Caroli's disease complicating pregnancy.

We describe two pregnancies in the same patient with Caroli's disease (congenital dilatation of the intrahepatic bile ducts). The first pregnancy was uneventful concerning the maternal Caroli's disease. The second pregnancy highlights the serious and potentially lethal complications of an acute episode to both the fetus and mother. The patient had acute ascending cholangitis, disseminated intravascular coagulopathy, and septic shock. Fetal distress necessitated delivery by cesarean section. The mother's postoperative course required prolonged critical care and interdisciplinary consultation. Both mother and neonate survived. We present the first report of Caroli's disease in pregnancy and review implications to the pregnant patient.

Rh sensitization after third-trimester fetal death.

Three primigravidas developed Rh sensitization after unexplained third-trimester fetal death. One patient manifested sensitization after the diagnosis of fetal death had been made but before delivery occurred. The other two demonstrated anti-D antibodies early in the next pregnancy, despite having received postpartum Rh immunoglobulin. Unsensitized Rh-negative women with unexplained third-trimester fetal death should be screened routinely for fetomaternal hemorrhage when fetal death is discovered, so that adequate prophylaxis against Rh sensitization can be given.

Interstitial deletion of chromosome 1 [del(1)(q25q32)] in an infant with prune belly sequence.

Relatively few cases of deletion 1q have been reported. These cases have been divided into three groups according to assigned breakpoints. They include proximal interstitial, intermediate interstitial, and terminal deletions. We present a male infant with an interstitial deletion of 1q with breakpoints determined by GTG banding as q25 and q32. Comparison with similar case reports suggests common physical features which include microcephaly, growth retardation, developmental delay, clinodactyly, and genital anomalies in affected males. However, no characteristic phenotypic appearance is definable. The infant also presented with prune belly sequence (PBS) with Potter facies. Fetal ascites, as noted in this case on prenatal ultrasound, appears to be an early factor in the pathogenesis of PBS. Therefore, detection of fetal ascites should suggest the presence of the PBS association and the need for more extensive prenatal evaluation.

Sonographic detection of fetal urinary-tract anomalies.

The diagnosis of urinary-tract anomalies in the fetus can frequently be made with antenatal ultrasound imaging. This report details 19 cases of fetal urinary-tract malformation diagnosed during a 30-month period. Early onset and more complete urinary-tract obstruction, oligohydramnios, renal dysplasia, and pulmonary hypoplasia occur frequently in cases where urinary-tract anomalies were detected in utero. The frequent association of other major structural anomalies, severe renal damage, and irreversible pulmonary hypoplasia make imperative an intensive evaluation of the fetus before considering invasive surgical procedures.

A sonographic artifact, fetal hair, mimicking a craniocervical meningocele in pregnancy complicated by hydramnios. A report of two cases.

The use of ultrasound in clinical obstetrics has provided a great opportunity to diagnose fetal developmental defects while the fetus is still in utero. Two cases of ours illustrate how an unusual sonographic artifact, fetal hair, can suggest abnormal fetal development.

Clinical utility of the relationship between fetal femur length and biparietal diameter.

The relationship between fetal femur length and biparietal diameter on ultrasound examination was evaluated in a large, unselected obstetric population of 12-40 weeks' gestational age. The derived ratio of fetal femur length to biparietal diameter was evaluated retrospectively as a tool for examining the technical reliability of fetal measurements obtained and as a screening mechanism for detecting asymmetric fetal development. The ratio of femur length to biparietal diameter is of value as an aid to the detection of fetal developmental anomalies and should be a routine parameter considered in the performance of an obstetric ultrasound examination.

Fetal pulmonary maturity in isoimmunized pregnancies.

That the lecithin/sphingomyelin (L/S) ratio correlates very closely with fetal gestational age in the uncomplicated pregnancy is well accepted. The presence of fetal or maternal disease has been found to accelerate or retard the development of fetal lung maturity in certain pregnancies. Although only a very small number of isoimmunized pregnancies has been evaluated in the obstetric literature, there is general acceptance that most isoimmunized pregnancies have delayed pulmonary maturation. Our observation has been that, in mild to moderately severe isoimmunized pregnancies, fetal lung maturation is not delayed. This study was designed to compare fetal pulmonary maturation in pregnancies affected by isoimmunization to that in a control group of unaffected but sensitized pregnancies. The results of this study suggest that pulmonary maturation in the mild to moderately affected pregnancy as measured by the L/S ratio is neither delayed nor accelerated but unchanged when comparison is made to the control group.

Hydramnios: ultrasound diagnosis and its impact on perinatal management and pregnancy outcome.

Ultrasonographic technique allows for noninvasive quantification of amniotic fluid volume in pregnancy. A series of 12 pregnancies with ultrasonographically confirmed hydramnios is presented in detail. Following the diagnosis of hydramnios an intensive system-by-system evaluation of the fetal structures was performed. Developmental defects known to be associated with the production of hydramnios were detected in 83% of these pregnancies. Anomalies detected included abdominal wall defects, abnormalities of the central nervous system, pleural effusions, obstructions of the fetal gastrointestinal tract, and others. The early definition of hydramnios and the subsequent discovery of anomalies associated with increased amniotic fluid volume may lead to alteration in prenatal care, in intrapartum management, and in postdelivery resuscitative measures in these complicated pregnancies. In this series, despite early diagnosis of hydramnios and associated fetal developmental anomalies, complications of the pregnancies directly resultant from hydramnios resulted in a high perinatal mortality rate.

Changes in placental ultrasonic appearance. I. Incidence of Grade III changes in the placenta in correlation to fetal pulmonary maturity.

Placental grading has been proposed as a substitute for amniotic fluid analysis for predicting fetal pulmonary maturity. The efficacy of placental grading was prospectively evaluated by grading the placenta at the time of 174 amniocentesis procedures done for determination of fetal pulmonary maturity. Grade III changes in the placenta were a rare finding that occurred in only 7% of the examinations performed. Placental grading proved to be insensitive in that it predicted pulmonary maturity in only 5% of pregnancies in which amniotic fluid analysis predicted a mature fetus. In addition, placental grading falsely predicted fetal pulmonary maturity in 42% of patients in whom Grade III placental changes were found. These findings suggest that the determination of Grade III changes in the placenta occurs too rarely to be of use in predicting fetal pulmonary maturity in the uncomplicated pregnancy. Incorrect prediction of pulmonary maturity by placental grading in the complicated pregnancy makes this test of little value in the management of high-risk pregnancy.

Changes in placental ultrasonic appearance. II. Pathologic significance of Grade III placental changes.

Changes in placental texture and structure on ultrasonic examination have been suggested as correlating to advancing fetal pulmonary maturity. The association of preterm appearance of Grade III changes in the placenta in the complicated pregnancy suggests that these changes are associated with a decrease in placental function and premature senescence of the placenta. Placentas were routinely graded on all obstetric examinations performed during a 1-year period of time. Those pregnancies in which Grade III changes in the placenta prior to term were reviewed. A high incidence (78%) of perinatal problems were found in association with preterm appearance of Grade III changes. Perinatal complications included maternal hypertensive disorders, intrauterine growth retardation, abruptio placentae, and fetal distress in labor. The high incidence of these disorders in preterm pregnancies with Grade III changes in the placenta suggests that the sonographic changes found reflect placental dysfunction or senescence rather than normal maturational development. The appearance of Grade III changes in the placenta in the preterm pregnancy is suggested as a predictive indicator of potential perinatal problems in late pregnancy.

Clostridium difficile in a pediatric outpatient population.

Clostridium difficile has been implicated as one cause of hospital-acquired diarrhea in children, yet the prevalence of this organism in outpatient children with diarrhea has not been established. Over a 1-year period, 306 outpatient children ranging in age from 2 weeks to 16 years were cultured for C. difficile and potential bacterial pathogens. C. difficile was isolated from 7.0% of patients with diarrhea (12 of 171) and 14.8% of controls with nondiarrheal illnesses (20 of 135). The 32 patients yielding C. difficile were significantly younger than the other study patients. C. difficile was isolated in conjunction with another enteric pathogen in only one case. Antibiotic exposure in the month prior to culture was no different between the 32 positive patients and the overall population. Moreover the patients yielding C. difficile were clinically indistinguishable from the other study patients. C. difficile appears to comprise part of the normal bowel flora in some children beyond the neonatal period and despite a negative history of recent antibiotic usage.

Ultrasonic measurement of femur length as a predictor of fetal gestational age.

Ultrasonic determination of fetal femur length is useful as a parameter in the determination of fetal gestational age. One hundred thirty measurements were made in pregnancies at 14 to 36 weeks' gestation. The measurements were plotted against gestational age, and we found that a strong correlation exists between fetal femur length and fetal gestational age, with 95% confidence limits of plus or minus seven days. This ultrasound parameter of fetal age assessment should be added to the other methods available for accurate dating of pregnancy.

Reproductive failure and cervical incompetence in women exposed to diethylstilbestrol.

Exposure to diethylstilbestrol in utero is recognized to be associated with a wide range of developmental abnormalities in the female genital tract, the most prominent being adenocarcinoma of the vagina. Recently anatomic anomalies have been discovered with increasing frequency. This case illustrates the association of exposure to diethylstilbestrol, cervical incompetence, and poor reproductive history, and stresses the importance of early evaluation.

Oxytocin challenge test results compared with simultaneously studied serum human placental lactogen and free estriol levels in high-risk pregnant women.

Three perinatal health indicators (serum human placental lactogen [hPL] levels, serum free estriol [E3] levels, and OCT) were simultaneously completed in 149 high-risk women who were more than 34 weeks pregnant. There was a significant correlation between the hPL and E3 results (r = 0.28, p less than 0.01). There was a 15.4% positive OCT result rate and the blood hormone values for the negative and positive OCT groups were compared. There was a significantly lower hPL value in the positive OCT group (4.7 +/- S.E.M. 0.4 vs. 6.2 +/- S.E.M. 0.2 micrograms/ml for positive and negative groups, respectively), whereas the free E3 levels were not different in these two OCT groups (14.8 +/- S.E.M. 1.5 vs. 16.3 +/- S.E.M. 0.7 ng/ml for positive and negative groups, respectively). The free E3 value did not help in predicting the OCT result as the low free E3 subgroup had the same frequency of positive OCT result as did the over-all group. There was a highly significant increase in the positive OCT results (42.9%) in women with a low hPL value (p less than 0.01). In those women with an hPL value of 6.0 microgram/ml or more there were only 8% positive OCT's. It is concluded that on hPL test at about 34 weeks of gestation could be used to screen pregnant women, and if the value were low (less than 4.0 microgram/ml) an OCT evaluation test would be indicated, for there is a very high frequency of positive results in that subgroup of women.