The International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) is a scientific organization that encourages sound clinical practice, and high-quality teaching and research related to diagnostic imaging in women’s healthcare. The ISUOG Clinical Standards Committee (CSC) has a remit to develop Practice Guidelines and Consensus Statements as educational recommendations that provide healthcare practitioners with a consensus-based approach, from experts, for diagnostic imaging. They are intended to reflect what is considered by ISUOG to be the best practice at the time at which they are issued. Although ISUOG has made every effort to ensure that Guidelines are accurate when issued, neither the Society nor any of its employees or members accepts any liability for the consequences of any inaccurate or misleading data, opinions or statements issued by the CSC. The ISUOG CSC documents are not intended to establish a legal standard of care, because interpretation of the evidence that underpins the Guidelines may be influenced by individual circumstances, local protocol and available resources. Approved Guidelines can be distributed freely with the permission of ISUOG (info@isuog.org).

ISUOG Practice Guidelines (updated): sonographic examination of the fetal central nervous system. Part 1: performance of screening examination and indications for targeted neurosonography

Aim The purpose of this paper is to review and analyze the published literature on the use of three-dimensional (3DUS) and four-dimensional (4DUS) ultrasound in perinatal medicine. Methods We systematically searched Medline through PubMED (January 2000-January 2006), including EMBASE/Excerpta Medica database as well as the Cochrane Database of Systematic Reviews. The search terms used to identify clinical application of 3DUS and 4DUS studies in perinatal medicine were technical development, special features, and recommendation for fetal imaging, research on 3DUS or 4DUS, and the usage of invasive 3DUS or 4DUS procedures. The reference bibliographies of relevant books were also manually searched for supplementary citations. Inclusion criteria were as follows: (1) studies related to the use of 3DUS or 4DUS in perinatal medicine; (2) full text were available in English; (3) publication format of original scientific articles, case reports, editorials or literature reviews and chapters in the books. Results Five hundred and seventy-five articles were identified, and among those, 438 were relevant to this review. Conclusions 3DUS and 4DUS provided additional information for the diagnosis of facial anomalies, evaluation of neural tube defects, and skeletal malformations. Additional research is needed to determine the clinical utility of 3DUS and 4DUS for the diagnosis of congenital heart disease, central nervous system (CNS) anomalies and detection of fetal neurodevelopmental impairment assessed by abnormal behavior in high-risk fetuses.

How useful is 3D and 4D ultrasound in perinatal medicine

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 4week 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 crosssectional 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. Copyright  2012 ISUOG. Published by John Wiley & Sons, Ltd.

https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.11169

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

OBJECTIVE The purpose of this study was to evaluate associated anomalies and outcomes of fetuses with prenatally diagnosed hemivertebrae. METHODS Fetuses with prenatally diagnosed hemivertebrae, excluding those associated with spina bifida, were identified by searching the prospectively maintained ultrasound databases of 4 institutions from 1997 to August 2007. Associated birth defects were tabulated by organ system and hemivertebra location. Outcomes included karyotypes, gestational ages, and routes and outcomes of deliveries. RESULTS Nineteen fetuses had a diagnosis of hemivertebrae at a mean gestational age +/- SD of 20.5 +/- 5.4 weeks. Fourteen (73.7%) fetuses had additional anomalies, of which 5 (35.7%) were syndromic (4 with cloacal exstrophy and omphaloceles and 1 with Jarcho-Levin syndrome). Karyotypes were normal in all 11 available cases, each of which had additional anomalies. Fourteen (73.7%) neonates were live born at a mean gestational age of 34.9 +/- 4.3 weeks, of which 7 (50%) were born by cesarean delivery. Ten neonates (71.4%) were delivered before term, and 4 (28.6%) were growth restricted (<10th percentile). Two (14.3%) of these neonates died; both had cloacal exstrophy and large omphaloceles. The remaining pregnancies were terminated (4 [21.1%]) or had a fetal death (1 [5.3%]). CONCLUSIONS Most fetuses with prenatally diagnosed hemivertebrae have additional anomalies, often syndromic, which affect the prognosis. Affected pregnancies have high rates of cesarean delivery and growth restriction. Neonates with nonisolated hemivertebrae are more often delivered before term and have higher mortality rates.

Prenatal Sonographic Diagnosis of Hemivertebrae Associations and Outcomes

Brain hemispheres are connected by commissural structures, which consist of white matter fiber tracts that spread excitatory stimuli to various regions of the cortex. This allows an interaction between the two cerebral halves. The largest commissure is the corpus callosum (CC) which is located inferior to the longitudinal fissure, serving as its lower border. Sometimes this structure is not completely developed, which results in the condition known as agenesis of the corpus callosum (ACC). The aim of this paper was to review the latest discoveries related to the genetic and metabolic background of ACC, including the genotype/phenotype correlations as well as the clinical and imaging symptomatology. Due to various factors, including genetic defects and metabolic diseases, the development of CC may be impaired in many ways, which results in complete or partial ACC. This creates several clinical implications, depending on the specificity of the malformation and other defects in patients. Epilepsy, motor impairment and intellectual disability are the most prevalent. However, an asymptomatic course of the disease is even more common. ACC presents with characteristic images on ultrasound and magnetic resonance imaging (MRI).

Corpus Callosum Agenesis: An Insight into the Etiology and Spectrum of Symptoms.

Objective
While complete agenesis of the corpus callosum is often suspected on fetal ultrasound due to absence of the cavum septi pellucidi (CSP), suspicion of partial agenesis of the corpus callosum (pACC) is a challenge since the CSP is almost always present. The aim of this study was to measure the length and width of the CSP and calculate the length-to-width ratio (CSP ratio), and compare these between fetuses with pACC and normal fetuses.

Methods
In this retrospective case–control study, the length and width of the CSP were measured in the axial plane of the fetal head, and the CSP length-to-width ratio calculated, in 323 normal fetuses and in 20 fetuses with pACC between 20 and 34 weeks' gestation. From the normal population we constructed reference ranges in relation to biparietal diameter (BPD). For all fetuses we calculated Z-scores for the CSP ratio.

Results
In the normal population, the length and width of the CSP increased with increasing BPD, while the CSP ratio decreased. The CSP was short ( 95th centile) in 65% (13/20). The CSP ratio was small (< 5th centile) in 95% (19/20) of pACC fetuses, with 16/20 (80%) having a ratio below an empirical cut-off of 1.5. Analysis of Z-scores showed that fetuses with pACC had a significantly smaller CSP ratio (P < 0.0001) compared with the normal population.

Conclusions
Fetuses with a normal-sized corpus callosum have a rectangular-shaped CSP, with a CSP ratio > 1.5 in the second half of gestation. Most fetuses with pACC have an abnormally shaped, wide and short CSP, with a decreased CSP ratio. This simple ratio has the potential to identify fetuses at high risk for pACC. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Cavum septi pellucidi (CSP) ratio: a marker for partial agenesis of the fetal corpus callosum.

Application of the three-dimensional maximum mode in prenatal diagnosis of Apert syndrome.

OBJECTIVES: The purpose of this study was to evaluate the potential of 3-dimensional ultrasonographic planar and nonplanar reslicing techniques. METHODS: Fetuses with severe brain anomalies diagnosed by means of 2-dimensional ultrasonography were prospectively included in the study. Good-quality 3-dimensional volumes of the fetal head were obtained in each case. Subsequently, these volumes were reviewed with use of 3-dimensional extended imaging with Oblique View and DynamicMR (Medison Co, Ltd, Seoul, Korea). RESULTS: Eight fetuses (mean gestational age, 23 weeks; range, 20-30 weeks) with the following central nervous system anomalies were examined: semilobar holoprosencephaly, absent cavum septum pellucidum, porencephaly in twin-to-twin transfusion syndrome, partial agenesis of the corpus callosum, Dandy-Walker variant, open-lip schizencephaly, aneurysm of the vein of Galen, and dilated cavum vergae. CONCLUSIONS: Planar and nonplanar reslicing of the volumes delivered informative images in any reconstructed plane. One important prerequisite, however, was the absence of acoustic shadowing during data acquisition.

Three-dimensional ultrasonographic reslicing of the fetal brain to assist prenatal diagnosis of central nervous system anomalies.

Uhl’s anomaly is only one of many causes of right ventricular dilatation, including Ebstein’s disease, partial anomalous venous return, absence of the pericardium, pulmonary atresia, and aortic coarctation or aortic interruption. A correct prenatal diagnosis can be useful for selecting patients who need more attention, especially in the first months of postnatal life, to improve ventricular function, reduce complications and increase the chances of survival.

https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.2785

Three-dimensional ultrasonographic demonstration of agenesis of the 12th rib in a fetus with trisomy 21.

We report the prenatal findings of congenital adrenal hyperplasia (CAH) in two consecutive fetuses of one family. The first pregnancy was terminated at 23 weeks' gestation due to the presence of a complex heart anomaly. The adrenal glands appeared enlarged on prenatal ultrasound examination and autopsy confirmed CAH. The parents were subsequently examined and were found to be heterozygous for nucleotide 656 of the CYP21B gene. In a subsequent pregnancy, chorionic villus sampling at 11 weeks confirmed CAH in the male fetus. At this gestational age, mild body edema was present and the nuchal translucency measured 2.1 mm. From 14 weeks onwards, enlargement of the adrenal glands was the only sign of CAH. These findings suggest that enlarged adrenal glands may be a prenatal sign for CAH. In fetal medicine, when a pregnancy is terminated due to fetal malformations, autopsy should be performed because it can provide additional information that is helpful in counseling women with regard to subsequent pregnancies.

https://obgyn.onlinelibrary.wiley.com/doi/pdf/10.1002/uog.994

Enlarged adrenal glands as a prenatal marker of congenital adrenal hyperplasia: a report of two cases.

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