Additional value of advanced neurosonography and magnetic resonance imaging in fetuses at risk for brain damage.

OBJECTIVE: To assess the additional value of fetal multiplanar (axial, coronal and sagittal) neurosonography and magnetic resonance imaging (MRI) to that of the standard axial ultrasound planes in diagnosing brain damage in fetuses at high risk. METHODS: This was a prospective, multicenter, observational study. Women were eligible for participation if their fetus was at risk for acquired brain anomalies. Risk factors were congenital infection, alloimmune thrombocytopenia, fetal growth restriction, trauma during pregnancy, fetal hydrops, monochorionic twins and prior ultrasound finding suggestive of an acquired brain anomaly. Examinations of the fetal brain before birth comprised axial ultrasound and advanced neurosonography biweekly and MRI once. After birth, neonatal cranial ultrasound was performed at < 24 h and at term-equivalent age. Neonatal brain MRI was performed once at term-equivalent age. An expert panel blinded to medical information, including imaging findings by the other methods, evaluated the presence of periventricular echogenicity (PVE) changes, peri- and intraventricular hemorrhage (IVH) and changes in basal ganglia and/or thalami echogenicity (BGTE) on ultrasound, and the equivalent signal intensity (SI) changes on MRI. Conclusions on imaging findings were generated by consensus. The children were followed up with examinations for psychomotor development at 1 year of age, using the Touwen examination and Alberta Infant Motor Scale, and at 2 years of age using Bayley Scale of Infant Development-III (BSID-III) and behavioral, sensory profile and linguistic questionnaires; scores > 1 SD below the mean were considered suspicious for neurodevelopmental sequelae. RESULTS: Fifty-six fetuses were examined, and in 39/56 fetuses, all fetal-imaging modalities were available. PVE/SI changes were observed in 6/39, 21/39 and 2/39 fetuses on axial ultrasound planes, multiplanar neurosonography and MRI, respectively. IVH was found in 3/39, 11/39 and 1/39 fetuses, and BGTE/SI changes in 0/39, 12/39 and 0/39 fetuses, respectively. Outcome was suspicious for neurodevelopmental sequelae in 13/46 infants at 1 year, and at 2 years, 41/41 children had scores within 1 SD of the mean on BSID-III and 20 had scores > 1 SD below the mean on the behavioral (5/38), sensory profile (17/37) and/or linguistic (6/39) questionnaires. CONCLUSIONS: In this cohort of fetuses at risk for brain damage, the severity of acquired brain anomalies was limited. Nevertheless, multiplanar neurosonography detected more fetal PVE changes, IVH and/or BGTE changes compared to the standard axial ultrasound planes and MRI. Fetal MRI did not demonstrate any anomalies that were not seen on neurosonography. Neurodevelopmental outcome at 2 years of age showed no or mild impairment in most cases. © 2019 Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Ultrasound markers for prediction of complex gastroschisis and adverse outcome: longitudinal prospective nationwide cohort study.

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 ≥ 97.7th 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.

Premaxillary protrusion assessment by the maxilla-nasion-mandible angle in fetuses with facial clefts.

OBJECTIVE: The aim of the study was to measure the degree of premaxillary protrusion in fetuses with orofacial clefts of various severities. METHODS: The maxilla-nasion-mandible (MNM) angle was measured retrospectively on by multiplanar corrected volumes. Sixty-two fetuses with orofacial clefts and known outcome volumes of the fetal head were available. RESULTS: In 48 of the 62 cases, the MNM angle could be measured [mean gestational age 23 (range, 18-30) weeks]. The mean MNM angle was normal in all nine cases with cleft lip and intact alveolar ridge (15.2°; range, 12.5°- 16.9°). In 24 cases with unilateral cleft lip with or without cleft palate (UCL/P), the mean MNM angle was 20.0° (range, 13.3-26.2°), being above the 95th percentile in 79% (n = 19) and normal in 21% (n = 5). In 14 bilateral cleft lip and palate (BCL/P) cases, the mean MNM angle was 26.5° (range, 19.2°-33.7°) and above the 95th percentile in all cases. There was no difference in MNM angle between isolated clefts and clefts associated with other anomalies. In one case with a Tessier 4 cleft, the MNM angle was above the 95th percentile (25.2°). CONCLUSION: The premaxilla tends to protrude in both BCL/P as UCL/P cases. The degree of protrusion varies greatly, especially in the BCL/P group.

Isolated abdominal wall defect with complete liver herniation without a covering or remnant membrane: an ominous sign: case report and review of literature.

Complete liver herniation in abdominal wall defects without a membrane is rare and its prognosis is not well documented. We present a case diagnosed at 12 weeks of gestation. At 27 weeks, a C-section was performed for fetal distress. The infant proved impossible to ventilate and died. In literature, 16 similar cases are described of whom 14 died in the neonatal period and two in infancy. This suggests that herniation of the complete liver in isolated abdominal wall defects without a remnant membrane is lethal and counselling should be provided accordingly.

Three-dimensional ultrasound and maternal bonding, a third trimester study and a review.

OBJECTIVE: To compare the effect of third trimester three-dimensional and four-dimensional (3D/4D) versus two-dimensional (2D) ultrasound (US) of the fetal face on maternal bonding. Studies quantifying the psychological effect of 3D/4D US on mothers, pregnant of a fetus with no detectable abnormalities, were reviewed. METHODS: One hundred sixty Caucasian women attended a third trimester 3D/4D or 2D US examination. Women filled out the Maternal Antenatal Attachment Scale (MAAS) 1 to 2 weeks before (MAAS1) and 1 to 2 weeks after (MAAS2) the US examination. Visibility, recognition and attractiveness were assessed. RESULTS: Within both US groups, the MAAS2 scores were significantly higher than the MAAS1 scores (p < 0.0001). No differences in MAAS scores between the US groups emerged. Visibility and recognition were significantly positively related with the increase in MAAS scores (p = 0.003 and p = 0.042) in the 3D/4D group. Of 13 psychological studies, eight studies evaluated bonding and found no difference between 3D/4D and 2D US. The effect of 3D/4D US on satisfaction or perception showed conflicting results, and on anxiety/stress, reduction was the same as after 2D US. CONCLUSIONS: Bonding increases after either a 3D/4D or 2D US. The effect of 3D/4D US on bonding is stronger at better degrees of visibility and recognition.

Compensatory enlargement of a solitary functioning kidney during fetal development.

OBJECTIVE: To determine the degree of compensatory enlargement, and its time of onset, of the solitary functioning kidney (SFK) in fetuses with unilateral renal agenesis (URA) or unilateral multicystic kidney dysplasia (MCKD). METHODS: This was a retrospective study of fetuses with URA or MCKD diagnosed prenatally and confirmed postnatally in the period from January 1999 to May 2011. Fetuses with any other congenital anomalies were excluded. Measurements of kidney length were retrieved from our prenatal ultrasound database and a nomogram was established and compared with that for normal kidney length. RESULTS: In total, 67 fetuses were identified, 60 with MCKD and seven with URA, for which we obtained 147 kidney length measurements from our database. Mean gestational age at time of measurement was 29.7 (range, 18.4-36.7) weeks. Compensatory enlargement, defined as renal length >95(th) percentile for gestational age, was demonstrated in 87% of the MCKD cases and 100% of the URA cases (88% of cases overall). We estimated the mean enlargement for an SFK (defined as difference from 50(th) percentile for normal renal length) at 36 weeks' gestation to be 23.1% (9.25 mm), with the mean value being 11.4% (5.04 mm) greater than the 95(th) percentile for a normal kidney at this gestational age. Compensatory enlargement of SFKs was evident from the 20(th) week of gestation onwards. CONCLUSION: Compensatory enlargement of SFKs occurs in almost 90% of fetuses with MCKD or URA, and may be observed from the 20(th) week of gestation onwards.

Size and volume charts of fetal kidney, renal pelvis and adrenal gland.

OBJECTIVE: To establish reference curves for size and volume of the fetal kidney, renal pelvis and adrenal gland, as measured using ultrasound from the 15(th) week of gestation. METHODS: This was a prospective, longitudinal study of 96 fetuses in low-risk singleton pregnancies, in which we performed serial ultrasound examinations at 4-week intervals. The length and anteroposterior and transverse diameters of both kidneys, the anteroposterior and transverse diameters of the renal pelvises and the length of the adrenal glands were measured three times at each examination, with the average being used for further analysis. Reference charts were constructed using multilevel statistical analysis and comparisons were made with previously published charts derived from cross-sectional data. RESULTS: We present nomograms for fetal kidney dimensions and volume, renal pelvis dimensions and adrenal gland length. The new charts show differences in shape and have narrower percentile bands in comparison to previously published reference ranges. CONCLUSIONS: These new charts of measurements of the fetal kidney, renal pelvis and adrenal gland, from a prospective, longitudinal study, may be useful in the diagnosis and assessment of pathology of the kidney and adrenal gland.

Prenasal thickness-to-nasal bone length ratio: a strong and simple second- and third-trimester marker for trisomy 21.

OBJECTIVES: To study the ratio of prenasal thickness (PT) to nasal bone length (NBL) in normal and trisomy-21 fetuses in the second and third trimesters of pregnancy. METHODS: The PT and NBL were measured retrospectively in 106 normal fetuses (in three-dimensional (3D) volumes) and in 30 fetuses with trisomy 21 (10 on two-dimensional (2D) images and 20 in 3D volumes). RESULTS: In normal fetuses the mean PT and NBL increased between 15 and 33 weeks' gestation from 2.3 to 6.1 mm (r = 0.85, P < 0.001) and from 3.3 to 9.6 mm (r = 0.87, P < 0.001), respectively. The PT : NBL ratio was stable throughout gestation, with a mean of 0.61 (95% CI, 0.59-0.63; r = - 0.04, P = 0.7). The 5(th) and 95(th) percentiles were 0.48 and 0.80, respectively. In trisomy-21 fetuses the mean PT and NBL increased between 14 and 34 weeks from 3.0 to 9.2 mm (r = 0.86, P < 0.001) and from 1.9 to 7.8 mm (r = 0.85, P < 0.001), respectively. The PT : NBL ratio was significantly higher than in normal fetuses (P < 0.001) but also stable throughout gestation, with a mean of 1.50 (95% CI, 1.20-1.80; r = - 0.35, P = 0.07). Twenty-three (77%) of the 30 fetuses with trisomy 21 had a PT above the 95(th) percentile and 20 (67%) had an NBL below the 5(th) percentile. All the trisomy-21 fetuses had a PT : NBL ratio above the 95(th) percentile. When the 95(th) percentile of the PT : NBL ratio was used as a cut-off value the detection and false positive rates for trisomy 21 were 100 (95% CI, 89-100)% and 5 (95% CI, 2-11)%, respectively. The positive likelihood ratio was 21.2. CONCLUSIONS: The PT : NBL ratio is stable in the second and third trimesters of pregnancy in both normal and trisomy-21 fetuses, but all trisomy-21 fetuses in this series had a PT : NBL ratio above the 95(th) percentile. The ratio is therefore a strong marker for trisomy 21.

Prenatal ultrasound screening for orofacial clefts.

OBJECTIVES: To evaluate the sensitivity and specificity of ultrasound for detecting prenatal facial clefts in low-risk and high-risk populations. METHODS: This study prospectively followed up a non-selected population, namely all pregnant women who underwent routine second-trimester prenatal ultrasound screening in the Utrecht region during the 2-year period from January 2007 to December 2008. RESULTS: A total of 35 924 low-risk and 2836 high-risk pregnant women underwent ultrasound screening. Orofacial clefts were present in 62 cases, an incidence of 1:624. The distribution of clefts was as follows: 18 (29%) cleft lip, 25 (40%) cleft lip with cleft palate, 17 (27%) cleft palate only, one median cleft and one atypical cleft. Of these, 38 (61%) were unilateral and 23 (37%) were bilateral. Thirty-nine per cent (24/62) had associated anomalies, with most chromosomal defects found in the cleft lip with cleft palate and cleft palate only groups. Cleft lip with or without cleft palate was detected prenatally in 38/43 cases, a sensitivity of 88%. No case of cleft palate only was detected prenatally. There were three false-positive cases, of which two were fetuses with multiple congenital deformities. CONCLUSIONS: Ultrasound screening has a high sensitivity for the detection of cleft lip with and without cleft palate in high-risk and low-risk pregnancies in our region, where well-trained sonographers carry out primary screening. The key to a high sensitivity of prenatal ultrasound is likely to be a combination of excellent training of sonographers, referral to specialized centers when a cleft is suspected, routine visualization of the fetal face and advances in ultrasound techniques.

Grade and symmetry of normal fetal cortical development: a longitudinal two- and three-dimensional ultrasound study.

OBJECTIVES: Recent studies have shown the capability of ultrasound to demonstrate fetal cortical development. For practical application, it would be useful to have more insight into the physiological progress of cortical folding. A longitudinal study was undertaken to grade fetal cortical development and to study physiological asymmetry by means of a simple scoring system. The reproducibility of the scoring system and the differences between two-dimensional (2D) and three-dimensional (3D) ultrasound were also examined. METHODS: A cohort of 28 patients was examined by 2D and 3D ultrasound from 20 to 40 weeks of gestation. The development of selected fissures, sulci and cortical areas were graded from 0 to 5. One examination per week of gestation was randomly selected for evaluation of intraobserver and interobserver variation. RESULTS: Two-hundred and fifteen ultrasound examinations were performed in 28 patients. The development of the different cortical areas is presented. The intraobserver and interobserver agreement for assessment of grade was good. Asymmetry was common between 24 and 28 weeks, especially in female fetuses. The right parieto-occipital fissure tended to be more advanced and the left calcarine and cingulate sulci tended to be more advanced. No clinically or statistically significant differences were found between 2D and 3D ultrasound. CONCLUSIONS: Evaluation of cortical development by means of a simple scoring system is feasible and reproducible. Asymmetrical development of sulci, previously only described in vitro, has now also been demonstrated in vivo. 3D ultrasound was equally accurate and repeatable and no more time consuming than 2D ultrasound.

Role of prenatal ultrasound in predicting survival and mental and motor functioning in children with spina bifida.

OBJECTIVE: To determine which prenatal ultrasound findings can predict survival and mental and motor functioning in children with spina bifida. METHODS: Prenatal ultrasound examinations of all liveborn children who were prenatally diagnosed with spina bifida between 1997 and 2002 at the University Medical Centre, Utrecht (n = 41) were retrospectively reviewed for lesion level, head circumference, ventriculomegaly, scoliosis and talipes. These measures were correlated with postnatal anatomical (as assessed by magnetic resonance imaging (MRI)) and functional lesion levels, survival and motor and mental outcome at 5 years of age. The capacity of prenatal ultrasonography to determine lesion level was also assessed in all fetuses diagnosed with spina bifida from 2006-2007 (n = 18). RESULTS: Nineteen infants died before the age of 5 years. Multivariate regression analysis showed that higher lesion level and head circumference > or = the 90th percentile on prenatal ultrasound examination were independent predictors of demise (P < 0.05 and P = 0.01, respectively). None of the ultrasound features was a predictor of motor or mental functioning. Ultrasound predicted anatomical lesion level within one level of the postnatal findings in 50% of the first cohort and 89% of the second cohort (P < 0.01). The level of the anatomical lesion as assessed by postnatal MRI differed from the functional lesion by as many as six vertebral levels. CONCLUSIONS: Lesion level and head circumference on prenatal ultrasound are predictive of survival in children with spina bifida. No predictors were found for mental or motor function at the age of 5 years.

Fetal cerebellar volume and symmetry on 3-d ultrasound: volume measurement with multiplanar and vocal techniques.

The purpose of this prospective longitudinal study was to evaluate the growth of the fetal cerebellar volume by means of 3-D ultrasound to evaluate whether there is a difference between the volumes of the left and right cerebellar hemispheres, and to evaluate the intra- and interobserver reliability of two different techniques of volume measurement. Three-dimensional ultrasound examinations were performed every two to three weeks on 27 fetuses between 20 and 40 weeks' gestation. Measurements of the total cerebellar volume and of the left and right cerebellar hemispheres were done using the multiplanar technique. Multilevel analysis was used to determine the growth of cerebellar volume based on individual developmental trajectories and compare the volume of the right and left hemispheres of the cerebellum. The intra- and interobserver reliability was calculated for the multiplanar and VOCAL techniques in a subgroup of 10 fetuses. A nonlinear growth curve of cerebellar volume in normal pregnancy was generated. The cerebellar growth per two-week decreased from a gain of 51% of the first measurement at 20 weeks to a gain of 16% of the first measurement at 38 weeks. The left cerebellar hemisphere was significantly larger (12.3%, p < 0.01) than the right. The intraclass correlation coefficient for the measurements by the two techniques was 0.99. Intraobserver reliability: the intraclass correlation coefficient for the measurements using the multiplanar technique was 0.96 and 0.97 and for VOCAL it was 0.98 and 0.97 for the two observers, respectively. Interobserver reliability: the intraclass correlation coefficient for the measurements using the multiplanar technique was 0.97 and for VOCAL 0.98. Longitudinal growth curves based on individual developmental trajectories were generated for the cerebellar volume. The left fetal cerebellar hemisphere was found to be significantly larger than the right. Both multiplanar and VOCAL techniques had a good intra- and interobserver reliability and yielded very similar results.

The tap test--an accurate first-line test for fetal lung maturity testing.

OBJECTIVE: To determine the accuracy of near-patient and laboratory-based fetal lung maturity tests in predicting the need for neonatal ventilation. DESIGN: A prospective descriptive study. SUBJECTS: One hundred high-risk obstetric patients where confirmation of fetal lung maturity would initiate delivery. METHODS: Fetal weight estimation, placental maturity grading, and amniocentesis were performed. The investigators examined the amniotic fluid visually, and performed the tap test and shake test. Laboratory technicians estimated the lecithin-sphingomyelin (L/S) ratio, determined the presence of a phosphatidyl glycerol (PG) band on gel electrophoresis, and the optical density at 650 nm. Neonates delivered within 1 week of amniocentesis were included in the analysis. The primary end-point was the ability of the lung maturity tests to predict the need for neonatal ventilation. RESULTS: Twelve of 100 neonates required ventilation. The tap test and optical density (OD) shift at 650 nm predicted the need for neonatal ventilation with the greatest accuracy. CONCLUSION: The tap test is a rapid, easy and accurate predictor of the need for neonatal ventilation. The OD shift at 650 nm is the laboratory-based test with the greatest accuracy in our setting.

The impact of a pregnancy confirmation clinic on the commencement of antenatal care.

OBJECTIVE: To introduce a pregnancy confirmation clinic as part of antenatal care and to determine whether this would alter the gestational age at which patients commence antenatal care. SETTING: Three municipal antenatal clinics in Atteridgeville and Central Pretoria. METHOD: A pregnancy confirmation clinic was set up at three sites. At the clinic any woman wishing to confirm whether she was pregnant was offered a urine beta-HCG test. If this test was positive, on-site testing for syphilis, anaemia and rhesus status, dipstick testing of the urine, clinical examination and ultrasound examination were performed. Women with abnormal test results were commenced on appropriate treatment immediately and women requiring further medical care or investigation were referred appropriately. RESULTS: The study recruited 382 women, 145 of whom were defaulters from contraception. Half of the women (191) had a positive pregnancy test. The mean presenting gestational age was 12 weeks 4 days (standard deviation 5 weeks, range 5 weeks-25 weeks 2 days). Treatable conditions with the potential to influence pregnancy outcome were identified in 37 of the pregnant women (19.4%) Forty-three of the pregnant women intended to terminate the pregnancy. CONCLUSION: It is possible to shift the commencement of antenatal care to an earlier gestational age.

Failure of amniotic septostomy in the management of 3 subsequent cases of severe previable twin-twin transfusion syndrome.

INTRODUCTION: Amniotic septostomy has been described as a method to treat twin-twin transfusion syndrome. A case report of 3 patients treated in this way is described. CASE REPORT: Three subsequent patients, who presented with twin-twin transfusion syndrome, were treated by amniotic septostomy. All 3 showed initial improvement in the amniotic fluid volume and mobility of the donor fetus. However, all three pregnancies were lost within 5 days of the amniotic septostomy due to ruptured membranes and premature labour. CONCLUSION: In our experience, amniotic septostomy did not improve the pregnancy outcome in twin-twin transfusion syndrome. Possible reasons for this are discussed.