Search

Objective: To analyze outcomes of singleton pregnancies with idiopathic polyhydramnios through a systematic review and meta-analysis., Methods: Electronic databases, including MEDLINE, OVID, EBSCO, Cochrane collection and Science Citation Index, were searched from 1946 to 2019. Gray literature and tables of contents of relevant journals were also screened. Prospective and retrospective studies with a control group were included. Two authors independently reviewed the abstracts retrieved from the literature search. Inclusion criteria were: studies documented in English, singleton pregnancy and idiopathic polyhydramnios determined by amniotic fluid volume assessment on ultrasound. Exclusion criteria were: maternal diabetes, fetal structural or chromosomal anomaly, alloimmunization and intrauterine fetal infection., Results: Twelve studies met the inclusion criteria, giving a total of 2392 patients with idiopathic polyhydramnios and 160 135 patients with normal amniotic fluid volume. Pregnancies complicated by idiopathic polyhydramnios were at a higher risk of neonatal death (odds ratio (OR), 8.68 (95% CI, 2.91-25.87)), intrauterine fetal demise (OR, 7.64 (95% CI, 2.50-23.38)), neonatal intensive care unit admission (OR, 1.94 (95% CI, 1.45-2.59)), 5-min Apgar score < 7 (OR, 2.21 (95% CI, 1.34-3.62)), macrosomia (OR, 2.93 (95% CI, 2.39-3.59)), malpresentation (OR, 2.73 (95% CI, 2.06-3.61)) and Cesarean delivery (OR, 2.31 (95% CI, 1.79-2.99))., Conclusions: This study suggests that pregnancies complicated by idiopathic polyhydramnios are at increased risk of adverse outcome. Future investigations should aim to determine an amniotic fluid volume threshold above which antenatal fetal surveillance is appropriate in the management of these pregnancies. © 2022 International Society of Ultrasound in Obstetrics and Gynecology., (© 2022 International Society of Ultrasound in Obstetrics and Gynecology.)

Objective: Esophageal atresia and/or tracheoesophageal fistula (EA/TEF) remains one of the most frequently missed congenital anomalies prenatally. The aim of our study was to elucidate the sonographic manifestation of EA/TEF throughout pregnancy., Methods: This was a retrospective study of data obtained from a tertiary center over a 12-year period. The prenatal ultrasound scans of fetuses with EA/TEF were assessed to determine the presence and timing of detection of three principal signs: small/absent stomach and worsening polyhydramnios, both of which were considered as 'suspected' EA/TEF, and esophageal pouch, which was considered as 'detected' EA/TEF. We assessed the yield of the early (14-16 weeks' gestation), routine mid-trimester (19-26 weeks) and third-trimester (≥ 27 weeks) anomaly scans in the prenatal diagnosis of EA/TEF., Results: Seventy-five cases of EA/TEF with available ultrasound images were included in the study. A small/absent stomach was detected on the early anomaly scan in 3.6% of fetuses scanned, without a definitive diagnosis. On the mid-trimester scan, 19.4% of scanned cases were suspected and 4.3% were detected. On the third-trimester anomaly scan, 43.9% of scanned cases were suspected and 33.9% were detected. An additional case with an esophageal pouch was detected on magnetic resonance imaging (MRI) in the mid-trimester and a further two were detected on MRI in the third trimester. In total, 44.0% of cases of EA/TEF in our cohort were suspected, 33.3% were detected and 10.7% were suspected but, eventually, not detected prenatally., Conclusions: Prenatal diagnosis of EA/TEF on ultrasound is not feasible before the late second trimester. A small/absent stomach may be visualized as early as 15 weeks' gestation. Polyhydramnios does not develop before the mid-trimester. An esophageal pouch can be detected as early as 22 weeks on a targeted scan in suspected cases. The detection rates of all three signs increase with advancing pregnancy, peaking in the third trimester. The early and mid-trimester anomaly scans perform poorly as a screening and diagnostic test for EA/TEF. © 2020 International Society of Ultrasound in Obstetrics and Gynecology., (© 2020 International Society of Ultrasound in Obstetrics and Gynecology.)

Objectives: To identify antenatal ultrasound markers that can differentiate between simple and complex gastroschisis and assess their predictive value., Methods: This was a prospective nationwide study of pregnancies with isolated fetal gastroschisis that underwent serial longitudinal ultrasound examination at regular specified intervals between 20 and 37 weeks' gestation. The primary outcome was simple or complex (i.e. involving bowel atresia, volvulus, perforation or necrosis) gastroschisis at birth. Fetal biometry (abdominal circumference and estimated fetal weight), the occurrence of polyhydramnios, intra- and extra-abdominal bowel diameters and the pulsatility index (PI) of the superior mesenteric artery (SMA) were assessed. Linear mixed modeling was used to compare the individual trajectories of cases with simple and those with complex gastroschisis, and logistic regression analysis was used to estimate the strength of association between the ultrasound parameters and outcome., Results: Of 104 pregnancies with isolated fetal gastroschisis included, four ended in intrauterine death. Eighty-one (81%) liveborn infants with simple and 19 (19%) with complex gastroschisis were included in the analysis. We found no relationship between fetal biometric variables and complex gastroschisis. The SMA-PI was significantly lower in fetuses with gastroschisis than in healthy controls, but did not differentiate between simple and complex gastroschisis. Both intra- and extra-abdominal bowel diameters were larger in cases with complex, compared to those with simple, gastroschisis (P < 0.001 and P < 0.005, respectively). The presence of intra-abdominal bowel diameter ≥ 97.7 th percentile on at least three occasions, not necessarily on successive examinations, was associated with an increased risk of the fetus having complex gastroschisis (relative risk, 1.56 (95% CI, 1.02-2.10); P = 0.006; positive predictive value, 50.0%; negative predictive value, 81.4%)., Conclusions: This large prospective longitudinal study found that intra-abdominal bowel dilatation when present repeatedly during fetal development can differentiate between simple and complex gastroschisis; however, the positive predictive value is low, and therefore the clinical usefulness of this marker is limited. © 2019 Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology., (© 2019 Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.)

Objectives: To establish gestational age-specific reference ranges for amniotic fluid measurements in monochorionic diamniotic twin pregnancies, to compare them with previously reported singleton and twin reference ranges and to examine the rationale for using a gestational age-dependent cut-off to define polyhydramnios in twin-twin transfusion syndrome, as is the practice in most European centers., Methods: We retrospectively evaluated amniotic fluid volume in 32 monochorionic diamniotic twin pregnancies that were followed longitudinally at 2-week intervals from the first trimester until birth. Amniotic fluid volume was assessed by measuring the deepest vertical pocket in both amniotic sacs. We used multilevel modeling to estimate the gestational age-specific reference ranges for deepest vertical pocket measurements., Results: Based on 429 observations in 64 fetuses, we constructed gestational age-specific reference ranges from 11 weeks until term. The deepest pocket increased from the first trimester to reach a maximum at 26 weeks, followed by a gradual decrease towards term. Measurements between 18 and 28 weeks were comparable to those in singleton pregnancies. However, before 18 weeks values were higher, whereas after 28 weeks they were lower, as compared to singleton references., Conclusion: In monochorionic twin pregnancies, the deepest vertical pocket is a gestational age-dependent measurement. Therefore, a gestational age-dependent definition of polyhydramnios in twin-twin transfusion syndrome, as used by most European centers, seems a logical approach., (Copyright © 2013 ISUOG. Published by John Wiley & Sons Ltd.)

Twin reversed arterial perfusion (TRAP) sequence is a serious complication of monochorionic twin pregnancies, in which arterioarterial anastomoses allow blood flow from a 'pump' fetus to an acardiac fetus via reversed flow in the latter's umbilical artery. Several trial treatments for TRAP sequence have been reported, but all of these have been invasive. We present a case of TRAP sequence in which high-intensity focused ultrasound (HIFU) was applied to the umbilical artery of the anomalous twin at 26 weeks as a non-invasive fetal therapy. The HIFU intensity was set at approximately 2300 W/cm(2) with exposure periods of 10 s. Color Doppler ultrasound showed a decrease in blood supply to the anomalous twin, although complete occlusion of the targeted vessel was not achieved. Delivery was by Cesarean section at 29 weeks' gestation and the pump twin survived, without severe clinical complications at 6 months., (Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd.)

'Starry sky' liver is one of the most common sonographic patterns in diffuse liver disease. It is characterized by clearly identified portal venules due to diminished parenchymal echogenicity. In advanced cases of twin-to-twin transfusion syndrome (TTTS), volume overload is considered the key factor in the pathogenesis of cardiac dysfunction of the recipient twin. When right-sided failure occurs, the liver might show signs of edema and, as in acute hepatitis, the appearance of starry sky might develop. We present a case in which the sonographic appearance of starry sky liver, along with right-sided cardiac failure (tricuspid regurgitation), were the first signs of TTTS in monochorionic twins at 20 weeks. A short time later, at 21 weeks, other signs of overload and signs of worsening heart failure were noted, as the typical triphasic waves in the inferior vena cava were replaced by a biphasic flow profile. Twin 2 at that time had relative oligohydramnios. A few days later, relative polyhydramnios and edema of the placental domain of the recipient twin were also noted. To the best of our knowledge, this is the first case report describing this hepatic sonographic pattern as an early sonographic sign of TTTS., (Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: To evaluate fetal thoracoamniotic shunting for isolated large macrocystic congenital cystic adenomatoid malformations (CCAM) of the lung., Methods: This was a retrospective study of 11 fetuses with macrocystic CCAM who underwent thoracoamniotic shunting. This procedure was offered if fetal hydrops or signs of evolving hydrops (such as ascites or polyhydramnios) were present, or when there were very large lesions or lesions rapidly increasing in size. If there were multiple large cysts within the lesion, a single shunt was used, aiming to traverse several cysts., Results: Shunts were inserted at a mean gestational age of 24.6 (range, 17-32) weeks. Marked mediastinal shift was present in all cases. Six fetuses were hydropic and, of the remaining five, one had severe polyhydramnios, three had lesions that were rapidly increasing in size and one had a very large lesion at initial presentation. In total, four cases had polyhydramnios. Shunting one cyst always decompressed the entire lesion and hydrops and/or polyhydramnios resolved in all surviving fetuses. One hydropic fetus that underwent the procedure at 17 weeks died 1 day later. The shunt dislodged in one case and the lesion did not re-expand. No mother went into labor or had ruptured membranes before 35.6 weeks. Mean gestational age at delivery was 38.2 weeks (n = 10). All pregnancies were delivered vaginally, with no maternal complications. All newborns had uneventful lobectomies, and pathology confirmed CCAM in all cases., Conclusion: Fetal thoracoamniotic shunting for large macrocystic CCAM is associated with favorable outcome in most cases, and should be considered in severe cases even before hydrops develops., (Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd.)

We describe a case of congenital meconium peritonitis with progressive fetal ascites and polyhydramnios. Fetal ascites could be only partially reduced on paracentesis at 29 weeks' gestation, and it subsequently increased. Urinary trypsin inhibitor (UTI), a physiological anti-inflammatory substance, was administered into the fetal abdominal cavity at a second paracentesis performed at 35 weeks' gestation. There was a significant amount of fetal ascites remaining 1 day after the second paracentesis, but this completely resolved within 5 days. A healthy infant was delivered vaginally and no surgical intervention was required. The case suggests that UTI can reduce meconium-induced chemical peritonitis and thereby facilitate intrauterine remission of fetal ascites., (Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: This study was carried out to evaluate the perinatal outcomes of pregnancy with pregestational diabetes mellitus complicated by polyhydramnios., Methods: This was a retrospective study of singleton pregnancies, with an antepartum diagnosis of polyhydramnios, seen at the maternal fetal medicine department of Mater Mothers' Hospital, a tertiary-level facility. All pregnancies in women with pregestational diabetes with a singleton pregnancy beyond 24 weeks of gestation, from 1996 to 2006, were reviewed (n = 314), and pregnancies complicated by polyhydramnios were identified (n = 59). Pregnancy outcomes of women whose pregnancy was complicated with polyhydramnios were compared to those without this complication., Results: The incidence of polyhydramnios in the study population was 18.8%. Women with polyhydramnios had increased hemoglobin A1c (HbA1c) levels throughout the pregnancy, and the difference was significant during the prepregnancy period and in the third trimester (P = 0.003 and P = 0.025, respectively). Significantly more mothers in the polyhydramnios group delivered preterm (54.2% vs. 33.3%, P = 0.004), the majority of which were iatrogenic preterm deliveries (44.1%). More pregnancies with polyhydramnios were delivered by Cesarean section (83.0% vs. 62%; P = 0.006), with the majority being performed electively in both groups (79.6% and 70.3%, respectively). Regardless, there were no significant differences in perinatal mortality rates, congenital abnormality rates, the incidences of low Apgar score, acidemia, hypoglycemia requiring intravenous therapy, phototherapy and ventilatory needs between the babies of the two groups., Conclusion: Pregestational diabetic pregnancy with polyhydramnios is associated with poor diabetic control. Despite this, there is no significant increase in adverse perinatal outcome in these pregnancies, apart from a higher iatrogenic preterm birth rate., ((c) 2010 ISUOG. Published by John Wiley & Sons, Ltd.)

Objectives: In polyhydramnios, amniotic fluid (AF) volume can be increased not only as a result of increased fetal urine production, but also due to several other factors, including impairment of both fetal swallowing and gastrointestinal (GI) absorption of AF. Our aim was to evaluate whether measurement of the fetal urine production rate (UPR) can be used to differentiate the causes of increased AF volume., Methods: This cross-sectional study included 54 pregnant women with an increased amniotic fluid index (AFI), defined as AFI > or = 18 cm, divided into two groups according to the presence of fetal anomalies that are associated with impairment of fetal swallowing or decreased GI absorption of AF (Group 1, n = 14) or the absence of fetal anomalies (Group 2, n = 40). The control group included 96 normal pregnancies with normal AFI (8 < or = AFI < 18 cm) (Group 3). Fetal UPR was obtained by serial bladder volume measurements (two to four times, with a median interval of 5 min between each) using the rotational method of Virtual Organ Computer-aided AnaLysis (VOCAL()) with three-dimensional ultrasound. To adjust for fetal weight (Wt) and gestational age (GA), UPR&#95;Wt and UPR&#95;SD were calculated using the following formulae: UPR&#95;Wt = measured UPR/estimated fetal weight and UPR&#95;SD = (measured UPR - mean UPR for each GA)/SD of UPR for each GA., Results: The AFI was increased significantly in Groups 1 and 2 compared with Group 3. However, the median fetal UPR in Group 1 did not differ from that of Group 3, in contrast to the higher median fetal UPR in Group 2 compared with Groups 1 and 3; this difference remained significant after adjusting for GA and estimated fetal weight in terms of UPR&#95;SD and UPR&#95;Wt. In Groups 2 and 3, AFI and UPR had a positive correlation in terms of UPR, UPR&#95;SD and UPR&#95;Wt., Conclusions: Our findings that fetal UPR is significantly increased in cases with increased AFI without fetal anomalies, but not in those with increased AFI and fetal anomalies involving decreased GI absorption of AF, might be used to differentiate causes of increased AF volume. In the absence of fetal anomalies, AFI and fetal UPR correlate positively.

Objectives: Giant placental chorioangiomas are associated with a high prevalence of pregnancy complications and a poor perinatal outcome. The aim of the study was to evaluate the natural history, intrauterine treatment and outcome of pregnancies complicated by giant placental chorioangioma., Methods: This was a retrospective study of 19 cases of giant placental chorioangioma, in which the natural history, intrauterine treatment and outcome of pregnancy were evaluated., Results: Eighteen of the 19 cases were associated with a wide variety of fetal complications, including polyhydramnios, growth restriction, hyperdynamic circulation, cardiomegaly, anemia and non-immune hydrops. Two-thirds of the cases developed complications that required either elective delivery for fetal growth restriction (n = 6) or intervention for cardiovascular effects (n = 7). Fetal therapy, when required, was tailored to the particular complication and resulted in a successful pregnancy in all cases., Conclusions: The presence of large placental chorioangioma is associated with the development of fetal growth restriction and hyperdynamic state in a significant number of cases. This series elucidates some of the putative underlying mechanisms for these complications, and reports on a safe and effective treatment modality, percutaneous ultrasound-guided interstitial laser therapy., ((c) 2009 ISUOG. Published by John Wiley & Sons, Ltd.)

Brachytelephalangic chondrodysplasia punctata is a rare congenital skeletal dysplasia. Within the heterogeneous group of chondrodysplasia punctata, the brachytelephalangic type is noteworthy because it has a better prognosis than do the other types. We report a case of brachytelephalangic chondrodysplasia punctata diagnosed by ultrasound imaging at 30 weeks' gestation; it was associated with polyhydramnios and a normal cervical spinal canal. Imaging features are described and differential diagnosis with other forms of chondrodysplasia punctata is discussed., (Copyright 2009 ISUOG. Published by John Wiley & Sons, Ltd.)

Objectives: To assess cardiovascular findings in twin-reversed arterial perfusion (TRAP) sequence pre- and post-therapy and compare these findings to traditional obstetric markers, defined as acardius to pump twin weight ratio and presence of polyhydramnios., Methods: This was a retrospective review of 27 cases of TRAP sequence diagnosed between 2004 and 2008. Echocardiographic data included indexed cardiac output and functional and anatomic parameters. Ultrasound reports were reviewed for acardius to pump twin weight ratio and polyhydramnios. We assessed the relationship between cardiac output and the remaining cardiac/obstetric variables obtained pre- and post-treatment., Results: Twenty-three subjects had complete echocardiographic data sets at initial evaluation (mean gestational age, 20.4 +/- 2.5 weeks) and, of these, post-treatment echocardiographic evaluation was available in 10. Six of seven (86%) pump twins with elevated indexed cardiac output had significant cardiovascular compromise. Most fetuses with abnormal cardiac output or right ventricular dysfunction normalized post-therapy. There was no relationship between cardiac output and obstetric markers., Conclusions: Elevated indexed cardiac output is strongly associated with cardiovascular compromise. Traditional obstetric prognosticators do not correlate with cardiovascular derangements. In pump twins with cardiac compromise, postoperative cardiovascular status improves acutely. Given this analysis, we conclude that assessment of cardiovascular findings should be incorporated into the management and treatment of TRAP sequence., (Copyright (c) 2009 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: To evaluate the clinical use of hourly fetal urine production rate (HFUPR) in polyhydramnios., Methods: This was a retrospective review of 33 singleton pregnancies with polyhydramnios, 30 of them unexplained and three due to gastrointestinal atresia. HFUPR was estimated using three-dimensional ultrasound and was compared with recently established nomograms. Abnormal midterm outcome, defined as diagnosis or persistence of pathology after the neonatal period until the age of 2 years, was analyzed according to prenatal HFUPR measurements and other polyhydramnios characteristics., Results: Seventeen of the 30 fetuses with unexplained polyhydramnios had an HFUPR above the 95(th) centile, and five (29.4%) of them developed midterm disorders. None of the 13 with normal HFUPR developed midterm disorders. The HFUPR was 1.9 (SD, 0.7) multiples of the median (MoM) in fetuses with an adverse childhood outcome and 1.4 (SD, 1.2) in fetuses with normal childhood outcome (P = 0.34). In the three fetuses with gastrointestinal atresia, the HFUPR was significantly lower than in those with unexplained polyhydramnios (P = 0.003)., Conclusion: HFUPR was associated with the mechanism of polyhydramnios but failed to help in the prognosis of unexplained polyhydramnios because of lack of power. Children with prenatal unexplained polyhydramnios and HFUPR above the 95(th) centile should nevertheless receive detailed pediatric follow-up., (Copyright (c) 2009 ISUOG. Published by John Wiley & Sons, Ltd.)

Objectives: To determine whether the pre- or postnatal diagnosis of either isolated or non-isolated duodenal obstruction (DO) is associated with different outcomes., Methods: A single-center retrospective analysis was carried out of 91 cases diagnosed with a DO between January 1991 and June 2003. Data on the diagnosis, treatment and outcomes of the cases were gathered, and differences between the groups were analyzed., Results: Twenty-eight cases of DO were diagnosed before and 63 after birth. Of 15 presumed isolated cases in the prenatally diagnosed group, four revealed associated or chromosomal anomalies after birth. The types of obstruction present were significantly different between the prenatally (n = 11) and postnatally (n = 27) detected subsets of isolated DO. The prenatally detected subset displayed a lower median gestational age at delivery, lower median birth weight and a higher prematurity rate (8/11 vs. 8/27). The diagnosis of DO occurred significantly later in the postnatally detected subset than the postnatal confirmation of the diagnosis in the prenatally detected cases. In the non-isolated cases of DO, no difference was found in the type of chromosomal or associated anomaly or the type of obstruction between the prenatally detected (n = 17) and postnatally detected subsets (n = 36). Trisomy 21 was present in 7/17 (41%) vs. 22/36 (61%) cases, respectively. Two terminations and three intrauterine deaths occurred in the prenatal non-isolated subset. The liveborn infants from the prenatally detected non-isolated subset (n = 12) showed a significantly higher prematurity rate (9/12 vs.14/36), lower median birth weight and earlier confirmation of diagnosis after delivery. After surgery, outcome was similar between both subsets of isolated and non-isolated DO. All the infants with an isolated DO survived. Neonatal death occurred in three prenatally and five postnatally diagnosed cases with non-isolated DO., Conclusions: The outcome of prenatally and postnatally diagnosed DO is not essentially different despite more prematurity and a lower birth weight in the former. Of the prenatally detected cases of DO assumed to be isolated, 25% revealed additional chromosomal or associated anomalies after delivery, which influenced outcome., ((c) 2008 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: Prenatal diagnosis of esophageal obstruction is believed to improve the outcome for the affected newborn. However, the prenatal detection rate is only 10-40%, the diagnosis is usually not made before the third trimester and the false-positive rate has been high. This study investigated the prenatal detection rate and time of prenatal diagnosis at our center and its influence on outcome. In addition, incidence, detection rate and accuracy of the diagnosis in a large non-selected population were determined., Methods: All cases diagnosed pre- or postnatally with esophageal obstruction and examined prenatally by ultrasound at the National Center for Fetal Medicine in Norway during 1987-2004 were evaluated., Results: Of 48 cases with esophageal obstruction, 21 (44%) were diagnosed prenatally (median, 32 + 0 weeks). All 21 had a small or empty stomach, 20/21 (95%) had polyhydramnios and 9/21 (43%) had a visible esophageal pouch. Associated anomalies were present in 38/48 cases (79%). The karyotype was abnormal in 11/48 cases (23%). Ten (21%) pregnancies with lethal fetal conditions were terminated. Two fetuses died in utero. Ten infants with associated anomalies died within 3 months after birth. The 26/48 (54%) survivors included 16/21 cases with a prenatal diagnosis of esophageal obstruction and 9/10 cases with isolated esophageal obstruction., Conclusions: The clinical signs of polyhydramnios were the most important factors for prenatal detection of esophageal obstruction. Consequently, the time of diagnosis was late and the detection rate was low (44%). An increased awareness of the possibility of esophageal obstruction, leading to targeted examinations whenever the suspicion is raised during pregnancy, might improve the prenatal detection rate and thereby provide a possibility to improve the outcome. Of the cases with isolated esophageal obstruction, 90% survived, irrespective of prenatal diagnosis., (Copyright (c) 2007 ISUOG. Published by John Wiley & Sons, Ltd.)

Bronchopulmonary sequestration (BPS), a non-functional pulmonary tissue mass, when complicated by fetal hydrops, carries a high risk of perinatal mortality. However, a limited number of cases of BPS complicated by fetal hydrops with an informative clinical course have been reported. We report here on three cases of BPS complicated by fetal hydrothorax and hydrops that were successfully treated by pleuroamniotic shunting, which should be considered as a treatment option for fetal hydrothorax and hydrops associated with BPS., (Copyright 2006 ISUOG. Published by John Wiley & Sons, Ltd.)

Objective: The aims of the study were to determine cervical length among patients with polyhydramnios and to assess the relationship between the severity of polyhydramnios, cervical length and gestational age at delivery., Patients and Methods: A prospective study was designed including 92 consecutive singleton pregnancies with polyhydramnios between 24 and 40 weeks' gestation. Cervical length was measured using transvaginal sonography. Polyhydramnios was defined when amniotic fluid index (AFI) was equal to or greater than 20 cm. A single sonologist performed all the examinations of the cervical length and the AFI., Results: The median cervical length and AFI were 37.5 (range, 7-52) mm and 28.8 (range, 20-43) cm, respectively. A significant gradual shortening of the cervical length was observed with advancing gestational age (P=0.027). No significant association was found between AFI and cervical length (P=0.24). A cut-off of 15 mm (n=5) was associated with a significantly lower gestational age at delivery (30+/-2.6 weeks vs. 37.2+/-4.2 weeks, respectively, P<0.001)., Conclusions: Women with polyhydramnios have a gradual shortening of cervical length with advancing gestational age. However, this finding is not related to the severity of polyhydramnios., (Copyright (c) 2006 ISUOG.)

Objective: To help develop an evidence-based approach to the best management of twin pregnancies discordant for anencephaly., Methods: We retrospectively examined the management and outcome of 18 pregnancies discordant for anencephaly diagnosed at 11 + 0 to 13 + 6 weeks of gestation in our center. We combined these data with those from other publications. In total, there were 44 dichorionic pregnancies that were managed expectantly (n = 35) or by selective feticide (n = 9) and 19 monochorionic pregnancies that were managed expectantly. We also reviewed the literature to ascertain the outcome of monochorionic twin pregnancies undergoing cord occlusion., Results: In the 35 dichorionic pregnancies that were managed expectantly, 20 (57.1%) developed polyhydramnios at 25-31 weeks; 13 were managed expectantly, five had amniodrainage and two had selective feticide. In 34 of the 35 cases the non-anencephalic twin was liveborn at a median gestation of 36 (range, 28-39) weeks and in six (17.6%) of these it was born before 33 weeks. In the dichorionic pregnancies that had selective feticide, there was one miscarriage and eight (88.9%) live births at a median gestation of 37 (range, 30-40) weeks and in one (12.5%) of these it was born before 33 weeks. In the monochorionic pregnancies, four (21.1%) anencephalic fetuses died at 20-32 weeks and in three of these the normal co-twin also died. In the 16 (84.2%) cases resulting in the live birth of the normal twin, delivery occurred at a median gestation of 33 (range, 27-39) weeks and in six (37.5%) of these it was before 33 weeks. Ultrasound-guided bipolar cord coagulation in 92 pregnancies, mostly complicated by twin reversed arterial perfusion sequence or severe twin-to-twin transfusion syndrome, was associated with a survival rate of 77.2% and early preterm delivery rate of 31.0%., Conclusion: Dichorionic twins discordant for anencephaly are best managed with serial ultrasound examinations for early diagnosis of polyhydramnios, which can then be treated either by amniodrainage or selective feticide. In monochorionic twins it is uncertain whether the best management is expectant or by cord occlusion., (Copyright 2006 ISUOG. Published by John Wiley & Sons, Ltd.)

Epidermolysis bullosa with pyloric atresia (EB-PA) is a rare autosomal recessive genetic disease with a poor prognosis. We report a case of EB-PA in a non-consanguineous couple with a non-contributory family history. The primigravid woman was referred to us because of polyhydramnios associated with fetal gastric dilatation at 33 weeks of gestation. Maternal serum alpha-fetoprotein (AFP) had been elevated at 15 weeks' gestation (3.08 multiples of the median), and ultrasound examination showed polyhydramnios with echogenic amniotic fluid, gastric dilatation, and no other associated malformation. The fetal karyotype was normal female (46,XX). Acetylcholinesterase (ACHe) and AFP levels in the amniotic fluid were normal. Labor occurred spontaneously at 35 weeks' gestation. Clinical examination of the newborn showed large areas of cutaneous blisters and erosions, as well as pyloric atresia. Immunofluorescence analysis of skin samples confirmed EB-PA. Molecular analysis showed a new mutation of the integrin beta-4 gene: heterozygote missense deletions (3807delC/310delC, respectively, exons 31 and 5). The child died from severe sepsis at the age of 13 days. Our observation emphasizes the difficulty of interpreting prenatal ultrasound findings when there is no suggestive context., (Copyright 2005 ISUOG)

We describe a case of congenital nephroblastoma (Wilms' tumor) presenting at 28 weeks of gestation with fetal hydrops and polyhydramnios. Prenatal diagnosis was made by biopsy. An emergency Cesarean section was performed due to deterioration in the cardiotocograph. A post-mortem examination confirmed the diagnosis of congenital nephroblastoma.

We describe a case of congenital cystic adenomatoid malformation treated by thoracoamniotic shunting using a double-flower catheter. An ultrasound examination at 25 weeks' gestation revealed a cystic mass within the fetal thoracic cavity, hydrops and polyhydramnios. Thoracoamniotic shunting under ultrasound guidance using a double-flower catheter was performed at 27 weeks. The procedure not only decompressed the lung cyst but also improved the secondary physiological changes, i.e. mediastinal shift, hydrops and polyhydramnios. A 2438-g male neonate was delivered at 37 weeks with no respiratory problems and resection of the right lower pulmonary lobe was successfully performed 24 h after birth.

Fetal hemoglobin concentration was measured in each twin from five monochorionic pregnancies complicated by twin-to-twin transfusion, diagnosed by growth discordance and coexistent oligohydramnios-polyhydramnios. In three cases the hemoglobin concentration of the smaller twin with oligohydramnios was lower than the normal mean for gestation, and the hemoglobin difference was more than 5 g/dl in two of these cases. In the other two cases, the hemoglobin concentration of the four fetuses with coexistent oligohydramnios-polyhydramnios was within the normal range. This suggests that twin-to-twin transfusion may occur without hemoglobin changes, or that alternatively coexistent oligohydramnios-polyhydramnios occurs in the absence of twin-to-twin transfusion.

A case is described in which intra-amniotic debris was identified during an ultrasound scan in a woman with polyhydramnios at 33 weeks' gestation. At delivery the neonate was found to have severe ichthyosis involving the whole of her head, trunk and limbs, with large plaques of hyperkeratotic skin on the palms of her hands and the soles of her feet. The finding of excessive debris in association with polyhydramnios should raise the possibility of an exfoliative skin disorder.

We present a case of meconium peritonitis which was associated with a short bowel and complicated by progressive bowel distension and difficulty in making a definitive diagnosis of cystic fibrosis. Treatment was by bowel resection and an ileostomy (and later bowel anastomosis), followed by parenteral nutrition which was complicated by hepatitis. The literature is reviewed and management dilemmas and options are discussed.

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.

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.

The study was designed to investigate the implications of the sonographic diagnosis of the two-vessel umbilical cord for patient counselling and pregnancy management. Retrospective analysis was carried out of prenatal findings and pregnancy outcomes when a two-vessel cord was diagnosed in utero. Eighty-two fetuses each with a single umbilical artery were diagnosed by ultrasound. Ten were aneuploid, including nine with visible structural defects and one with early onset intrauterine growth retardation. Of the remaining 72, 31 had other anomalies diagnosed postnatally; 27 of these had structural defects detected on ultrasound examination. However, in nine of these 27 sonographically abnormal fetuses, one or more major structural defects were missed by ultrasound examination. Among the 45 chromosomally normal fetuses with no visible defects on scan, four had anomalies diagnosed after birth. Among the chromosomally normal singletons, six of 22 with other anomalies seen on scan and seven of 38 with no other visible defects on scan had intrauterine growth retardation. Among chromosomally normal twins, one of two with other anomalies seen and two of five appearing otherwise normal had intrauterine growth retardation; one twin set was delivered at 23 weeks after the demise of both twins. Karyotyping is recommended whenever a two-vessel cord is seen in association with symmetric intrauterine growth retardation or any other defect. The fetus diagnosed with a two-vessel cord and any other anomaly by ultrasound often has additional structural defects not seen on scan. The fetus with an isolated two-vessel cord on scan seldom has unrecognized major anomalies, but is at risk for intrauterine growth retardation.