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Myotonic muscular dystrophy associated with ritodrine tocolysis.

A patient thought to be normal was admitted with premature labor at 29+ weeks' gestation. Treatment with the beta-mimetic ritodrine hydrochloride appeared to provoke symptoms of myotonic muscular dystrophy. Neurological history and examination confirmed the presence of previously unsuspected myotonic dystrophy in the patient, her father, and paternal grandfather. Discontinuation of the drug led to improvement in myotonia symptoms but worsening premature labor. Magnesium sulfate did not provoke the same symptoms but was unsuccessful in controlling premature contractions. Long-term management with bed rest, phenytoin, and isoxsuprine hydrochloride resulted in term delivery. Subsequently, maternal symptoms of myotonia disappeared. Congenital myotonia was suspected in the fetus because of the ultrasonic demonstration of polyhydramnios and reduced fetal movements. This was confirmed at delivery. The mechanism by which ritodrine unmasked the myotonia is unclear but may be related to changes in the cell membrane (chloride conductance, the sodium-potassium pump, or membrane polarization).

Adult↗

Fetal diaphragmatic hernia: ultrasound diagnosis and clinical outcome in 19 cases.

Nineteen cases of congenital diaphragmatic hernia diagnosed in utero are reported with emphasis on sonographic findings, associated congenital and karyotypic abnormalities, the presence or absence of polyhydramnios, and clinical outcome. The survival of these infants was very poor despite accurate prenatal diagnosis, maximal surgical and medical treatment and maximal postnatal care. The overall survival rate was 10.5%, and for fetuses who lived beyond delivery the survival rate was 20%.

Female↗

Fryns syndrome: a predictable, lethal pattern of multiple congenital anomalies.

Fryns syndrome is a unique pattern of lethal multiple congenital malformations with variable expression. A family in which all four sibs conformed to Fryns syndrome is detailed and substantiates the criteria for definition of the syndrome; perinatal mortality, hypoplastic lungs, and facial deformities should be highly suggestive of the syndrome. The addition of a strong family history, diaphragmatic hernias, distal limb deformities, and early onset of polyhydramnios with subsequent premature delivery should definitely confirm the diagnosis.

Abnormalities, Multiple↗

Quantitation of uterine activity preceding preterm, term, and postterm labor.

To assess uterine activity before labor in patients delivering preterm, at term, and postterm, the maximum spontaneous contraction frequency per 10-minute window during the initial portion of antepartum fetal heart rate monitoring was analyzed. Patients with multiple gestation, third trimester bleeding, polyhydramnios, or premature rupture of membranes and those already diagnosed with preterm labor were eliminated from the study. Of the 2446 remaining patients (7247 antepartum fetal heart rate tests) who went into spontaneous labor, 237 did so before 37 completed weeks of gestation, 1077 entered labor at term (38 to 42 completed weeks), and 1132 did so after 42 weeks. There was a significant increase in maximum uterine activity per 10-minute window from 30 to 44 weeks of gestation (average 4.7% per week; r = 0.97, p less than 0.0001). When compared with patients delivering spontaneously at term, average maximum uterine activity per 10-minute window was greatest in the preterm labor group (p less than 0.05) and least in the postterm labor group (p less than 0.05). These differences were present for several weeks preceding the onset of spontaneous labor. All three groups showed a surge of uterine activity during the 3 days before the onset of spontaneous labor.

Female↗

Sonographically estimated fetal weights: accuracy and limitation.

To determine the accuracy of our ultrasonographically predicted birth weights we studied 1301 women delivered of infants within a week of an obstetric ultrasonogram to compare the ultrasonographically predicted birth weights with the actual birth weights. The fetuses varied from 700 to 5800 gm and were consecutive singleton fetuses in vertex presentations delivered at a single institution. Overall 74% of the infants had birth weights within 10% of the ultrasonographic estimates and 42% had birth weights within 5% of the ultrasonographic estimates. The presence of oligohydramnios or polyhydramnios made no difference in the percent errors. The sensitivity for identifying a fetus with macrosomia (birth weight greater than 4000 gm) with an estimated weight of greater than or equal to 4000 gm was 65%. The specificity or percent of fetuses correctly identified ultrasonographically as not macrosomic was 90%. If fetuses predicted by ultrasonography to be greater than 3800 gm were included, the sensitivity for the prediction of macrosomia rises to 82% but the specificity would be 79%. There appears to be a fixed limitation to obtaining estimated fetal weights by ultrasonography, even in large series, because these data reaffirm the success and limitations of other methods used to estimate fetal weight previously reported in the literature.

Birth Weight↗

Doppler assessment of the pulsatility index of the middle cerebral artery during constriction of the fetal ductus arteriosus after indomethacin therapy.

To investigate the effects of constriction of the ductus arteriosus on the pulsatility index of the middle cerebral artery, maximum velocity waveforms were obtained in 13 fetuses (one set of twins) whose mothers were treated with indomethacin for preterm labor (n = 9) or polyhydramnios (n = 3). Eleven of the fetuses manifested ductal constriction within 48 hours of therapy, whereas two fetuses had constriction after 1 week of therapy. Six of the 13 fetuses also manifested tricuspid insufficiency in association with constriction of the ductus arteriosus. All abnormal cardiac changes resolved in utero after discontinuation of indomethacin. No difference in the pulsatility index values of the middle cerebral artery was observed in the fetuses with ductal constriction but without tricuspid regurgitation (n = 7) when the values were compared with those obtained in absence of ductal constriction (1.87 +/- 0.37 vs. 1.88 +/- 0.33). In the fetuses that manifested both ductal constriction and tricuspid insufficiency (n = 6), the pulsatility index values in the middle cerebral artery were significantly lower in the presence of ductal constriction when compared with the values obtained in the absence of ductal constriction (2.22 +/- 0.26 vs. 1.57 +/- 0.34). These results indicate that a response to indomethacin sufficient to cause both ductal constriction and tricuspid insufficiency decreases the pulsatility index of the middle cerebral artery.

Blood Circulation↗

The effect of indomethacin on the pulsatility index of the umbilical artery in human fetuses.

Pulsed and continuous-wave Doppler ultrasonography was used to assess blood velocities in the ductus arteriosus and the pulsatility index of the umbilical artery in 20 fetuses (1 pair of twins and 18 singletons) at 23 to 33 weeks' gestation during maternal indomethacin therapy for preterm labor or polyhydramnios. There was no statistically significant difference in the pulsatility index of the umbilical artery during maternal indomethacin treatment (mean +/- SD, 1.11 +/- 0.20) when compared with baseline values (1.12 +/- 0.19) in all 20 fetuses studied. An increase in the peak systolic and diastolic blood velocities in the ductus arteriosus consistent with ductal constriction was noted in 9 fetuses. Analysis of the umbilical artery pulsatility index values before and during indomethacin in this subgroup of fetuses failed to reveal a statistically significant change (1.12 +/- 0.19 vs. 1.11 +/- 0.20). Indomethacin therapy does not influence the pulsatility index of the umbilical artery at the gestational ages studied.

Blood Flow Velocity↗

Twin gestation: influence of placentation on fetal growth.

To study fetal growth in twin gestation, morphometric autopsy data of 52 midgestation twin pairs who were stillborn or who died less than or equal to 24 hours after birth were analyzed. Twins were divided into three groups: (1) monozygotic: diamniotic, monochorionic placenta (n = 18); (2) dizygotic: diamniotic, dichorionic placenta, unlike sex (n = 12); (3) like-sex: placenta diamniotic, dichorionic in 63.6%, unknown in 36.4% (n = 22). The monozygotic group had a significantly higher rate of growth discordance, which was defined as a greater than 20% difference in body weight (monozygotic 72.2%, dizygotic 16.7%, like-sex 0%), and polyhydramnios (monozygotic 50%, dizygotic 0%, like-sex 9.1%). Organ weight z scores for body weight and brain weight standards were calculated for the smaller and larger of each twin pair. In the monozygotic group highly significant z scores were obtained for brain weight in the smaller twin (z = 2.71, p = 0.003, body weight standards) and heart weight in the larger twin (body weight standards, z = 3.87, p less than 0.001; brain weight standards, z = 3.64, p less than 0.001). We conclude that monozygotic twins with diamniotic, monochorionic placentation have a high degree of brain-sparing growth restriction in the smaller twin and cardiac hyperplasia in the larger twin, most likely caused by hemodynamic inequalities.

Adolescent↗

Transplacental, amniotic, urinary, and fetal fluid dynamics during very-large-volume fetal intravenous infusions.

With rapid intravenous infusion of very large volumes of isotonic saline solutions into the fetus, the fluid could stay within the fetal body, thereby creating hydrops fetalis, be transferred into the amniotic fluid through the fetal kidneys, thereby creating polyhydramnios, or be transferred across the placenta into the maternal circulation. This study was designed to explore these possibilities. After a 1-hour control period, 10 near-term chronically catheterized ovine fetuses were infused intravenously with 4 L (greater than 100% of fetal weight) of either isotonic saline solution or lactated Ringer's solution over 4 hours. Fetal arterial pressure was significantly elevated by 7 mm Hg throughout the infusion (p less than 0.00001). Venous pressure underwent a transient rise (4.8 mm Hg) at 20 minutes of infusion and remained elevated (2.7 mm Hg) during the rest of the infusion (p less than 0.00001). Fetal urine flow increased by an average of 5.7 +/- 0.4 ml/min throughout the infusion (p less than 0.00001) and accounted for 34.1% +/- 2.6% of the infused volume. Estimated fetal extracellular fluid volume increased by 17.7% +/- 1.8% of the infused volume. Because fetal fluid retention, urine flow, and amniotic fluid volume changes accounted for only half of the infused fluid, the remainder of the infused volume must have crossed the placenta and entered the maternal circulation. Given the above changes in vascular pressures, this requires a filtration coefficient of the placenta 50 to 100 times the previously reported values. Thus we conclude that relatively small changes in fetal vascular pressures dramatically alter the filtration capacity of the ovine placenta and transplacental volume flow.

Amniotic Fluid↗

Birth weights of infants of black and white mothers without pregnancy complications.

An analysis of 10,159 normal spontaneous vaginal deliveries was performed to examine racial differences in mean birth weight of infants whose mothers were without antepartum or intrapartum medical complications of pregnancy. The study was limited to black and white infants of low-income mothers who were inborn, singleton, and weighed greater than or equal to 500 gm at birth. High-risk maternal transfer patients and patients with hypertension, toxemia, bacteriuria, pyelonephritis, renal failure, diabetes, anemia, polyhydramnios, oligohydramnios, prolapsed cord, vaginal bleeding, placenta previa, abruptio placentae, prolonged rupture of membranes, maternal fever on admission, amnionitis, sexually transmitted diseases, or fewer than five prenatal care visits were excluded. When statistically significant differences in demographic characteristics were controlled, black infants had an average birth weight 181 gm less than that of white infants.

Birth Weight↗

Therapeutic amniocentesis in twin-twin transfusion syndrome appearing in the second trimester of pregnancy.

Amniocentesis and drainage were performed in 19 twin pregnancies initially seen at less than 28 weeks' gestation with acute polyhydramnios caused by the twin-twin transfusion syndrome. The procedure was performed an average of three times for each patient (range one to six), with the removal of a mean volume of amniotic fluid of 2600 ml on each occasion (range 500 to 6700 ml). The interval between initial examination and delivery averaged 7 weeks (range 1 day to 18 weeks); 14 (37%) of the 38 babies survived the neonatal period. In the pregnancies with poor outcomes, the intertwin disparity in fetal size and the mean volume of amniotic fluid drained at each amniocentesis were greater than in the pregnancies in which one or both twins survived.

Abortion, Spontaneous↗

Massive congenital intracranial teratoma with skull rupture at delivery.

The majority of case reports concerning intracerebral tumors are almost equally divided between teratomas and gliomas. We report a case of congenital intracranial teratoma with suggested management. An 18-year-old woman at 30 weeks' gestation in active labor because of severe fetal hydrocephalus and polyhydramnios was delivered by cesarean section. Spontaneous rupture of the neonatal skull led to delivery of a stillborn infant; a congenital teratoma was found. Although intracranial teratoma is a well-recognized entity in the differential diagnosis of pediatric brain tumors, massive congenital teratoma replacing cerebral hemispheres of a fetus has been seldom reported. Antenatal diagnosis of the neoplasm and abdominal delivery may not improve outcome.

Adolescent↗

Associations between adverse perinatal outcome and serially obtained second- and third-trimester maternal serum alpha-fetoprotein measurements.

OBJECTIVE: Our purpose was to determine whether third-trimester maternal serum alpha-fetoprotein predicts adverse perinatal outcome and whether use of both second- and third-trimester maternal serum alpha-fetoprotein enhances the positive predictive value for various abnormal outcomes. STUDY DESIGN: A cohort study with obstetric outcome assessed by chart analysis after delivery was performed at Regional Medical Center (Memphis, Tennessee), a hospital staffed by university-based physicians serving a large urban population with many indigent patients. A total of 650 women attending prenatal clinics in the above setting with a singleton pregnancy without a neural tube defect, contributing a maternal blood samples in both the second and third trimesters, and delivered in the above hospital participated. Various pregnancy outcomes were correlated with maternal serum alpha-fetoprotein levels in the second and third trimesters and in both. RESULTS: In the third trimester no significant associations were found between maternal serum alpha-fetoprotein elevations and pregnancy complications. In the second trimester elevation ( > or = 2.0 multiples of the median) were, by contrast, significantly associated with preterm premature rupture of the membranes, preterm birth, and low birth weight. No association was found with certain other complications. When second-trimester data were grouped according to the types of complications occurring in individual women, only preterm premature rupture of the membrane proved statistically significant. CONCLUSIONS: Second-trimester but not third-trimester maternal serum alpha-fetoprotein is significantly elevated with preterm premature rupture of the membranes, preterm birth, and low birth weight; in this cohort study no association was found with preeclampsia, oligohydramnios, or polyhydramnios.

Cohort Studies↗

Diagnosis and management of fetal sacrococcygeal teratoma.

Four cases of fetal sacrococcygeal teratomas were managed in Kuwait Maternity Hospital in the previous 2.5 years, making an incidence of 1 in 10,000 deliveries. Three were diagnosed antenatally and one unbooked case presented during labor with dystocia. Polyhydramnios and characteristic ultrasonic tumor echogenicity were documented. Two patients were delivered vaginally after partial excision of the tumor through hysterotomy. In all cases female infants were delivered. Tumor size dictated the mode of delivery being vaginal in cases with maximum tumor diameter less than 10 cm. A single infant survived after tumor excision done 48 h after delivery. All tumors were histopathologically benign. Routine antenatal ultrasonic examinations should allow early diagnosis of such cases to avoid unanticipated dystocia. In the absence of associated major anomalies amniocentesis may relieve maternal symptoms, when necessary, and postpone onset of premature labor, vaginal delivery should be allowed in cases with maximal tumor diameter less than 10 cm. This should be conducted in a center with experienced neonatal and pediatric surgical care.

Adult↗

Prenatal sonographic diagnosis of thanatophoric dwarfism.

A case of thanatophoric dwarfism is reported in a 35-year-old woman. The diagnosis was made at 34 weeks gestation by ultrasound. Marked shortening of the extremities, macrocephaly, narrow thorax and polyhydramnios were observed. The importance of routine ultrasound screening in pregnancy is emphasized, since such malformations can be detected prenatally.

Adult↗

Incidence and contribution of predisposing factors to transverse lie presentation.

OBJECTIVES: Reassessment of predisposing factors to transverse lie presentation. METHODS: Retrospective analysis of delivery data on 92 women with transverse lie presentation, using ultrasonography, and 92 randomly chosen control vertex deliveries. RESULTS: The association between transverse lie and multiparity, prematurity, placenta previa, polyhydramnios and uterine anomalies is reaffirmed. Predisposing factors were found in 66% of primiparas but in only 33% of multiparas. CONCLUSIONS: Known predisposing factors to transverse lie presentation withstood a closer and more accurate assessment of their incidence.

Adult↗

Intrauterine volvulus without malrotation.

This report describes two cases of intrauterine volvulus without associated intestinal malrotation. Polyhydramnios was present in one neonate; the presentation was very sudden and catastrophic in the other. Neither plain films nor contrast enema indicated the correct preoperative diagnosis in these infants. A "normal" plain film or enema in a suspected case of volvulus may lead to a delay in surgical management resulting in extensive ischemic necrosis of the bowel, often with extremely high mortality. Bile emesis or aspirate in neonates demands a high index of suspicion of intestinal obstruction. Shock, bloody diarrhea, and tense and distended abdomen are ominous, indicating volvulus often with gangrene.

Female↗

Coagulopathy associated with large sacrococcygeal teratomas.

Twelve neonates with sacrococcygeal teratoma (SCT) have been treated at British Columbia Children's Hospital over the past 5 years. Clinically significant coagulopathy developed in four of these neonates and two died, one before surgical intervention could be undertaken. Disseminated intravascular coagulation (DIC) was found in one patient and thrombocytopenia in another on preoperative laboratory studies. Etiology of the coagulopathy is unclear, but appears to be multifactorial. Although several clinical reviews have noted mortalities due to exsanguinating hemorrhage, no study has focused solely on this issue. The diagnosis of SCT in the neonate at high risk for development of coagulopathy is usually made prenatally. Premature labor is often precipitated by associated polyhydramnios and large tumor size. Fetal distress, prematurity, and low birth weight are common. Presence of placentamegaly, hydrops fetalis, and congestive heart failure are ominous prognostic signs. Early identification of patients at increased risk for development of hemorrhagic complications may allow optimization of their management. Cesarean section should minimize trauma to the SCT during delivery. Expeditious resection of the lesion may improve survival.

Blood Coagulation Tests↗