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Maternal and fetal factors related to abnormal amniotic fluid.

OBJECTIVE: The objective of this study was to identify maternal and infant characteristics related to alteration of amniotic fluid volume at birth. STUDY DESIGN: A series of 27,145 consecutive malformed newborn infants from the Spanish Collaborative Study of Congenital Malformations (ECEMC) was analyzed. From this total, 3.01% were found to have oligohydramnios and 3.69% were found to have polyhydramnios. RESULTS: As expected, renal/urinary tract and lung defects were associated with oligohydramnios, whereas esophageal and intestinal atresias, neural tube defects, and other central nervous system malformations were associated with polyhydramnios. In addition, other defects such as cardiovascular anomalies, hydrocephaly, and microcephaly were also related to abnormalities of amniotic fluid volume. After excluding the defects whose association to oligo- or polyhydramnios is well recognized, we compared the frequency of different variables among them and with infants with a normal volume of amniotic fluid. In comparison with infants with normal amniotic fluid volume, the groups with oligo- and polyhydramnios had lower birth weight, shorter gestational age and umbilical cord, higher parental ages, and a greater frequency of spontaneous abortions. The differences were more marked for weight in newborn infants with oligohydramnios, and for gestational age, umbilical cord length, number of previous pregnancies, and spontaneous abortions in polyhydramnios cases. Placental weight was lower in oligohydramnios cases than in infants with normal amniotic fluid, and higher in polyhydramnios cases. Parental consanguinity and twinning were more frequent in polyhydramnios. Maternal morbidity was higher in both groups with abnormal amniotic fluid volume, especially for acute diseases such as hypertension, diabetes mellitus, and gestational diabetes. Chromosomal aberrations were more frequent in the oligo- and polyhydramnios groups than in cases with a normal volume of amniotic fluid, which supports the suggestion of performing prenatal cytogenetic analysis in any pregnancy complicated by an abnormal volume of amniotic fluid. CONCLUSION: The fact that all of these results are similar in the control group of healthy infants suggests that at least some of the variables associated with abnormal amniotic volume could be considered as causal factors altering the production of fluid.

Birth Weight↗

Fetal vascular pressure responses to nonlabor uterine contractions: dependence on amniotic fluid volume in the ovine fetus.

OBJECTIVE: This investigation tested the hypothesis that elevations in fetal vascular pressures during nonlabor uterine contractions would be eliminated during polyhydramnios and augmented during oligohydramnios. STUDY DESIGN: Twenty-two chronically catheterized fetal sheep were divided into three groups: (1) control with normal amniotic fluid volume, (2) polyhydramnios produced by amnioinfusion, and (3) oligohydramnios produced by fluid drainage. Fetal arterial pressure, venous pressure, and amniotic fluid pressure were continuously recorded with on-line computer techniques. Amniotic fluid pressure was continuously subtracted from fetal arterial pressure and fetal venous pressure to correct to the fetal zero pressure reference. One minute averages were collected for 30 minutes centered around the nonlabor uterine contraction. RESULTS: Amniotic fluid pressure increased significantly during nonlabor uterine contractions in the control group. During polyhydramnios the amplitude and duration of the amniotic fluid pressure elevation were similar to those of controls (not significant). During oligohydramnios the duration of the uterine contractions was similar to that of both controls and polyhydramnios groups; however, the amplitude was less (p < 0.001). Fetal vascular pressures increased significantly during nonlabor uterine contractions in the control group (p < 0.001) but did not change significantly during nonlabor contractions in the polyhydramnios group. The contraction-induced elevations in fetal arterial pressure and fetal venous pressure in the oligohydramnios group were significantly greater than those in the control group (p < 0.001). CONCLUSION: This study shows that the fetal vascular pressure responses to nonlabor uterine contractions were modified significantly by increases or decreases in amniotic fluid volume. The fact that the fetal arterial pressure and fetal venous pressure responses were augmented during oligohydramnios and eliminated during polyhydramnios suggests that direct compression or a conformational change of the fetus may be the cause of the vascular pressure changes during nonlabor contractions.

Amniotic Fluid↗

Congenital adenomatoid malformation of the lung: when is active fetal therapy indicated?

OBJECTIVE: Although aggressive fetal therapies such as thoracoamniotic shunting can be applied to cystic adenomatoid malformations of the lung diagnosed in utero, there is no clear consensus regarding their indications. Our purpose was to evaluate a management policy in which aggressive fetal therapy was restricted to those cases complicated by major polyhydramnios or hydrops; all other cases were managed conservatively. STUDY DESIGN: A prospective cohort study of 33 cases with a prenatal diagnosis of cystic adenomatoid malformations of the lung was performed. Thoracoamniotic shunting was offered only in nine macrocystic cases with acute polyhydramnios or hydrops. RESULTS: Four cases were diagnosed postnatally as sequestrations. Of 12 cases complicated by acute polyhydramnios or hydrops, 5 survived (1 type III with spontaneous incomplete resolution in utero, 4 type I with substantial volume reduction after shunting). The 17 cases without acute polyhydramnios or hydrops were managed conservatively and survived. CONCLUSION: Conservative management is indicated in cases of cystic adenomatoid malformations of the lung without acute polyhydramnios or hydrops.

Cohort Studies↗

Alteration of the amniotic fluid and neonatal outcome.

Quantitative and qualitative alterations of the amniotic fluid complicate 7% of the pregnancies. Polyhydramnios complicates 1-3% while oligohydramnios involves 3-5% of the pregnancies. The most common causes of polyhydramnios are fetal abnormalities, maternal diabetes and twin pregnancies, but are idiopathic in the 60%. Perinatal mortality has been reported to range between 10-30% while the risk of preterm birth reaches up to 22% in pregnancies complicated by polyhydramnios. The neonatal outcome, in cases where polyhydramnios is due to fetal-neonatal abnormalities, depends on the underlying pathology. Polyhydramnios due to defects in intestinal canalisation in particular, has been correlated to good neonatal prognosis. In our experience no early postoperative deaths occurred in a group of 16 newborns consequtively admitted to our unit in the last two years, with abnormalities of the gastrointestinal tract with need of surgery within the second week of life. Most cases of oligohydramnios are due to premature rupture of membranes, other causes are fetal abnormalities, such as urinary tract malformations, or chromosomopaties and drugs e.g. NSAID's. Oligohydramnios of mild entities is often associated to preterm birth, fetal growth restriction. In some cases of oligohydramnios, neonatal survival is highly conditioned by pulmonary hypoplasia which develops with rates that range between 13 and 21%. Neonatal prognosis is often disastrous in cases with severe oligohydramnios, which however could be improved by amnioinfusion, which restores an amniotic fluid volume sufficient in reducing the adverse environmental effects and in prolonging, where possible, pregnancy. Beside the quantity also the quality of the amniotic fluid may be related to the neonatal outcome. Finding of some inflammatory factors (interleukines) in the amniotic fluid seems to be significantly correlated to periventricular leucomalacia (PVL), cerebral paralysis and long-term neurological abnormalities, both in the preterm and term neonate. Therefore, increase of the cytokines in the amniotic fluid could give information not only of the infection but also regarding the risk of developing neurological sequelae in neonatal period. Diagnosis and therapy for pathologies that alter the amniotic fluid have progressed, however efforts have still to be made in the identification and search for those quantitative-qualitative alterations of the amniotic fluid, for their potential implications on neonatal outcome.

Amniotic Fluid↗

Excess extrafetal fluid without demonstrable changes in placental concentration gradients after week-long infusions of angiotensin into fetal lambs.

It is known that a week-long infusion of angiotensin into fetal sheep produces polyhydramnios. The purpose of the present experiments was to determine whether an increased osmotic force across the placental barrier could account for the excess transfer of water. Six fetuses with indwelling catheters were infused with angiotensin-I and one with angiotensin-II; all, except one fetus in the first group, developed gross polyhydramnios. None of the transplacental concentration differences of the small plasma solutes Na+, Cl-, HCO3-, K+, urea, or glucose showed a demonstrable change and the same was true of the transplacental difference in freezing point osmolality and for the transplacental difference in plasma protein concentration. It is concluded that the infusion of angiotensin at a low dose rate is a reliable protocol for producing polyhydramnios. However, the present findings lend no support to the hypothesis that a primary change in transplacental osmotic force is the cause of the increased transplacental water transfer in this form of polyhydramnios. Alternative hypotheses are discussed in the light of recent discoveries.

Angiotensin I↗

Color Doppler imaging in the diagnosis and management of chorioangiomas.

Color Doppler imaging was used to investigate nine consecutive cases of placental tumors referred for evaluation to a tertiary referral unit. Gray-scale ultrasound findings were of a chorioangioma which was subsequently confirmed on pathologic examination. On color Doppler imaging (CDI), three tumors appeared avascular and the pregnancies were uncomplicated, two had only a few vessels in their periphery, and four contained numerous vessels. These vascular chorioangiomas were complicated by polyhydramnios and premature labor (n = 3), isolated polyhydramnios (n = 1), fetal growth restriction (n = 1) and generalized non-immune fetal hydrops (NIHF) (n = 1). The maximum diameter of the tumors ranged between 3 and 10 cm. There was no direct association between the size and location of the tumor and the development of complications. Successful amnioreduction was performed in the three cases of polyhydramnios and premature onset of labor. Vascular sclerosis was attempted unsuccessfully in the case complicated by severe NIHF. Our data indicate that the vascularization of the tumor is a pivotal determinant factor of pregnancy outcome. Where the tumor is avascular, no specific complications should be expected. Where the tumor is vascularized, and in particular if it contains numerous large vessels, serial ultrasound and Doppler examinations are warranted to detect polyhydramnios and early features of fetal congestive heart failure.

Diagnosis, Differential↗

[Placental specific findings in twin pregnancies with hydramnion].

Eleven of 157 twin pregnancies were complicated with polyhydramnios. Chorionicity, vascular anastomosis and insertion of the umbilical cord were analyzed with relation to polyhydramnios. 1. Polyhydramnios was found commonly in monochorionic placentation. 2. Vascular anastomosis was found in 40% of monochorionic polyhydramnotic placentas, which was lower than the anastomosis of monochorionic non-polyhydramniotic placentas. 3. The placentas with hydramnios had a higher incidence of abnormal umbilical insertion (marginal or velamentous insertion). These data indicate that incompetent vascular connections between the two fetoplacental circulations may be the pathogenesis of polyhydramnios.

Female↗

Factors associated with preterm delivery in mothers of children with Beckwith-Wiedemann syndrome: a case cohort study from the BWS registry.

Pregnancy that results in a child with Beckwith-Wiedemann syndrome (BWS) is associated with preterm delivery. Based on previous case series, we hypothesized that preterm delivery of a child with BWS was due to known risk factors for preterm delivery such as polyhydramnios and gestational hypertension. A case cohort study using the BWS Registry at Washington University School of Medicine was undertaken. Cases were pregnancies that resulted in the birth of a child with BWS, controls were pregnancies resulting in the birth of siblings without BWS. Univariate analyses of maternal complications and logistic regression to predict preterm delivery were used. Children with BWS (n = 304) were delivered preterm at a significantly higher rate than their siblings (n = 269) odds ratio 19.1 (95% CI 9.1-40.2). Polyhydramnios, gestational hypertension, and vaginal bleeding also occurred at high rates in the BWS group with odds ratios of 31.6 (95% CI 12.6-79.1), 2.4 (95% CI 1.4-4.1), and 3.9 (95% CI 2.3-6.4), respectively. In a multivariate logistic regression model within the BWS group, polyhydramnios, vaginal bleeding, and gestational hypertension were significant predictors of preterm delivery, odds ratios of 2.9 (95% CI 1.6-5.4), 2.6 (95% CI 1.3-5.0), and 5.3 (2.3-12.0), respectively. However, a significant proportion, 6.5% (18 of 277), of patients in the BWS group delivered preterm without known risk factors. Preterm delivery of a child with BWS is associated with an increased frequency of polyhydramnios, gestational hypertension, and vaginal bleeding in the mother. However, preterm delivery also occurs in the absence of these risk factors.

Adolescent↗

Intrauterine growth and ultrasound findings in fetuses with Beckwith-Wiedemann syndrome.

OBJECTIVE: To assess intrauterine growth in a series of nine fetuses diagnosed with Beckwith-Wiedemann syndrome. METHODS: Infants confirmed postnatally to have Beckwith-Wiedemann syndrome were identified from records maintained in the Division of Clinical Genetics. Antenatal ultrasound and birth records were evaluated. Head circumference (HC), abdominal circumference (AC), and estimated fetal weight (EFW) were assigned percentiles based on gestational age. Newborn HC and birth weight were also assigned percentiles. Polyhydramnios was diagnosed using either amniotic fluid index or documented subjective assessment. RESULTS: Nine infants with Beckwith-Wiedemann syndrome had antenatal ultrasound examinations. Seven of these had more than one examination. Two infants were suspected to have Beckwith-Wiedemann syndrome in utero. Important ultrasound findings included omphalocele (four), enlarged liver and kidneys (one), and enlarged liver (one). Fetal tongue protrusion on ultrasound was not identified in any fetus. Six of nine fetuses (66%) with ultrasound examinations after 25 weeks' gestation had polyhydramnios. Evaluation of the fetal HC, AC, and EFW percentiles demonstrated that fetuses with Beckwith-Wiedemann syndrome may exhibit accelerated growth as early as 25-30 weeks' gestation, but may exceed the 90th percentile only after 36 weeks' gestation. CONCLUSIONS: Fetuses with omphalocele, polyhydramnios, and an AC less than the 90th percentile may have Beckwith-Wiedemann syndrome. Polyhydramnios and accelerated growth beginning between 25 and 36 weeks' gestation, even without omphalocele, should alert the physician to the possibility of Beckwith-Wiedemann syndrome.

Beckwith-Wiedemann Syndrome↗

Tracheoesophageal fistula in utero. Twenty-two cases.

A retrospective review of 22 infants born with tracheoesophageal fistula (TEF) with in utero sonograms was performed and the literature was reviewed. TEF or esophageal atresia should be considered when there is polyhydramnios and an absent fluid-filled stomach; these findings were seen in 32% of our cases. Amniotic fluid flows freely through some TEFs, resulting in a normal amount of amniotic fluid and a fluid filled stomach (six of 22 patients), while in other cases, the fluid does not traverse the fistula easily and polyhydramnios results. Polyhydramnios was present in 62% of our cases and was the most common sonographic finding. The earliest age at which polyhydramnios was diagnosed was 24 weeks. Sonography will detect approximately one third of fetuses with TEF; an improved outcome is expected in these fetuses.

Female↗

The prenatal diagnosis of Pierre-Robin sequence.

The purpose of this study was to evaluate the spectrum of prenatal sonographic and chromosomal findings, associated anomalies and perinatal and neonatal outcomes in cases with Pierre-Robin sequence. All cases (20) with Pierre Robin sequence, who were born at China Medical College Hospital between 1990 and 1997, were included and analysed in this series. 12 pregnancies (60 per cent) were complicated by polyhydramnios and 9 (45 per cent) were combined with cleft palate. Four cases (20 per cent) with cardiac anomalies were also observed. Two fetuses (10 per cent) had abnormal karyotyping (one trisomy 21, one trisomy 18). All fetuses were delivered at or near term. Male deviation was observed in cases with isolated Pierre-Robin sequence or combined mild anomalies (male female ratio: 13:3). Two neonatal mortalities and three with mental retardation were observed. This investigation provides a basis for counselling patients with fetal micrognathia or neonatal Pierre-Robin sequence. The main prenatal sonographic findings of Pierre-Robin sequence are micrognathia, polyhydramnios and cleft palate. In cases of polyhydramnios, sonographic examination of the facial profile and palate are recommended. After the finding of polyhydramnios, micrognathia, and even cleft palate, clinicians should be aware of the possibility of neonatal Pierre-Robin sequence. Cardiac evaluation and karyotyping is also recommended.

Abnormalities, Multiple↗

Determination of bile acid concentration in human amniotic fluid for prenatal diagnosis of intestinal obstruction.

Bile acid concentration was measured in amniotic fluid obtained for standard indications from 11 healthy pregnant women without polyhydramnios (28 to 42 weeks of gestation) and from 9 patients with polyhydramnios (28 to 38 weeks of gestation). Two of the latter women delivered infants with intestinal obstruction distal to the papilla of Vater, a condition that causes regurgitation of bile into the amniotic fluid. In the women without polyhydramios, the total bile acid concentration ranged from 1.4 to 2.4 micronmol/liter. In the seven patients with polyhydramnios not associated with fetal intestinal obstruction, the bile acid concentration in amniotic fluid was not significantly different (0.9 to 1.9 micronmol/liter). By contrast, the bile acid concentration in amniotic fluid specimens from the two patients with polyhydramnios who gave birth to children with intestinal obstruction was considerably elevated (30.3 to 83.1 micronmol/liter). These findings suggest that determination of bile acid concentration in amniotic fluid permits prenatal diagnosis of intestinal obstruction distal to the papilla of Vater.

Amniotic Fluid↗

The Perlman familial nephroblastomatosis syndrome.

In 1973, Perlman et al described a familial syndrome of bilateral renal hamartomas with or without nephroblastomatosis, macrosomia, islet cell hypertrophy, unusual facies, and early lethality. Two additional sibs were recently reported by Neri et al [1984]. We report on two sibs with polyhydramnios, fetal ascites, and abdominal muscular hypoplasia, visceromegaly, and subsequent development of Wilms tumor in one of them. Delineated features of this syndrome include visceromegaly, macrosomia, renal hamartomas, nephroblastomatosis, cryptorchidism in males, unusual facial appearance, polyhydramnios, fetal ascites, and Wilms tumor but do not include hemihypertrophy, omphalocele or umbilical abnormalities, aniridia, or other conditions known to be associated with Wilms tumor. This condition should be considered primarily in the differential diagnosis of fetal ascites without hydrops and possibly in the differential diagnosis of familial Wilms tumor, polyhydramnios, congenital hepatomegaly, or nephromegaly.

Adult↗

Prenatal diagnosis of inherited epidermolysis bullosa in a patient with no family history: a case report and literature review.

OBJECTIVE: The junctional form of epidermolysis bullosa (EB) is a recessively inherited mechanobullous disease in which minimal trauma results in blister formation at the dermal-epidermal junction. A rare form associated with pyloric atresia (JEB-PA) is a severe clinical subtype leading to rapid demise after birth, thus justifying prenatal diagnosis. The case characterized by abnormal ultrasound findings at 35 weeks of gestation (gastric dilatation associated with polyhydramnios) of a patient with no family history is reported. METHOD: Postabortion skin biopsies were analyzed by immunofluorescence that revealed marked reduction of integrin alpha6beta4 in accordance with the diagnosis of JEB-PA. RESULTS: Amniotic fluid contained excess total protein (4 MoM), abnormally high AFP (20.4 MoM) related to skin lesions and abnormally elevated digestive enzyme suggestive of fetal vomiting of bile. The electrophoretic pattern of cholinesterases was unusual (additional slow band). Maternal serum AFP was 3.14 MoM and free beta-hCG 13.1 MoM. Because of these concomitant findings, JEB-PA was suspected. CONCLUSION: The case under study was atypical because of late clinical manifestations of the disease: polyhydramnios, gastric enlargement. As maternal serum AFP at 15 weeks may be normal, it was suggested that discovery of polyhydramnios during the second or the third trimester should prompt biochemical analysis of amniotic fluid, such as AFP and GGTP assay in all cases.

Adult↗

Prenatal counselling of small bowel atresia: watch the fluid!

OBJECTIVE: To evaluate polyhydramnios as a sign of extreme disproportion of atretic segments in small bowel atresia (SBA). METHODS: Twenty-eight patients with a prenatal diagnosis (PD) of SBA undergoing neonatal surgical treatment were reviewed retrospectively. Parameters recorded were gestational age, birth weight, surgical procedure, rate of complications, parenteral nutrition (PN) days and length of stay (LOS). Patients were divided into two groups: Group A with delayed anastomosis and Group B with direct anastomosis. RESULTS: Seventeen subjects were in Group A while 11 were in Group B. The two groups did not differ with regard to gestational age at diagnosis, birth weight and obstetrical management. Polyhydramnios was present in both Group A (64.7%) and Group B (9%) (p < 0.05). Patients in Group A needed a longer period on PN, had a longer LOS and exhibited significantly higher rates of complication. CONCLUSION: In the absence of other malformations, association of dilated bowel loops and polyhydramnios is highly predictive of severe SBA, which can in no instance be amenable to one-stage reconstruction. Because of its consequences on postnatal treatment, such information should be conveyed to the prospective parents at the time of counselling.

Counseling↗

Prenatal diagnosis of congenital cystic adenomatoid lung malformation: a report of seven cases.

Six cases of macrocystic and one case of microcystic congenital adenomatoid lung malformation were diagnosed by ultrasound between 20 and 31 weeks of gestation. Combined polyhydramnios and fetal hydrops was present in three cases, polyhydramnios alone in one case, and isolated fetal hydrops also in one case. In the remaining two cases, both polyhydramnios and fetal hydrops were absent. Fetal outcome was poor, i.e., two terminations of pregnancy, three early neonatal deaths, and two survivors.

Adult↗

Congenital malformation syndromes and elevation of amniotic fluid alpha-fetoprotein.

Fetal malformations may introduce complications of maternal pregnancy. A polyhydramnios represents one such complication during pregnancy. We want to report five abnormal pregnancies which were marked by acute polyhydramnios and/or premature labor due to an amniotic band syndrome associated with cerebral herniation in two cases, malignant oral teratoma in one case, bilateral cystic hygromas associated with generalized fetal hydrops in one case, and multiple internal malformations in one case alpha-fetoprotein (AFP) values between the 25th and 34th week of gestation were elevated 3.5 to 44 times the normal median value. Since all fetuses showed severe malformations incompatible with life our observations indicate the necessity to determine AFP in cases of acute polyhydramnios independent of the week of gestation. Conversely, elevated AFP levels in amniotic fluid obtained during prenatal diagnosis in the 16th week of gestation may also suggest rare fetal malformations outlined above.

Amniotic Fluid↗

The relationship of increased amniotic fluid volume to perinatal outcome.

We studied a group of 135 patients with polyhydramnios diagnosed on the basis of ultrasonic findings (greatest vertical diameter of the liquor column greater than or equal to 8.0 cm) between 32 and 36 weeks gestation (study group). We compared the obstetric characteristics and perinatal outcome of the study group with a similar number who constituted our control group. The incidence of women aged 20 years or less was higher in the study group (8.9%) compared with 4.5% in the control group. Of the 135 patients who were diagnosed to have polyhydramnios ultrasonically, the clinical diagnoses prior to referral for ultrasonic scanning, were, suspected large for date fetuses in 34 patients (25.2%), clinically suspected polyhydramnios in 28 (20.7%), gestational diabetes in 21 (15.6%) and insulin dependent diabetes in 6 (4.4%) compared with 13.3%, 5.2%, 3.0% and 0.7%, respectively in the control group (P less than 0.05). Preterm delivery occurred in 11.1% in the study group compared with the incidence of 6.7% in the control group. The incidence of fetal distress, low Apgar Score, macrosomic infants, major fetal anomalies, gross and corrected perinatal mortality rate and admission to special/intensive care nursery was significantly higher in the study group compared with that of the control (P less than 0.01). We found ultrasonic examination is a reliable technique to assess the amount of amniotic fluid volume and it alerts the clinician to possible future problems in pregnancy, labor and neonatal period.

Adult↗