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Placental pathophysiology of the twin oligohydramnios-polyhydramnios sequence and the twin-twin transfusion syndrome.

Currently accepted sonographic criteria for antenatal diagnosis of twin-twin transfusion (TTT) syndrome include a monochorionic placenta with same-sex twins, marked growth discordance, and oligohydramnios of the growth-retarded twin with coexistent polyhydramnios of the larger twin. Our previous report of nine women fulfilling these criteria, examined using sequential funipuncture of both fetuses, demonstrated inter-twin blood transfusion in only four cases (44 per cent). It was proposed that traditional sonographic criteria actually describe a heterogeneous group of disorders more appropriately described as the twin oligohydramnios-polydramnios sequence (TOPS). True TTT is a subset of this population, the antenatal diagnosis of which requires specific demonstration of transfusion from one fetus (donor) to the other (recipient). In this report, antenatal placental evaluation has been correlated using duplex pulsed-wave Doppler analysis of arterial blood flow velocity with postpartum gross and histopathologic evaluation of the placenta, with special attention to microvasculature. There was a higher incidence of resistance to blood flow, abnormal umbilical cord insertion, and diminished placental microvasculature associated with oligohydramnic growth-retarded (donor) twins when compared with polyhydramnic (recipient) twins. Based on these observations, it is proposed that TTT and TOPS represent asymmetric placental insufficiency resulting from aberrant placentation.

Blood Flow Velocity↗

Management of a triplet pregnancy with two anencephalic fetuses and polyhydramnios.

The occurrence of a triplet pregnancy discordant for anencephaly is rare and its management presents a clinical dilemma. We report what appears to be the first case of a triplet pregnancy with two anencephalic fetuses complicated by premature contractions and severe polyhydramnios. Its management, which results a healthy newborn weighing 1385 g is discussed.

Adult↗

Benign multiple diffuse neonatal hemangiomatosis after a pregnancy complicated by polyhydramnios and a placental chorioangioma.

A male newborn with multiple cutaneous hemangiomatosis is described. Pregnancy was complicated by polyhydramnios and a large placental chorioangioma. After an initial outburst of the hemangiomas in the first two weeks of life, spontaneous and almost complete regression occurred before the age of 3 months. The relationship between hemangiomas and placental chorioangioma is briefly discussed.

Female↗

Influence of vasopressin in the pathogenesis of oligohydramnios-polyhydramnios in monochorionic twins.

OBJECTIVES: The pathophysiology of oligohydramnios-polyhydramnios in monochorionic (MC) twins complicated by chronic twin-twin transfusion syndrome (TTTS) is poorly understood. We hypothesise that oliguria and oligohydramnios in the donor twin of chronic TTTS, occurs due to antidiuretic and vasoconstrictive activity of vasopressin (AVP). METHODS: We measured AVP levels in maternal, fetal and amniotic fluid samples obtained in utero and at birth from 44 MC twins with (n=27) or without chronic TTTS (n=17). Concentrations of AVP in pg/ml were determined by immuno-radiometry assay. RESULTS: In donor fetuses, plasma and amniotic fluid AVP levels were higher than those of the recipient twins in utero (P<0.001) and at birth (P<0.001). No such differences were found between the non-TTTS twins. The plasma AVP concentrations were higher in the recipient fetuses with severe hydrops than those without hydrops (2.8+/-0.7 pg/ml versus 0.3+/-0.3 pg/ml; P<0.05). Maternal AVP levels were comparable between the TTTS and non-TTTS groups. In the non-TTTS twins, both plasma and amniotic fluid AVP levels were higher than those of the recipient twins (P<0.001) but lower than those of the donor twins (P<0.001). There was a significant association between amniotic fluid and plasma AVP levels both in the TTTS (r=0.78; P<0.001) and non-TTTS (r=0.70; P<0.01) infants. CONCLUSIONS: Vasopressin concentrations in the donor twins were three times higher than their co-twins which suggests that oligohydramnios may occur as a consequence of AVP mediated reduction in fetal urine output.

Adult↗

Serial amniocenteses in the treatment of twin to twin transfusion complicated with acute polyhydramnios.

Twin to twin transfusion, complicated by acute polyhydramnios in a monozygous twin pregnancy, is a difficult clinical problem. A precipitous course usually results in termination of the pregnancy within a few days and often is associated with a high perinatal mortality rate. Two cases are presented that were treated with repeated amniocenteses for the relief of extreme abdominal discomfort and to prevent imminent premature labor. The amount of amniotic fluid removed each time varied from 300 cc to 1200 cc, which was enough to relieve symptoms but not enough to induce uncontrolled uterine activity. A total of 3500 cc and 4750 cc of amniotic fluid were removed from the first and the second patient, respectively. The procedure was found to be safe and resulted in prolonging the pregnancies by 14 and 11 days, respectively. This management, with the addition of tocolysis and close fetal surveillance can offer some hope in an otherwise hopeless situation.

Adult↗

Newborn with transverse facial cleft associated with polyhydramnios.

We describe here the case of a female newborn baby with a bilateral complete transverse facial cleft. Obstetrical ultrasound had revealed an increased amount of amniotic fluid from 28 weeks' gestation without fetal hydrops or congenital anomalies. A 1900-g baby girl born at 36 weeks' gestation presented with bilateral wide facial clefts with macrostomia, microphthalmia, nose, and auricular deformities. Her breathing was dependent on life support, which was discontinued 2 hours after birth. An autopsy revealed no congenital malformations in vital organs but the absence of the olfactory nerves. Polyhydramnios and respiratory arrest after birth were presumed to be due to central disintegration of swallowing and breathing, in this case with brain anomaly.

Adult↗

Atrial natriuretic peptide mediated polyuria: pathogenesis of polyhydramnios in the recipient twin of twin-twin transfusion syndrome.

We studied the role of atrial natriuretic peptide (ANP) in the pathophysiology of polyhydramnios in monochorionic (MC) twins with and without twin-twin transfusion syndrome (TTTS). Matched maternal, fetal blood samples and amniotic fluids (AF) were obtained in utero (n=12) and at birth (n=20) from MC twins with TTTS. Blood and amniotic fluid samples were also collected from non-TTTS MC twin pairs in utero (n=6) and at birth (n=20). In both groups cellular localization of ANP in the fetal kidney and heart was performed using anti ANP rabbit polyclonal antibody. Concentrations of ANP in pg/ml were determined by radioimmunoassay.In recipient fetuses, ANP levels were higher than the donors both in utero (P< 0.001) and at birth (P< 0.001). No such differences were found between the non-TTTS twins. In the TTTS group maternal ANP levels were lower than the non-TTTS group (P< 0.05). A linear relationship was found between fetal ANP levels and the AF volumes removed at fetal blood sampling (r(2)=0.68;P< 0.01, n=12). ANP was localized predominantly to the cytoplasm of the distal convoluted tubules of the fetal kidney and heart, and the intensity of immunostaining for ANP in kidney and heart were markedly greater in the recipient than the donor twin. No such differences were found between the twin pairs. These data suggest that polyhdramnios in the recipient twin occurs as a consequence of ANP mediated increase in fetal urine output and raises the possibility of direct fetal therapy with ANP blocking agents.

Amniotic Fluid↗

Reduction in the middle cerebral artery pulsatility index after decompression of polyhydramnios in twin gestation.

Amniotic fluid decompression was performed for symptomatic polyhydramnios complicating four sets of discordant twin gestations. Doppler ultrasound waveforms of the middle cerebral artery were determined before and after the procedure in seven of the eight fetuses. After amniocentesis, the pulsatility index of the middle cerebral artery (PIMCA) was reduced in all fetuses (p < 0.01). When one considers only the larger twin in each set, the magnitude of the change in PIMCA was relatively consistent among the different sets (-0.60 +/- 0.14; p < 0.01; range, -0.45 to -0.79). The smaller twins showed a much more variable response (-0.82 +/- 0.70; range, -0.05 to -1.75). The pulsatility index of the umbilical artery (PIUA) showed no consistent trend in the five subjects in which it was determined. In principle, the cerebrovascular dilation indicated by the PIMCA measurements may in part be due to acute increases in maternal and fetal carbon dioxide tensions following relief of maternal restrictive lung dysfunction. More importantly, the acute fall in amniotic fluid pressure--the external pressure to which the fetoplacental unit is exposed--leads to pooling of blood in fetal and placental veins, and thereby reduces the effective blood volume of the fetoplacental unit. This effective hypovolemia, functionally analogous to that produced by fetal hemorrhage, elicits changes in regional vascular resistances that favor cerebrovascular perfusion. The impact of acute amniotic fluid decompression on the fetal circulation reflected in the marked changes in PIMCA suggests a role for monitoring to avoid large acute changes in pressure during therapeutic amniocentesis.

Amniocentesis↗

Resolving polyhydramnios. A sign of improved fetal status.

Four cases of transient polyhydramnios are reviewed. It is proposed that by taking note of changes in amniotic fluid volume, one can more accurately assess a fetal-maternal unit that is affected by a number of specific disease processes.

Adult↗

Congenital hypertrophic pyloric stenosis with associated polyhydramnios in a premature infant.

Congenital pyloric stenosis is rare in premature infants. This is a report of pyloric stenosis in a premature infant (35 weeks gestation) discovered on the 2nd day of life. There appears to be an association with polyhydramnios. Congenital hypertrophic pyloric stenosis should be considered in the differential diagnosis of premature infants with upper gastrointestinal symptoms. A review of the literature is included.

Cesarean Section↗

Brain natriuretic peptide and endothelin-1 in the pathogenesis of polyhydramnios-oligohydramnios in monochorionic twins.

OBJECTIVE: We investigated the association between amniotic fluid levels of human brain natriuretic peptide, endothelin-1, and abnormal amniotic fluid volume in monochorionic twins with and without chronic twin-twin transfusion syndrome. STUDY DESIGN: Amniotic fluid and fetal blood samples were obtained in utero or at cesarean delivery from monochorionic twins with (n = 20) or without chronic twin-twin transfusion syndrome (n = 10). Concentrations of atrial natriuretic peptide, human brain natriuretic peptide, and endothelin-1 (in picograms per milliliters) were determined by radioimmunoassay. RESULTS: The amniotic fluid concentrations of human brain natriuretic peptide (P <.001) and endothelin-1 (P <.001) in the recipient fetuses were higher than the donor twins but were similar in the twins with no twin-twin transfusion syndrome. In the donor twins, amniotic fluid concentrations of human brain natriuretic peptide (P <.001) and endothelin-1 (P <.001) were lower than the twin pairs with no twin-twin transfusion syndrome. In both chronic twin-twin transfusion syndrome fetuses (P <.01) and fetuses with no twin-twin transfusion syndrome (P <.001), the amniotic fluid concentrations of human brain natriuretic peptide were high, although the concentrations of the endothelin-1 were lower than the fetal plasma concentrations. A positive association was present between amniotic fluid levels of human brain natriuretic peptide and endothelin-1 (R (2) = 0.51, P <.001, n = 60). Amniotic fluid human brain natriuretic peptide (r = 0.67, P <.001) and endothelin-1 (r = 0.57, P <.01) levels of the recipient twins correlated with the amniotic fluid index. CONCLUSION: These data suggest that amniotic fluid concentrations of human brain natriuretic peptide and endothelin-1 were highest in the twins with polyhydramnios and lowest in the twins with oligohydramnios, which suggests the importance of these hormones in the regulation of amniotic fluid volume.

Amniotic Fluid↗

D-bifunctional protein deficiency with fetal ascites, polyhydramnios, and contractures of hands and toes.

Fetal abnormalities including chylous ascites, polyhydramnios, claw hands, and hammer toes were identified in an infant who had a missense mutation R106P and a 52bp deletion in the gene for a peroxisomal beta-oxidation enzyme, D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase, D-bifunctional protein. The patient had psychomotor retardation and craniofacial dysmorphism and died at 7 months of age. The patient had atypical fetal manifestations of this enzyme deficiency.

17-Hydroxysteroid Dehydrogenases↗

Umbilical cord hemangioma associated with polyhydramnios, congenital abnormalities and perinatal death in a twin pregnancy.

A twin pregnancy is described in which an umbilical cord hemangioma, polyhydramnios, developmental abnormalities and perinatal death were restricted to one twin, while the other twin was unaffected. Cord hemangiomas are rare and their association with fetal abnormalities is controversial. This case study supports a direct association between the cord hemangioma and the adverse pregnancy outcome, since congenital abnormalities and a cord hemangioma were present in only one of the twins.

Abnormalities, Multiple↗

Indomethacin therapy in the treatment of symptomatic polyhydramnios.

It appears that maternal indomethacin therapy may be a useful adjunct in selected cases of polyhydramnios. Initial evaluation should include glucose tolerance testing and a thorough search for fetal abnormalities by ultrasonography. In the patient with symptoms such as premature labor or respiratory compromise, an initial amniocentesis should be considered for decompression and fetal karotype. Oral indomethacin therapy can then be started. Although the optimal dose is unknown, a 25-mg oral dose every 6 hours appears adequate. Ultrasound assessment of amniotic fluid volume should be done once or twice weekly. If oligohydramnios develops, the indomethacin should be discontinued, and the amniotic fluid volume serially monitored. Fetal echocardiography should be considered in the first 24 hours after therapy has been initiated and weekly thereafter. Evidence of severe constriction of the ductus arteriosus or tricuspid regurgitation warrants discontinuation of the indomethacin; lesser degrees of ductal constriction can be treated by decreasing the dose of the medication.

Female↗

Polyhydramnios: a review.

Polyhydramnios is a relatively common obstetrical complication. Major causes include maternal diabetes, chromosomal disorders, isoimmunologic disease, congenital abnormalities, multiple gestations, and idiopathic reasons. Perinatal morbidity and mortality is high and maternal complications are frequent. Successful management depends upon appropriate diagnostic evaluation. Prolongation of the pregnancy for further fetal maturation may be achieved with timely therapeutic amniocenteses.

Female↗

Indomethacin therapy in the treatment of polyhydramnios due to placental chorioangioma.

A 26-year-old primigravida presented with acute polyhydramnios at 30 weeks gestation. Ultrasonography revealed a large placental chorioangioma with severe hydramnios. No anomalies were detected in the fetus. Preterm labor started with respiratory distress and indomethacin, 25 mg was given every 6 hours. The patient showed a good response with improvement of the hydramnios and respiratory symptoms. A normal infant with no neonatal complications was delivered 3 weeks later.

Adult↗