Survival and unique clinical practices of extremely preterm infants born at 22-23 weeks' gestation in Japan: a national survey.

OBJECTIVES: To investigate prognosis and clinical practices of infants born at 22-23 weeks' gestational age (wkGA) in Japan. DESIGN: A national institutional-level electronic questionnaire surveys performed in September 2021. SETTING: All perinatal centres across Japan. PATIENTS: Infants born at 22-23 wkGA in 2018-2020. MAIN OUTCOME MEASURES: Proportion of active resuscitation and survival at neonatal intensive care unit (NICU) discharge, and various clinical practices. RESULTS: In total, 255 of 295 NICUs (86%) responded. Among them, 145 took care of infants born at 22-23 wkGA and answered the questions regarding their outcomes and care. In most NICUs (129 of 145 (89%)), infants born at 22+0 wkGA can be actively resuscitated. In almost half of the NICUs (79 of 145 (54%)), infants born at ≥22+0 wkGA were always actively resuscitated. Among 341 and 757 infants born alive at 22 and 23 wkGA, respectively, 85% (291 of 341) and 98% (745 of 757) received active resuscitation after birth. Among infants actively resuscitated at birth, 63% (183 of 291) and 80% (594 of 745) of infants born at 22 and 23 wkGA survived, respectively. The survey revealed unique clinical management for these infants in Japan, including delivery with caul in caesarean section, cut-cord milking after clamping cord, immediate intubation at birth, hydrocortisone use for chronic lung disease, analgesia/sedation use for infants on mechanical ventilation, routine echocardiography and brain ultrasound, probiotics administration, routine glycerin enema and skin dressing to prevent pressure ulcers. CONCLUSIONS: Many 22-23 wkGA infants were actively resuscitated in Japan and had a high survival rate. Various unique clinical practices were highlighted.

Impact of the COVID-19 pandemic on surgery for benign diseases in gynecology: A nationwide survey by the Japan Society of Obstetrics and Gynecology.

AIM: This study aimed to clarify the impact of coronavirus disease 2019 on gynecology practice in Japan, in particular, on surgeries for benign gynecological diseases. METHODS: An online questionnaire was distributed to 966 facilities in Japan, including core facilities, facilities participating in perinatal and gynecologic oncology registries, and facilities certified for training by the Japanese Society of Obstetrics and Gynecology Endoscopy. The number of surgeries performed was compared between 2019 and 2020, when the novel coronavirus disease was prevalent. RESULTS: Five hundred and eighty (58.2%) facilities responded. The total number of surgeries decreased from 129 648 in 2019 to 118 565 in 2020, by 8.5%, for all surgical procedures. However, there was a clear increase in the number of robotic surgeries performed in 2020 compared to that in 2019 for all populations. The number of total hysterectomies decreased markedly from 15 385 in 2019 to 12 531 in 2020, a fall of 10.1%. CONCLUSIONS: The number of surgeries for benign gynecological diseases decreased by 8.5% in 2020 compared to that in 2019. This value is among the lowest in the world.

Measurement of fetal automated fractional shortening using two-dimensional tracking in multiple centers.

PURPOSE: To establish a normal reference range for automated fractional shortening (Auto FS) in normal singleton fetuses measured at multiple centers. METHODS: This study was conducted from May 2017 to March 2019. It was undertaken on normal singleton fetuses. First, a four-chamber view of the fetal heart was recorded in the B-mode. Then, the region of interest was set on the edge of the ventricular septum and on the edge of the ventricular muscle at a point one-third away from the atrioventricular valve and toward the cardiac apex. Tracking was automatically performed. Values measured in the right ventricle were defined as R-Auto FS, and in the left ventricle as L-Auto FS. Furthermore, combined-Auto FS was defined as the measurement across both ventricles. RESULTS: A total of 442 normal fetuses were assessed. R-Auto FS decreased significantly with gestational age, and L-Auto FS showed a similar tendency (Spearman's correlation analysis: rs = - 0.415 and rs = - 0.252, respectively). Combined-Auto FS showed a similar decline as the gestational age increased (rs = - 0.451). CONCLUSION: In this study, we succeeded in defining a reference Auto FS value not only at one institution but also multiple centers. This study suggests that Auto FS can be used clinically and effectively.

Poor uterine contractility and postpartum hemorrhage among low-risk women: A case-control study of a large-scale database from Japan.

OBJECTIVE: To examine the association between the risk of postpartum hemorrhage (PPH) and poor uterine contractility, which is suggested by the characteristics of labor. METHODS: This case-control study used cases recorded in the Japan Perinatal Registry database during the period 2013-2016. After exclusion of women with specified known risk factors for PPH, we enrolled 174 082 primiparas who had a full-term live singleton vaginal birth. Participants were classified into four classes according to the diagnosis of abnormal labor patterns and use of uterotonics. χ2 tests were used to compare PPH cases with controls, and odds ratios (OR) were calculated by univariate and multivariate analyses. RESULTS: Among the enrolled women, 10 508 (6.0%) had PPH. Abnormal labor patterns were significantly associated with an increased risk of PPH. Compared with women without any abnormal labor patterns who had not used uterotonics, women with abnormal labor patterns were at a significantly increased risk for PPH regardless of whether they had used uterotonics (adjusted OR 1.23, 95% confidence interval [CI] 1.10-1.37) or not (adjusted OR 1.30, 95% CI 1.23-1.37). CONCLUSION: Our study suggests that among low-risk women with PPH, poor uterine contractility in labor could be a significant predisposing risk factor for PPH.

Ultrasound measurement of fetal arterial pulse pressure using phased-tracking methods: A phantom study and clinical experience with antenatal corticosteroid therapy.

AIM: This study aimed to compare the accuracy of fetal pulse pressure estimated with a vascular simulator with that obtained by a manometer (reference) and evaluate the pulse pressure in normal human fetuses and fetuses whose mothers received corticosteroids. METHODS: Fetal pulse pressure was estimated as the product of blood flow velocity and pulse wave velocity, based on the water hammer equation. Ultrasonic raw radiofrequency signals for blood flow velocity were captured from the fetal descending aortas at the diaphragm level, and pulse wave velocity was simultaneously measured from different directions using the phased-tracking method. First, the precision and accuracy of pulse pressure in the estimated method were verified by a circulatory phantom simulator, which reproduced fetal blood flow using a pulsating pump. Then, the pulse pressure of 98 normal human fetuses after 17 weeks of gestation and the fetal pulse pressure in 21 mothers who received antenatal corticosteroids for fetal maturation were measured. RESULTS: A significant correlation between the estimated pulse pressure values and the actual values was found in the phantom simulation (r = 0.99, P < 0.01). The estimated pulse pressure was significantly correlated with gestational age in normal fetuses (r = 0.74, P < 0.01). In steroid-treated pregnant women, fetal pulse pressure was observed to increase significantly on the second day of administration (P < 0.01). CONCLUSION: A noninvasive and accurate estimation model of fetal pulse pressure could be established using phased-tracking method, and this method has the potential to improve the assessment of human fetal hemodynamics.

Role of obstetricians in promoting pregnancy-related knowledge among women with epilepsy in Japan.

The importance of informing women with epilepsy (WWE) of pregnancy-related issues when they are of reproductive age is well recognized. However, in Japan, education on pregnancy-related issues for these patients is insufficient. Obstetricians encounter patients who have self-discontinued their medications upon realizing they are pregnant. In this study, we aimed to assess the needs and understand knowledge levels regarding pregnancy-related issues among Japanese WWE. We contacted 400 board-certified epileptologists and asked them to administer our questionnaire to their patients; 51 responses were analyzed. Among all participating WWE, 100% were taking medication at the time of the study, 34% had given birth, and 63% expressed a desire to have children in the near future. Additionally, the median questionnaire score on pregnancy-related matters was as low as 9.5 out of 13 points. Only 31% of WWE knew that the mode of delivery is not always cesarean delivery. Among WWE, 62.7% stated that they had received counseling about pregnancy-related matters. Opinions differed regarding the optimum timing for counseling according to whether patients had received counseling. As for topics WWE wished to have explained to them, inheritance of epilepsy by their children ranked highly. The average satisfaction level with counseling was 5.6 of 7 points. Regarding satisfaction with counseling, there was a significant difference between WWE who expressed a desire to receive information from obstetricians and those who did not. In summary, we believe that our study shows that Japanese WWE remain ill-informed about pregnancy-related issues and that existing counseling must be revised. Obstetricians must play a role in preconception counseling.

Prenatal sonographic findings and prognosis of craniosynostosis diagnosed during the fetal and neonatal periods.

The aim of the study was to explore the sonographic findings of fetuses with craniosynostosis and investigate their prognosis. We conducted a 5-year, multicenter retrospective study and collected data on patients with craniosynostosis diagnosed in the perinatal period. Of 41 cases, 30 cases (73%) were syndromic craniosynostosis, eight cases (20%) were non-syndromic craniosynostosis and the other three cases (7%) were secondary craniosynostosis of chromosomal deletion syndromes. The prenatal ultrasound detection rate was 61%. Half of the cases of syndromic craniosynostosis detected during the perinatal period were Pfeiffer syndrome; there were also six cases of Apert syndrome, three cases of Crouzon syndrome and other rare form of syndromic craniosynostosis (Beare-Stevenson syndrome, Saethre-Chotzen syndrome, cranioectodermal dysplasia, and thanatophoric dysplasia). Abnormal shape of the skull was the most common finding leading to prenatal diagnosis of craniosynostosis. Abnormal head biometry, which was the second most frequent finding, was closely correlated with deformation of the cranial shape. Three cases presented with ventriculomegaly and exophthalmos but normal cranial shape and size. The overall survival rate of infants with syndromic craniosynostosis was 79%, while all of the infants with non-syndromic craniosynostosis survived. In conclusion, prenatal diagnosis of craniosynostosis is difficult, especially when dysmorphic change of the fetal cranium is not evident. Abnormal head biometry and ventriculomegaly could potentially be additional markers of fetal craniosynostosis and consequently increase the prenatal detection rate.

Reproducibility of automated fetal heart rate measurement using a novel technique with two-dimensional tracking.

PURPOSE: To assess the accuracy of automated fetal heart rate measurement using two-dimensional tracking (AutoFHR) by comparison with the conventional free-angle M-mode (M-mode) and pulsed-waved Doppler (PWD) methods. METHODS: A multicenter prospective comparative study was conducted. AutoFHR is a novel technique for the automatic calculation of fetal heart rate using a two-dimensional speckle-tracking method. The fetal heart rate (FHR) obtained by AutoFHR was compared with that obtained by the conventional M-mode and PWD. Statistical analysis was performed on the correlation between the FHR measured by AutoFHR and that determined by M-mode and PWD. RESULTS: Data from 326 singleton pregnancies were analyzed, and all the data and the data from 178 cases were compared with M-mode and PWD, respectively. The intraobserver ICC was 0.96 (95% CI: 0.93-0.98), whereas the interobserver ICC was 0.97 (95% CI: 0.95-0.99). Systematic bias was not observed between M-mode and PWD, based on the Bland-Altman plots. Analyses of the relationships among the FHRs yielded by each method revealed that AutoFHR was strongly associated with M-mode (rs = 0.99, p < 0.001) and PWD (rs = 0.86, p < 0.001). CONCLUSION: Evaluation of FHR by AutoFHR was proved to be equivalent to evaluation by the conventional M-mode and PWD. AutoFHR can be employed with only B-mode data, making FHR measurement easier and safer.

Measurement of internal diameter changes and pulse wave velocity in fetal descending aorta using the ultrasonic phased-tracking method in normal and growth-restricted fetuses.

Phased tracking (PT) is an ultrasound-based technique that enables precise measurement of a target velocity. The aims of this study were to use PT to evaluate arterial pulse waveform, pulse wave velocity and fetal pulse pressure in normal and growth-restricted fetuses. One hundred fetuses with normal development and 15 fetuses with growth restriction were analyzed. Ultrasonic raw radiofrequency signals were captured from a direction perpendicular to the vascular axis at the fetal diaphragmatic level for the difference in internal dimensions (DID), or simultaneously from different directions for the pulse wave velocity. Pulsatile movement of the proximal and distal intima of the vessels was analyzed using PT. The fetal DID exhibited no significant changes in growth-restricted fetuses. Pulse wave velocity (3.8 ± 0.32 m/s vs. 2.2 ± 0.069 m/s, p < 0.001) and estimated pulse pressure (6.9 ± 0.90 kPa vs. 2.5 ± 0.18 kPa, p < 0.001) were significantly elevated in growth-restricted fetuses. Assessment of DID and pulse wave velocity of the descending aorta using PT is a feasible, non-invasive approach to evaluation of fetal hemodynamics.

Assessing Fetal Cardiac Function by Measuring Myocardial Radial Velocity Using the Phased-Tracking Method.

OBJECTIVE: This study aimed to assess the cardiac function of healthy and pathological fetuses by measuring radial velocity using phased tracking (PT). Based on phase differences, PT allows the displacement of a specified point to be detected with improved spatial and temporal resolution. METHODS: PT was used to assess cardiac radial velocity in the basal free wall of the left and right ventricles in 134 healthy fetuses, 10 second-trimester intrauterine growth-restricted (IUGR) fetuses, and 10 recipient twins with twin-to-twin transfusion syndrome (TTTS). Maximum velocities were measured in systole and early diastole. RESULTS: Maximum radial velocity was successfully measured in 126 healthy fetuses (94%) at gestational ages of 16-40 weeks. Systolic and early diastolic maximum velocities increased with gestational age in both ventricles. As compared with controls, IUGR fetuses had significantly lower early diastolic maximum velocities in the right ventricle, and recipient twins with TTTS had significantly lower systolic and early diastolic maximum velocities in both ventricles. CONCLUSIONS: PT demonstrated right ventricular diastolic dysfunction in second-trimester IUGR fetuses as well as systolic and diastolic dysfunctions in both ventricles in recipient twins with TTTS. PT could be useful for evaluating fetal cardiac radial function.

Successful prenatal and perinatal management of a massive cervicofacial teratoma.

We report a fetus with a massive cervicofacial teratoma successfully managed in the prenatal and perinatal period. A 34-year-old woman was referred to our hospital at 16 weeks' gestation. Ultrasound examination revealed a left anterolateral fetal neck mass indicating typical findings of a teratoma. The tumor grew to tremendous size until occupying most of the uterine cavity causing severe discomfort for the patient. This necessitated ultrasound-guided removal of the intracystic fluid of the massive cervicofacial teratoma during pregnancy. Fetal MRI clearly demonstrated the mass extending from the orbit to the anterior chest wall without compression or deviation of the fetal airway. The entire mass increased to 18 cm in diameter at 37 weeks' gestation. Classical cesarean section was performed immediately after ultrasound-guided removal of the fluid from the cyst. A female infant weighing 3562 g was delivered. Resection of the tumor was accomplished on day 28 of life. The successful outcome in our case illustrates the value of accurate prenatal diagnosis of a giant cervicofacial teratoma using ultrasound and MRI, and careful management of the mother and fetus during the prenatal and perinatal period. It seems that ultrasound-guided removal of the intracystic fluid from the tumor during pregnancy should be considered to prevent preterm delivery.

Prenatal findings in a fetus with contiguous gene syndrome caused by deletion of Xp22.3 that includes locus for X-linked recessive type of chondrodysplasia punctata (CDPX1).

The X-linked recessive type of chondrodysplasia punctata (CDPX1) is a skeletal disorder that is characterized by stippled calcification at an epiphyseal nucleus and the surrounding soft tissue, short stature and an unusual face because of nasal hypoplasia. In most of the patients, this condition is noted after birth because of a characteristic face or respiratory problems. Here, we report a fetus with CDPX1. Two-dimensional ultrasound examination revealed unexplained polyhydramnios and a male fetus. Fetal biometry showed shortened long bones. Three-dimensional ultrasonography clearly demonstrated a hypoplastic nose with a depressed nasal bridge and contracture of wrists and fingers. Chromosome analysis of the amniotic fluid cells revealed the 46,Y,del(X)(p22.3) karyotype. Fluorescence in situ hybridization revealed a deletion of subtelomeric sequences at the Xpter and STS gene, but not a deletion of the KAL gene. The genomic copy number analysis demonstrated terminal deletion of 8.33 Mb that included SHOX, CSF2RA, XG, ARSE, NLGN4 and STS genes. We think that our case presents typical features of a fetus with this disorder and will be of great help in prenatal ultrasound diagnosis.

Umbilical cord cysts of allantoic and omphalomesenteric remnants with progressive umbilical cord edema: a case report.

We present a unique case of umbilical cord cysts of allantoic and omphalomesenteric remnants with progressive cord edema during pregnancy. Enlargement of the umbilical cord was observed initially at 28 weeks' gestation; the cord cysts were first recognized at 17 weeks. At 37 weeks, a cesarean section was performed and a male infant weighing 2,300 g was delivered. The entire length of the umbilical cord was 80 cm; the 50-cm proximal portion was strikingly edematous and was 7 cm in diameter. On the 1st day of life, operative exploration was undertaken via an infraumbilical incision. It was evident that a patent urachus protruded from the bladder. The lesion was excised and the bladder was closed in a two-layer anastomosis. Histologically, the intestinal epithelium was connected to the transitional epithelium at the top of the urachus, indicating allantoic remnants fused with omphalomesenteric remnants. Such a case has not been reported previously. It was considered that the overflow of urine from allantoic remnants to omphalomesenteric remnants caused the extraordinary edema of the umbilical matrix. Based on our experience, finding progressive umbilical cord edema may indicate a patent urachus co-existing with allantoic and omphalomesenteric remnants.

Fetus with prenatally diagnosed posterior mediastinal lymphangioma: characteristic ultrasound and magnetic resonance imaging findings.

Prenatal diagnosis of lymphangiomas located in the posterior mediastinum has been reported to be extremely rare. We present a fetus with a prenatally diagnosed posterior mediastinal lymphangioma found at 28 weeks' gestation. Fetal ultrasound and magnetic resonance imaging (MRI) revealed a 46 x 26 x 30 mm multicystic mass extending above the diaphragm, located in the posterior mediastinum. The mass was symmetrically shaped and surrounded the thoracic aorta. No remarkable change was noted in the size, shape and texture of the cyst by serial ultrasound and the fetus did not develop cardiac failure or hydrops in the antenatal period. Postnatal chest X-ray and MRI confirmed the prenatal findings. The infant was asymptomatic, so he was placed on close follow-up without any medical or surgical treatment.

Dichorionic twin fetuses with VACTERL association.

Although prenatal diagnosis of VACTERL (vertebral defects, anal atresia, cardiac anomaly, tracheal-esophageal fistula with esophageal atresia, renal defects, and radial limb dysplasia) association is not always possible, ultrasonography and magnetic resonance imaging can visualize some of the characteristic findings of this condition. Because infants with this condition usually require significant surgical treatment and care, prenatal detection with those imaging modalities should assist in proper planning for delivery and anticipated care of the neonate. In this report, we present dichorionic twin fetuses, both of whom were found postnatally to have this condition, and suggest the possible relationship between this disorder and intracytoplasmic sperm injection. As far as we know, this is the first report of twin fetuses affected by VACTERL association.

Identification of development and autonomic nerve activity from heart rate variability in preterm infants.

Heartbeat intervals, which are determined basically by regular excitations of the sinoatrial node, show significant fluctuation referred to as the heart rate variability (HRV). The HRV is mostly due to nerve activities through the sympathetic and parasympathetic branches of the autonomic nervous system (ANS). In recent years, it has been recognized that the HRV shows a greater complexity than ever expected, suggesting that it includes much information about ANS activities. In this study, we investigated relationship between HRV and development in preterm infants. To this end, heartbeat intervals were continuously recorded from 11 preterm infants in NICU. The recording periods were ranging from several days to weeks depending on the individuals. The HRV at various ages was then characterized by several indices. They include power spectrum as well as the mean and standard deviation of the series. For the power spectrum, the low-frequency band power (LF), high-frequency band power (HF), LF/HF (the ratio between LF and HF), beta (scaling exponent of the spectrum) were estimated. The detrended fluctuation analysis (DFA) was also employed to obtain short- and long-range scaling exponents. Each of these indices showed a correlation with the age. We showed that the long-range scaling exponent, derived from the DFA, was most significantly correlated with the age, suggesting that it could be a robust index to characterize the development of preterm infants.

Counterclockwise barber-pole sign on prenatal three-dimensional power Doppler sonography in a case of duodenal obstruction without intestinal malrotation.

Three-dimensional (3D) power Doppler sonographic imaging provides a 3D view of the blood vessels. This technique reportedly has advantages over other forms of sonography in visualizing normal and abnormal fetal vascular anatomy. We report the case of a 36-year-old pregnant woman in whom 3D power Doppler sonography with simultaneous gray-scale imaging was performed at 32 weeks' gestational age to investigate an intestinal obstruction in the fetus that was suspected on the basis of 2-dimensional sonographic findings. The 3D sonograms revealed that the superior mesenteric artery and vein of the fetus were wound counterclockwise, forming the "barber-pole" sign. At 38 weeks' menstrual age, the female infant was delivered vaginally. Only duodenal atresia and annular pancreas, but not intestinal malrotation, were found postnatally. Surgical reconstruction of the duodenum was performed, with no major complications. Previous research suggests that the counterclockwise barber-pole sign is a normal finding. On the basis of our case, we believe that this "negative finding" might be used to exclude the diagnosis of midgut volvulus, a condition that would be expected to cause these vessels to wrap around in a clockwise direction.

Prenatal demonstration of major aortopulmonary collateral arteries with tetralogy of Fallot and pulmonary atresia.

Major aortopulmonary collateral arteries were prenatally demonstrated in a case of tetralogy of Fallot and pulmonary atresia (TOF-PA). Color Doppler and pulsed Doppler ultrasonography revealed abnormal arteries arising directly from the descending aorta. An aortogram after birth confirmed two collateral arteries arising from the descending aorta and one from the left subclavian artery. Fetal echocardiography was essential for the diagnosis of TOF-PA, but further examination was needed to find collateral arteries, which provided useful information for management after birth.

Fetal atrial tachycardia diagnosed by magnetocardiography and direct fetal electrocardiography. A case report of treatment with propranolol hydrochloride.

At 26 weeks of gestation, fetal tachyarrhythmias (about 250 bpm) and ascites were detected by ultrasonography, and oral treatment with propranolol (30 mg/day) was commenced. Within 10 h, the fetal heart rate changed to approximately 85 bpm. The averaged fetal magnetocardiogram triggered by R peaks showed P wave and QRS complexes and an extra P wave. In addition, many extra nonconducted P-waves were detected in a fetal direct electrocardiogram. At 27 weeks of gestation, fetal tachycardia occurred again, and arrhythmia was diagnosed as the result of a blocked premature atrial contraction (PAC) with intermittent atrial tachycardia by fetal electrocardiogram. Administration of transplacental propranolol (90 mg/day) resolved the fetal tachyarrhythmias and ascites. Further studies are required to evaluate the efficacy and adverse effects of propranolol for fetal atrial tachycardia.

Prenatal diagnosis and antenatal history of persistent truncus arteriosus: A case report.

We report the prenatal diagnosis and the natural history of a fetus with truncus arteriosus. A 31-year-old woman, gravida 3, was referred for detailed examination for suspected cardiac anomaly during her 33rd week of gestation. Fetal echocardiography demonstrated a large truncal vessel overriding a ventricular septal defect, a right-side aortic arch, and a dilated ascending aortic arch. Pulsed-wave and color-flow Doppler imaging showed truncal valvular stenosis without insufficiency. No sign of congestive heart failure was detected during the prenatal period. A fetus weighing 2860 grams was delivered during the 38th week of gestation. Cyanosis was noted the day after delivery, however, and a Rastelli procedure was carried out 9 days after delivery. The baby died 7 days after the operation.