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Bladder visualisation as a prognostic sign in oligohydramnios-polyhydramnios sequence in twin pregnancies treated using therapeutic amniocentesis.

Oligohydramnios-polyhydramnios sequence in twin pregnancies may be managed by aggressive amniocentesis and is described in nine consecutive cases. In four of the nine pregnancies both twins survived, one pair died in the neonatal period, and the other four pairs all suffered intrauterine death. The median number of amnioreductions performed was five (range 2-7). In this series the reaccumulation of urine in the bladder of the 'stuck twin' was a predictive prognostic marker of survival in both twins, with a sensitivity and specificity of 100%.

Adult↗

The significance of polyhydramnios diagnosed during labour or at caesarean section.

In the 38 cases of polyhydramnios reported here the diagnosis was made only at the time of artificial rupture of the membranes to induce or augment labour, or at Caesarean section. Usually this was a benign finding often associated with overweight in the mother (60%) or baby (39.4%). However there was an increased incidence of major congenital abnormality (13.1%) resulting in a high perinatal mortality rate (10.5%); the paediatrician should therefore be alerted prior to delivery of these patients.

Abnormalities, Multiple↗

Successful outcome with serial amniocenteses for polyhydramnios complicating cystic adenomatoid malformation of the lung.

Hydrops fetalis secondary to congenital cystic adenomatoid malformation of the fetal lung is almost invariably a lethal condition. This poor prognosis may be attributed to the combined effects of tissue compression from the thoracic space occupying lesion and premature delivery of a hydropic baby following rupture of the membranes in the presence of polyhydramnios. We describe a successful outcome with serial amniocenteses. Partial in utero resolution of the lung lesion with complete return to normal of the aberrant liquor volume was documented, and a live infant was delivered at term.

Adult↗

Amniotic septostomy for the treatment of twin oligohydramnios-polyhydramnios sequence.

OBJECTIVE: To report our experience with intentional puncture of the intervening membrane ('septostomy') for the treatment of the twin oligohydramnios-polyhydramnios sequence (TOPS). METHODS: 12 patients were diagnosed with TOPS based on ultrasonographic findings. A 20- to 22-gauge spinal needle was used to puncture the membrane between the twins without any attempt at amnioreduction in 9 patients, while the procedure was combined with amnioreductions in 3 patients. RESULTS: Gestational age was 23.1 +/- 3.3 weeks at the time of septostomy and 31.1 +/- 4.4 weeks at delivery. Rapid accumulation of fluid around the 'stuck' fetus occurred in all cases following a single procedure. Three of the 24 fetuses died in utero and 1 died on the fifth day of life, for a combined survival of 83.3%. In the survivors, the septostomy to delivery interval ranged between 0.6 and 13 weeks (mean +/- SD 8.3 +/- 4.8). CONCLUSION: Amniotic septostomy is a promising new method for the management of TOPS and is associated with survival rates that are better than, or comparable to, more invasive modalities. A multicenter trial comparing septostomy to other modalities is warranted.

Amnion↗

Equalization of amniotic fluid volumes after decompression amniocentesis for treatment of the twin oligohydramnios-polyhydramnios sequence.

OBJECTIVE: To measure acute and chronic changes in the placenta and amniotic fluid associated with performance of decompression amniocentesis in pregnancies with the twin oligohydramnios-polyhydramnios sequence (TOPS). METHODS: Amniotic fluid pressures, placental thickness, placental perfusion, and amniotic fluid volumes were measured in each sac of a monochorionic diamniotic twin gestation before and after decompression amniocentesis. Indigo carmine was injected into the polyhydramnic sac after decompression, and fluid from the oligohydramnic sac was sampled after equilibration. Spectrophotometric analysis of amniotic fluid specimens was performed for dye detection. Amniotic fluid volume and placental perfusion studies were repeated 1 week later. RESULTS: Three patients with TOPS were enrolled, and decompression amniocentesis was performed in the midtrimester. After decompression, amniotic fluid volume decreased in the polyhydramnic sac, amniotic fluid pressures decreased in both sacs, placental thickness increased, and umbilical artery Doppler velocimetry was unaffected. The amniotic fluid volume increased acutely in only one oligohydramnic sac after decompression, and ultrasonographic examination, amniotic fluid spectrophotometric analysis, and placental pathologic examination all identified interfetal membrane disruption as the etiology. CONCLUSIONS: Decompression amniocentesis as a treatment for TOPS does not result in acute or chronic changes in the amniotic fluid volume of the oligohydramnic sac in the absence of interfetal membrane disruption.

Adult↗

Fetal and neonatal outcomes in twin oligohydramnios-polyhydramnios sequence including cerebral palsy.

OBJECTIVE: The purpose of this study was to review consecutive pregnancies complicated by twin oligohydramnios-polyhydramnios sequence (TOPS) and to clarify the probable factors influencing the perinatal outcomes. METHODS: Twenty-five pregnancies with a diagnosis of TOPS were treated with and without amniocentesis. A poor perinatal outcome was defined when either perinatal mortality or cerebral palsy was observed. RESULTS: The mean gestational age at the time of diagnosis and of delivery was 27.8 (range 21-34) and 30.7 (range 23-35) weeks, respectively. Tocolytic therapy and therapeutic amniocentesis were done in 16 (64%) and 13 (52%) cases. Hydrops fetalis was present in 10 cases. The survival rate was 72%, and cerebral palsy was diagnosed in 8 of 36 infants (22%). The poor prognostic factors were gestational age at delivery (<30 weeks) and presence of symptomatic preterm labor and hydrops fetalis. Therapeutic amniocentesis did not influence the perinatal outcome. CONCLUSIONS: In this observational study, the long-term as well as the short-term perinatal outcome of pregnancy with TOPS seems to be poor. In addition, therapeutic amniocentesis in TOPS does not appear to affect fetal and neonatal outcomes.

Amniocentesis↗

Fetal urine production in normal twins and in twins with acute polyhydramnios.

In twelve twin pregnancies with normal amniotic fluid volume, the urine output of each twin was lower than in fetuses from singleton pregnancies, and the combined urine output of both twins was between the 50th and 95th centile for singletons. In three twin pregnancies at 21-24 weeks of gestation with acute polyhydramnios, presumed to be due to the twin-twin transfusion syndrome, the urine output of the smaller fetus was zero and that of the larger was above the 95th centile for normal singleton pregnancies. These three pregnancies were managed by repeated amniocenteses and rapid drainage of large volumes of amniotic fluid. With advancing gestation, there was a tendency for normalization of urine output in the twins.

Amniocentesis↗

Congenital difficulties with swallowing and breathing associated with maternal polyhydramnios: neurocristopathy or medullary infarction?

Two babies with congenital difficulties in swallowing and breathing are presented and contrasted. Both were associated with maternal polyhydramnios, but one was due to medullary infarction and the other to a malformation ("neurocristopathy"). In the former case, isolated tenth and twelfth cranial nerve palsies provided the clue as to the correct etiology, whereas the latter baby had dyscoordination of swallowing and breathing with sleep apnea ("Ondine's curse"). It is somewhat ironic that the case without a difficult delivery had the infarct, probably prenatal in onset, whereas the one with a difficult delivery had the congenital malformation. Neural crest cells are known to migrate widely and to develop greatly different functions, but the fact that their central associations of neural tube origin may also be affected has not previously been emphasized in the etiopathogenesis of the congenital malformation. A variety of syndromes with combinations of many defects may be seen in which too many or too few cells are formed, with more or less serious consequences especially for the respiratory and gastrointestinal systems.

Breech Presentation↗

Acute polyhydramnios in twin pregnancy. A retrospective study with special reference to therapeutic amniocentesis.

Acute polyhydramnios in monozygotic twin pregnancy causes severe maternal discomfort and carries a high risk of premature labor. During the years 1980 to 1987, 36 patients with this complication were delivered in Sweden, giving an incidence of 1/20,000 births, or 1/200 twin births. In 18 patients (group A) who were treated with one or more amniotic taps, the delivery was postponed by 2 weeks, as compared with one week in 18 conservatively treated patients (group B). The peri- and neonatal death rate was 47% in group A and 58% in group B. Our own experience is that amniotic taps are safe if the amniotic fluid is removed slowly under prophylactic tocolysis. It gives symptomatic relief to the mother and may postpone labor until the gestational age of the twins is more compatible with survival.

Acute Disease↗

Isolated craniosynostosis: prenatal ultrasound of scaphocephaly with polyhydramnios.

Craniosynostosis is an abnormal shape or dimension of the skull caused by premature closure of one or more skull sutures. It includes scaphocephaly, brachycephaly, oxycephaly, plagiocephaly, trigonocephaly, turricephaly, and a cloverleaf-shaped head. The only cases of craniosynostosis that have been reported concern fetuses with complex and marked craniosynostosis syndromes such as acrocraniofacial dysostosis, Apert's syndrome, Beare-Stevenson cutis gyratum syndrome, Calabro's syndrome, etc. Isolated or simple craniosynostosis, however, is a developmental anomaly rarely found on routine ultrasound examination. We present a case that was diagnosed as scaphocephaly with moderate polyhydramnios by prenatal ultrasound. No other structural anomaly was detected, and the karyotyping was normal. So isolated sagittal craniosynostosis was diagnosed prenatally and was confirmed by postnatal skull radiography as well as 3-dimensional computed tomography. The infant underwent bilateral parietal craniectomy at the age of 3 months. He obtained a good result with no mental problems 4 years after the operation. To our knowledge, this is the second report on the prenatal detection of an isolated form of craniosynostosis.

Adult↗

Fumarase deficiency: two siblings with enlarged cerebral ventricles and polyhydramnios in utero.

A family having two boys with progressive encephalomyopathy and fumaric aciduria due to fumarase deficiency is described. Both patients initially presented with polyhydramnios and enlarged cerebral ventricles in utero, with subsequent cerebral atrophy, severe developmental delay, infantile spasms, and hypsarythmia on electroencephalogram. Fumarase activity in blood mononuclear cells and in the mitochondrial and cytosolic fractions of cultured skin fibroblasts was less than 0.5% of the control mean or undetectable. The older boy died at the age of 5 years and 4 months and the younger one is now 2 years and 10 months. The unrelated parents are symptomless and the other three children in the family are clinically healthy. Fumarase activities in the blood mononuclear cells of the father, mother, sister, and two brothers were 59%, 52%, 52%, 120%, and 44% of the control mean, respectively. The results strongly support autosomal recessive inheritance of fumarase deficiency and suggest its consideration in children with congenital hydrocephalus, progressive brain atrophy, and infantile spasms.

Cells, Cultured↗

[Bartter syndrome--a rare cause of severe polyhydramnios].

We report a case of a 25-year-old woman, gravida II, Para I, in pregnancy week 26 + 4 with severe polyhydramnios. The amniotic fluid index was about 39. Type 2 scanning showed a large urinary bladder only. Treated in pregnancy with repeated aminodrinage, total 21 litres. In week 33 + 5, a Caesarean section was performed. A girl with Apgar score 10/1, 0/10 was born, weight 1,804 grammes. Immediately after birth the child developed polyuria with diuresis, about 8-10 ml/kg/hour, electrolyte disturbance, and vomiting, treated with Losec, Gaviscon, indometacin, and Vioxx. The diagnosis was Bartter syndrome.

Adult↗

Polyhydramnios: fetal and neonatal implications.

Polyhydramnios is a common obstetric condition with a high incidence of neonatal morbidity and mortality. Early prenatal diagnosis is essential to provide proper perinatal care. Understanding the physiology of these defects can assist the entire perinatal team in optimizing care for the mother, fetus, and infant.

Amniocentesis↗

Antenatal evaluation of upper gastrointestinal dilatation complicated by non-immune hydrops fetalis and polyhydramnios.

A case of upper gastrointestinal dilatation associated with fluid collection in the peritoneal cavity is reported where the prenatal diagnosis was made sonographically in a non-immunologic hydrops fetalis. Neither generalized edema nor pleural effusion were detected. Polyhydramnios was complicated. Almost complete drainage of the abdominal fluid was accomplished with rapid re-accumulation of fetal ascites and no resolution of intestinal dilatation. After delivery by Cesarean section, the male infant died the next day due to respiratory distress syndrome. Postmortem histologic examination failed to confirm the ultrasonographic findings including ileal obstruction and subsequent hypoplasia in the colon. Despite the discrepancy between clinical and pathologic findings, the findings of this case would improve the etiology of hydrops where no possible cause is found.

Adult↗