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Mortality among infants with high-risk congenital diaphragmatic hernia in Singapore.

Several factors suggested to predict mortality in congenital diaphragmatic hernia (CDH) have not always been applicable in different centers. A retrospective review was conducted of 19 consecutive neonates in Singapore in whom CDH was diagnosed within 12 hours of birth to identify factors associated with mortality. Of the 19 cases, 15 (79%) were diagnosed using antenatal ultrasonography. Eight (42%) underwent primary repair at a median age of 23 hours (range, 12 to 50 hours). Of the 19 infants, 15 died (mortality rate, 79%). Survivors until hospital discharge were compared with nonsurvivors. Antenatal diagnosis and stomach position in left-sided defects had no effect on outcome, although polyhydramnios tended to be associated with nonsurvival. Significant postnatal factors associated with mortality included a low arterial pH level, low initial arterial-alveolar oxygen ratio, high initial alveolar-arterial oxygen gradient, as well as high oxygenation and ventilation indices. These results reflect difficulty in oxygenation because of pulmonary hypoplasia despite evidence of adequate ventilation. There was no difference between survivors and nonsurvivors in either their initial or best postductal blood gases. The "Bohn quadrants" did not aid in predicting survival of infants who underwent repair because all eight such infants had best postductal carbon dioxide values of less than 40 mm Hg and ventilation indices of less than 1,000. Yet only four (50%) survived until hospital discharge. Large-scale evaluation of these factors may be required in the future to demonstrate their validity and reliability because of changing management strategies for CDH.

Carbon Dioxide↗

Successful outcome after serial amnioreductions in triplet fetofetal transfusion syndrome.

BACKGROUND: Triplet fetofetal transfusion is an extremely rare complication with high perinatal mortality. Its rarity does not allow any prospective randomized study on various interventional methods to be conducted. CASE: We report one case of triplet fetofetal transfusion syndrome with survival of all three fetuses. Two were donor fetuses, and one was the recipient fetus. Serial amnioreductions were performed at 22, 24, and 26 weeks' gestation to relieve symptomatic polyhydramnios. Premature rupture of membranes occurred at 27 weeks and cesarean delivery was performed. All three babies were discharged home by 4 months of age, and all had normal neurological development when assessed at 6 months of age. CONCLUSION: The option of serial amnioreduction, with the anticipation and preparation for delivery at around 28 weeks, should be seriously considered when triplet fetofetal transfusion syndrome is encountered.

Adult↗

Relationship between change in amniotic fluid index and volume of fluid removed at amnioreduction.

OBJECTIVE: To determine the relationship between the change in amniotic fluid index (AFI) and the volume of fluid removed at amnioreduction. METHODS: In a prospective study of 20 twin and 19 singleton pregnancies undergoing amnioreduction for severe polyhydramnios, AFI was measured immediately before and after the procedure and was correlated with the volume of fluid withdrawn. RESULTS: A significant linear relationship was found between AFI change and the volume drained in all pregnancies (Y = -4.2X; R2 = 0.49; P = .002). This relationship was similar in singleton and twin pregnancies. However, there was a significant absolute difference in AFI change between singleton and twin pregnancies of 7.9 cm (95% confidence interval 0.41, 15.2; P = .04). CONCLUSION: These findings support the use of the four-quadrant AFI as a semiquantitative index of amniotic fluid volume. This relationship can be used to predict the drainage volume required to achieve a target AFI with amnioreduction and thus avoid repeated AFI measurements during the procedure itself.

Adult↗

Iatrogenic monoamniotic twins as a complication of therapeutic amniocentesis.

BACKGROUND: Therapeutic amniocentesis has been accepted widely as a safe and efficacious way to treat the polyhydramnios-oligohydramnios sequence associated with twin-twin transfusion syndrome. CASE: A 28-year-old woman, gravida 2, para 1, diagnosed with twin-twin transfusion syndrome at 28 weeks' gestation was treated with serial amniocenteses. The dividing membrane was ruptured inadvertently during therapeutic amniocentesis, with subsequent complete disruption of the amniotic membrane. Iatrogenic monoamniotic twins with cord entanglement and knotting resulted. CONCLUSION: Creation of monoamniotic twins by disruption of the dividing membrane can be a complication of therapeutic amniocentesis for twin-twin transfusion syndrome. Such disruption may result in the same morbidity and mortality that are seen in naturally occurring monoamniotic twins.

Adult↗

Nasopharyngeal teratoma and mosaic tetrasomy 1q detected at amniocentesis. A case report and review of the literature.

The occurrence of nasopharyngeal teratomas (NPT) is an infrequent event and prenatal detection of such tumors is even rarer. We present a case report and review of the literature (N = 78 cases), in which we describe the cytogenetic, DNA, and pathological findings of a fetus with a mature NPT which was detected prenatally by ultrasound investigation following complaints of severe polyhydramnios by the mother.

Adult↗

Obstructive acute renal failure by a gravid uterus: a case report and review.

Acute renal failure caused from an obstruction by the gravid uterus is a rare complication of pregnancy. Only 13 cases have been reported. We report a case of obstructive acute renal failure in a patient at 34 weeks' gestation with a twin pregnancy complicated by polyhydramnios. The serum creatinine peaked at 1,078 mumol/L (12.2 mg/dL), higher than in the previously reported patients. The striking feature of her clinical course was the immediate resolution of anuria following amniotomy, thus avoiding the need for dialysis, ureteral stenting, or immediate surgical delivery. This patient illustrates the potential importance of increased uterine pressure on the ureters as a cause of significant obstructive renal failure during pregnancy.

Acute Kidney Injury↗

Renal tubular dysgenesis-like lesions and hypocalvaria. Report of two cases involving indomethacin.

We describe a case of twins with twin-to-twin transfusion syndrome (TTS) who were found to have renal tubular dysgenesis (TRD)-like lesions and hypocalvaria attributed to indomethacin treatment of the mother for acute polyhydramnios. History of pregnancy, postnatal clinical course, pathological findings of the kidneys, and the skulls are presented and discussed. These findings include incompletely differentiated proximal tubules in the kidneys and hypoplastic calvaria in both twins. The renal tubular lesions were more marked in the donor than in the transfused twin, probably due to the greater degree of ischemia in that twin. This seems to be in favor of a vascular etiology of the renal defects. However, the fact that similar renal lesions and hypocalvaria were also present in the transfused twin seems to indicate that indomethacin played a role in their onset. This so-called kidney-skull connection has never been reported in conjunction with indomethacin therapy.

Adult↗

Fetal ultrasound.

Ultrasound provides an opportunity to visualize the fetus and to assess its response to the intrauterine environment. The topic of fetal ultrasound is broad, and whole textbooks have been devoted to areas such as the diagnosis of anomalies. In an effort to provide current practical information, I have focused on the most common clinical questions asked when pregnant women are referred for obstetric ultrasound. We review the diagnoses of oligohydramnios, polyhydramnios, intrauterine growth retardation (IUGR), macrosomia, multiple pregnancy, and common fetal anomalies. Noninvasive assessment of fetal well-being is addressed, along with the use of sonography to refine risk assessment in patients with abnormal antenatal screening tests.

Congenital Abnormalities↗

[The therapeutic role of tocolysis].

In twin pregnancies, the use of beta-adrenergics is associated with a significantly higher incidence of cardiovascular complications, and calcium channel blockers as well as oxytocin antagonists currently appear as first line agents. After extreme preterm delivery of the first twin and in selected patients, the birth of second twin may be delayed with a mean gain of 10-50 days. In cases of symptomatic placenta previa with mild-to-moderate bleeding, tocolytic agents may be associated with a prolongation of pregnancy and increased birth weight without significant impact on frequency or severity of bleeding. Calcium channel blockers are the drugs of choice in the event of diabetes. Indomethacin is a potent tocolytic, in particular in patients with polyhydramnios. However, it may cause oligohydramnios, premature closure of the ductus arteriosus and intrauterine fetal death when high doses are administered for a duration exceeding 48 to 72 hours, particularly beyond 32 weeks' gestation. The neonatal complications of indomethacin occur frequently. Tocolysis appears to reduce the failure rate of external cephalic version at term.

Female↗

Amniotic fluid volume: fluid dynamics and measurement technique.

Evaluation of amniotic fluid volume is an important aspect of obstetrical ultrasound. Several methods are currently used to describe amniotic fluid volumes. The most common are the subjective assessment of fluid and the semiquantitative methods. The reproducibility of these methods is excellent in experienced hands. Because of the multiple methods used to assess fluid, both oligohydramnios and polyhydramnios have numerous definitions. Regardless of which definition is used, it is clear that both of these entities are associated with a variety of fetal and maternal conditions. Abnormal amniotic fluid volume may be the only or earliest sonographic sign of an obstetrical problem. Therefore, it is important that sonologists are familiar with amniotic fluid volume assessment. Although subjective and semiquantitative techniques may produce similar results, a numerical scale is no substitute for experience and training. One should be able to recognize lesser degrees of fluid abnormality and be able to assess the trend of amniotic fluid volume in serial examinations.

Amniotic Fluid↗

Amniotic fluid and the umbilical cord: the fetal milieu and lifeline.

Amniotic fluid volume should be routinely assessed in every second and third trimester case. A review of amniotic fluid physiology and techniques for ultrasound evaluation of fluid volume is presented. The causes and significance of oligohydramnios and polyhydramnios are stressed. Umbilical cord abnormalities are often incidently observed at the time of amniotic fluid evaluation. The clinical significance of some common umbilical cord abnormalities such as a two-vessel cord and nuchal cord are discussed. Other, more uncommon entities such as cord mass lesions are also reviewed. Finally, the role of cord Doppler interrogation in determining fetal well-being is discussed.

Amniotic Fluid↗

Amniotic fluid embolism and isolated coagulopathy: atypical presentation of amniotic fluid embolism.

A 41-year-old multigravida presented at 32 weeks of gestation with polyhydramnios and an anencephalic fetus. Abnormal bleeding as a result of disseminated intravascular coagulation complicated an emergency Caesarean section for severe abdominal pain thought to be due to uterine rupture. Massive transfusion with blood products was necessary and the abdomen packed to control bleeding. The patient was transferred to the intensive care unit where she made a slow but complete recovery. Amniotic fluid embolism with atypical presentation of isolated coagulopathy is the likely diagnosis in this case. The case serves to demonstrate that amniotic fluid embolism may present with symptoms and signs other than the classical pattern of dyspnoea, cyanosis and hypotension.

Adult↗

Course and outcome of a pregnancy with a giant fetal cervical teratoma diagnosed prenatally.

We report the course and outcome of a pregnancy involving a giant fetal neck teratoma which was diagnosed at 23 weeks of gestation. Sonographic surveillance of the fetal neck revealed continuing growth of the tumor with development of polyhydramnios. Three-dimensional ultrasound provided additional detailed information on the external extent of the lesion. Color Doppler ultrasound showed intense arterial and venous flow with low resistance indices. Cesarean section under general anesthesia was planned in close cooperation with the neonatologist, pediatric surgeon and anesthesiologist because the size of the neck mass precluded vaginal delivery. Cesarean section was performed at 34 weeks of gestation following preterm rupture of the membranes. Orotracheal intubation was not successful because of compression of the airway and a tracheostomy could not be performed because of the risk of severe fetal hemorrhage from the tumor. The neonate died from respiratory insufficiency 66 min after birth.

Adult↗

The use of maternal weight measurements during antenatal care. A national survey of midwifery practice throughout the United Kingdom.

The aim of the present study was to examine the use of maternal weight measurements during antenatal care throughout the United Kingdom. A postal questionnaire and follow-up letter were sent to 1500 midwives throughout the United Kingdom, selected at random from the UKCC register. The postal survey achieved a response rate of 44.8% (672/1500 questionnaires), and obtained responses from at least 10 midwives in all but the lowest grade. Respondents were representative of midwives practising throughout the country, in terms of their gender, working hours and grade, although there were fewer midwives in community settings than those in a contemporary representative English sample. The questionnaire obtained information on the background, training and experience of each midwife, together with their attitudes towards antenatal weighing. For those midwives currently involved in antenatal care, additional information was collected on the schedule of antenatal weight measurements, the criteria used to identify 'abnormal' weight gain, and the action taken in response to 'abnormal' weight gain. 61.8% of the midwives thought that the pattern of maternal weight gain was 'not important' in antenatal care, and only 51.5% of those who currently provided antenatal care weighted women at every antenatal visit. However, most midwives (86.1%) cited at least one clinical condition to explain why women are routinely weighed during pregnancy, and over a third of midwives thought that maternal weight gain could detect seven clinical conditions, including obesity, oedema, pre-eclampsia and polyhydramnios. Midwives with more advanced qualifications (degrees and teaching qualifications) and those working in educational or community settings were least likely to believe that weight gain was good at detecting clinical outcomes. Differences in the perceived utility of antenatal weighing influenced whether midwives would act in response to 'abnormal' maternal weight gain, and whether they advised women to gain or lose weight during pregnancy. However, the criteria that midwives used for identifying 'abnormal' weight gain were variable, and often inappropriate, so that different midwives are unlikely to intervene consistently or to give consistent advice on the basis of maternal weight gain. These differences in practice may lead to extensive and inappropriate variation in antenatal care. Clear guidelines are urgently required to ensure that, if maternal weight measurements are collected during antenatal care, they are collected and used consistently.

Adult↗

Cephalocele detection in utero: sonographic and clinical features.

Sonographic and clinical features of 26 fetal cephaloceles were reviewed retrospectively. The most frequent reason for referral was elevated maternal serum alpha-fetoprotein levels. The smallest lesion identified was 0.4 x 0.5 cm (frontal, at 20 weeks); the largest was 9.0 x 10.0 cm (frontal, at 27 weeks). Twenty-four of 26 cephaloceles were detected on prenatal ultrasound examination; in 13 of these, more than 50% of the intracranial contents were exteriorized. Fifteen of 24 cephaloceles detected prenatally had a sulcal pattern (identified between 16 and 36 weeks' gestation); five were solid without a sulcal pattern (identified between 13 and 21 weeks' gestation), three were cystic, and one underwent a change in appearance from solid at 21 weeks to cystic at 26 weeks. Other cranial features were evaluated and included visible skull defect, seen in 23/24 (96%), ventriculomegaly, in 6/26 (23%); microcephaly, in 12/24 (50%); beaked tectal plate, in 6/16 (38%); and flattened basiocciput, in 9/24 (38%). Of the 26 cases, 14 had normal amniotic fluid volume, five had oligohydramnios and seven had polyhydramnios. Fetuses with oligohydramnios had the highest incidence of concurrent fetal abnormalities; four of five fetuses (80%) with oligohydramnios had additional structural abnormalities. In the overall population, a very high incidence of other abnormalities was found; 17/26 (65%) cases showed additional abnormalities, some of which were not detected by ultrasound. Five fetuses had Meckel-Gruber syndrome and three had amniotic band syndrome. Only one of the 18 karyotypes obtained was abnormal (trisomy 18). Survival was very poor; only two of the eight who survived until birth are currently living.

Abnormalities, Multiple↗

The hyperechogenic lungs of laryngotracheal obstruction.

A case is reported in which enlarged, hyperechogenic lungs, ascites and polyhydramnios on prenatal ultrasound were indications of a partial tracheal agenesis and tracheoesophageal fistula. A review of the literature is given to assist clinicians in effectively counselling women in whom this ultrasound abnormality is detected.

Adult↗

Intrauterine sling: a complication of the stuck twin syndrome.

Stuck twin syndrome usually presents with polyhydramnios in the recipient sac and severe oligohydramnios in the donor sac. The donor is displaced against the uterine wall and remains adherent in that position. We present a case in which the diagnosis was more complicated, owing to the suspension of the stuck twin by a sling within the sac of the recipient. A monochorionic diamnionic twin gestation was complicated by twin-twin transfusion syndrome at 18 weeks of gestation. In our example, the stuck twin was suspended by a sling from the placenta. The sling band represented the intertwin membrane that was folded upon itself. Amniotic fluid from the recipient twin was present in three dimensions around the stuck twin, except for the sling band. The suspension of the stuck twin by a sling within the amniotic fluid of the recipient is an unusual manifestation of the stuck twin syndrome.

Adult↗