, it is often impossible to successfully culture these tissues for cytogenetic studies. We performed genetic amniocentesis in four cases of fetal death and were successful in obtaining cytogenetic results in all four, whereas the culture of fetal tissues for cytogenetics was successful in only one case. Chromosomal abnormalities were found in three of the four cases, including two fetuses with Down's syndrome and one fetus with Turner's syndrome. Because of the importance of cytogenetic studies in most cases of fetal death, we recommend amniocentesis at the time of diagnosis rather than waiting for delivery of fetal tissues, when postmortem changes may make it impossible to successfully culture fetal cells.

Preterm premature rupture of the membranes: a risk factor for the development of abruptio placentae.

A retrospective study of 298 patients with preterm premature rupture of the membranes managed expectantly during a 3-year period investigated the association between preterm premature rupture of the membranes and abruptio placentae. Expectant management was associated with the development of abruptio placentae in 19 of these 298 patients (6.3%). The prevalence of abruptio placentae in the entire population during the same 3-year period was 2.7%, whereas in patients without preterm premature rupture of the membranes it was 2%. None of the patients developed clinical or laboratory evidence of disseminated intravascular coagulation and no infection (maternal or neonatal) was noted among the patients who had abruptio placentae. Patients with preterm premature rupture of the membranes and severe oligohydramnios (largest pocket less than 1 cm) seem to be at particular risk for developing this complication. These data suggest that abruptio placentae should be considered as one of the possible risks of expectant management in preterm premature rupture of the membranes.

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.

Congenital midline porencephaly. Prenatal sonographic findings and review of the literature.

A case of congenital midline porencephaly, including the prenatal sonographic findings, is presented. A review of the literature showed that six cases of congenital midline porencephaly have been reported, though none had sonographic evaluation in the prepartum period. The prenatal sonographic diagnosis of this rare disorder is discussed along with the clinical and pathologic findings and outcome of all reported cases.

Pregnancy management and successful outcome of Ehlers-Danlos syndrome type IV.

Ehlers-Danlos syndrome type IV (EDS IV) is a rare but serious disorder of connective tissue that is characterized by the abnormal production or secretion of type III collagen. Experience with pregnant patients exhibiting this syndrome is limited. This report describes the successful management of a pregnancy in a woman with EDS IV that included activity restriction and complete bed rest after 32 weeks, use of 1-desamino-8-D-arginine vasopressin (DDAVP) before delivery to improve coagulation, and cesarean section before the onset of labor. The possibility of prenatal diagnosis based on genetic linkage studies also is discussed.

The fetal biophysical profile in pregnancies with grade III placentas.

The components of the fetal biophysical profile of pregnancies with grade III placentas and good outcome were retrospectively analyzed and compared to the fetal biophysical components of pregnancies with grade 0 to II placentas. The results of the present study suggest that the dynamic components of the fetal biophysical profile (nonstress test, fetal breathing movements, fetal movements, fetal tone) are not altered in the presence of a grade III placenta and good pregnancy outcome; however, a greater incidence of reduced amniotic fluid volume was found in the presence of grade III placenta. The clinical significance of grade III placenta is discussed.

Hydrops fetalis associated with immunologic and nonimmunologic factors.

Described is a pregnancy complicated by severe Rh sensitization and hydrops fetalis which was managed aggressively with frequent intrauterine transfusions initiated at 21 1/2 weeks of gestation. No improvement or only temporary improvement of the fetal hydrops was observed after each intrauterine transfusion. Postmortem examination of the infant revealed the additional presence of nonimmunologic factors involved such as pulmonic atresia and lung hypoplasia. In cases of severe Rh sensitization associated with persistent fetal hydrops despite frequent intrauterine transfusions, the presence of coexistent factors for nonimmunologic fetal hydrops should be considered.

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.

The use and misuse of the fetal biophysical profile.

The value of the fetal biophysical profile in determining fetal well-being has been well documented. The increasing clinical use of the fetal biophysical profile, however, has led to the recognition of frequent errors in the interpretation and application of this modality. These errors may result in unnecessary interventions or adverse perinatal outcome. The most frequent errors in the interpretation and application of the fetal biophysical profile are described; in an attempt to minimize the misuse of the fetal biophysical profile, an alternative protocol of antepartum fetal evaluation is suggested based upon the information obtained from the biophysical monitoring of the fetus.

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%).

Fetal biophysical profile versus amniocentesis in predicting infection in preterm premature rupture of the membranes.

A comparison between daily fetal biophysical profile determinations and amniocentesis (for Gram stain and culture) was studied prospectively in 58 patients who presented with preterm premature rupture of the membranes and no apparent infection or labor. The efficacy of these two methods in predicting infection outcome--as reflected by the development of clinical amnionitis and/or neonatal sepsis--was determined. These data suggest that daily fetal biophysical profile assessment is superior to amniocentesis in predicting infection outcome in these patients. The use of frequent biophysical profile determinations should replace amniocentesis in selecting those patients with premature rupture of the membranes who are more likely to develop infection with associated fetal/neonatal sepsis.

Telephone transmission of fetal heart rate monitor data. The experience at the University of Connecticut Health Center.

This report reviews the experience at the University of Connecticut Health Center using the Fetal Assessment Consultative Transmission Service (FACTS) system during a 2 1/2-year period. This system, which permits direct transmission of antepartum and/or intrapartum fetal heart rate tracings via a telephone line, allows the obstetric staffs of smaller community hospitals to obtain an immediate consultation from the University of Connecticut Health Center on a 24 hour per day basis. A total of 511 fetal heart rate tracings were analyzed. Two hundred forty-five were intrapartum, 206 antepartum, and 60 were transmitted for educational purposes. The results indicate an imperative need for such a service from a tertiary care center to improve the quality of regional perinatal care and to determine the future direction of the regional educational program for physicians and nurses.

Amniocentesis with premature rupture of membranes.

Transabdominal amniocentesis was used in 73 consecutive, referred patients with premature rupture of membranes and no clinical signs of infection or labor. Positive Gram stain or positive culture of obtained amniotic fluid was used as an indication for delivery. The outcome of pregnancy in this group of patients was compared with the outcome of a historic control group of 73 patients managed conservatively without amniocentesis. Clinical amnionitis and low five-minute Apgar scores occurred significantly less often in the study than in the control group; however there was no difference in the frequency of neonatal infection, one-minute Apgar score, cord blood pH, or perinatal mortality between the two groups.

The use of the nonstress test in patients with premature rupture of the membranes.

The value of the nonstress test in predicting the outcome of infection was determined by a retrospective analysis of 127 consecutive high-risk patients who presented with premature rupture of the membranes and no clinical signs of infection or labor. The last study performed within 48 hours of delivery was used for comparison with the outcome of pregnancy. The relationship between nonstress test results and the outcome of pregnancy, as reflected by the development of clinical amnionitis and/or neonatal sepsis, was determined. The sensitivity and specificity of the nonstress test in predicting infection outcome in patients with premature rupture of the membranes were 78.1% and 86.3%, respectively. These data suggest that the nonstress test is a useful tool for evaluating patients with premature rupture of the membranes.

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 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.

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.