Malrotation is caused by abnormal intestinal tract rotation and abnormal mesentery fixation when the intestinal tract fits into the abdominal cavity during the fetal period. It often develops in the neonatal period as bilious vomiting. If midgut volvulus occurs and extensive intestinal necrosis occurs, large-scale small bowel resection may lead to short bowel syndrome. Accurate diagnosis and appropriate treatment are required, as some cases are asymptomatic and discovered incidentally, and others are observed over a long period to develop chronic symptoms.

 In the general article, we will explain the occurrence of malrotation. This disease classification has been reported so far, with symptoms that differ depending on age and imaging findings that are central to diagnosis, surgical treatment, and complications. In line with the content covered in the clinical practice guidelines published by the Japanese Society of Pediatric Surgery, we will discuss the superiority of imaging tests in terms of diagnostic yield, indications for surgery in asymptomatic cases, and surgery in cases without midgut volvulus. We will provide an overview of intestinal fixation, prophylactic appendectomy, adhesion prevention procedures, laparoscopic surgery, and second look operations, which differ in policy depending on the timing and facility.

 We retrospectively investigated newborns and infants who were treated at our hospital under the diagnosis of ovarian cyst. We divided them into two groups, a spontaneous regression group and a surgery group, and examined the details. We excluded fetal treatment cases. The maximum size of the ovarian cyst at fetal period was 3.3±1.1 cm in the spontaneous regression group and 5.0±1.3 cm in the surgery group and at birth was 1.6± 1.2 cm and 5.2 ± 1.6 cm, in both cases, which was significantly larger in the surgery group（p <0.01）. The rate of change in cyst diameter from the time when the cyst diameter was the largest to after birth was -42±36％ in the spontaneous regression group and + 5.1±27％ in the surgery group, and the spontaneous regression group tended to decrease significantly（p <0.01）. The number of weeks of gestation at the time of the largest fetal cyst diameter was 32±3.2 weeks in the spontaneous regression group and 37 ± 2.1 weeks in the surgery group, which was significantly earlier in the spontaneous regression group（p<0.01）. Similar results were confirmed when comparing cases that had twisted at the time of surgery. From these results, in addition to the cyst diameter itself, the cases which the cyst diameter peaks in the fetal period after 38 weeks and in cases which the rate of change until birth is greater than -5％, it should be considered that the cyst has already torsioned or is likely to torsion.

 ［Objective］Ulcerative colitis（UC）has been reported to increase the risk of adverse pregnancy outcomes, such as cesarean section（CS）, preterm delivery, low birth weight（LBW）, and congenital malformations; however, only a few studies have examined Japanese pregnant women with UC with total proctocolectomy（TP）. The aim of this study is to clarify pregnancy outcomes among pregnant women with UC with TP compared with those without TP at our department.

 ［Methods］We retrospectively investigated the data of pregnant women treated at our department between 1999 and 2018. We compared outcomes between patients with TP（TP group）and without TP（non-TP group）. In addition, we compared these outcomes with the control group without medical complications including UC, which was extracted by propensity score matching based on maternal age and pregnancy history.

 ［Results］We analyzed 23 pregnancies of 15 women with UC, including seven pregnancies of four women with TP（TP group）and 16 pregnancies of 11 women without TP（non-TP group）. Preterm delivery occurred in one pregnancy（14％）in the TP group and in two（13%）in the non-TP group. CS was performed in three pregnancies（43%）in the TP group and two（13%）in the non-TP group. Perinatal deaths occurred in two pregnancies（13%）from the non-TP group, also one pregnancy（0.9%）from the control group（115 pregnancies）. Statistical differences were not observed for all outcomes between the TP and non-TP groups.

 ［Conclusion］This study did not report an increased risk of adverse pregnancy outcomes in pregnancies with TP history compared to pregnancies of UC patients without TP.

 This study aimed to investigate the trends of direct bilirubin（DB）values in very low birth weight infants（VLBWI）and identify perinatal factors associated with the development of direct hyperbilirubinemia（peak DB ≥ 2.0 mg/dL）.

 The study enrolled 206 VLBWI（29.4 weeks, 1, 066 g）born and managed in our NICU between January 2017 and May 2021. We routinely measured serum direct bilirubin（SDB）value in each patient every two weeks, starting from day 14, and determined the chronological and postconceptional age at which peak SDB was achieved. Additionally, we investigated perinatal factors contributing to the development of direct hyperbilirubinemia.

 The mean chronological age of peak SDB was 42±27 days, displaying multimodal distribution characteristics. In contrast, the mean postconceptional age of peak SDB was at 35.3±4.0 weeks with unimodal distribution. Further, logistic regression analysis revealed that birth weight z score（p=0.001; odds ratio［OR］0.345）and age at full enteral nutrition establishment（feeding volume ≥100 ml/kg/day; p=0.012; OR 1.663）were significant perinatal factors influencing direct hyperbilirubinemia.

 Our findings indicated that the excretion of DB was influenced by the term period, and a higher degree of fetal growth retardation and delayed attainment of enteral nutrition may be risk factors for subsequent direct hyperbilirubinemia in VLBWIs.

 In recent years, there has been an emphasis on families in neonatal medicine focusing on Family-Centered Care. However, it hasn’t been uncleared how families accept the experience in NICU. As such, this author: a mother of premature infants, attempted to participate in online peer support groups for families with premature infants as a non-participant observer. This practice aims to extract the meaning of the emotions of the families and the themes they discussed from psychological perspective. Consequently, we could see various minds classified in negative and positive aspects, and some of them are expressed in non-verbal ways. In terms of functions of self-help / mutual help / public assistance provided by governments and medical care, peer support groups for families work as places for mutual help to exchange information which is not sufficient from self-help efforts. On the other hand, from the aspect of Psychology, we revealed the limits of peer support groups for families as mutual help, as shown in the ways of families control their real emotions and use a defense mechanism themselves. Therefore, we revealed that it is important to be considerate of the emotions of families and support their recovery.

 Objective: The aim of this study is to investigate the relationship between the time interval from onset of respiratory distress after birth to decision to refer neonate for transport, and the duration of mechanical ventilation after neonatal transport.

 Methods: This retrospective study analyzed cases of neonatal transport due to respiratory distress from local obstetrical clinics to St. Mary’s Hospital’s neonatal intensive care unit between January 2020 and February 2022. Patients who received respiratory support for less than three days were classified as the short-term respiratory support group, while those who received respiratory support for three days or more were classified as the long-term respiratory support group.

 Results: A total of 89 patients were admitted, consisting of 67 patients in the short-term respiratory support group and 22 patients in the long-term respiratory support group. The median time from onset of respiratory distress to decision to refer patient for neonatal transport was significantly longer in the long-term respiratory support group （9 hours 27 minutes）than in the short-term respiratory support group（1 hour 59 minutes） （p = 0.007）. There were 29 cases where time from onset to referral decision was equal to or greater than 8 hours. Among these 29 cases, the majority of referral decisions（24 cases or 82.8%）were made during the day shift.

 Conclusion: The group of neonates requiring long-term respiratory support had more cases with a longer time to referral decision. Moreover, it was suggested that the longer times until referral were related to the work shift.

 Aim: This study aimed to assess the effectiveness of a new management protocol that we devised for threatened preterm labor, called the "modified short-term tocolysis."

 Methods: This was a single-center, retrospective, observational study conducted between April 2017 and March 2022. Patients were distributed into long-term and modified short-term tocolysis protocol groups and compared. The primary endpoint was the frequency of preterm delivery before 34 weeks of gestation. Secondary endpoints were perinatal and neonatal outcomes.

 Results: There were 61 and 35 patients in the long-term and modified short-term tocolysis protocol groups, respectively. Regarding maternal background, only the primipara rate was higher in the modified short-term group. There were no differences between the two protocols in terms of the frequency of preterm births before 34 weeks of gestation, adverse effects and neonatal intensive care unit admission rates. The duration of intravenous ritodrine hydrochloride and number of ampules were significantly shortened or decreased, respectively, in the modified short-term tocolysis protocol. These results were similar even after adjusting for differences in the primipara rate.

 Conclusions: The frequency of preterm delivery before 34 weeks of gestation did not differ between the two protocols. Thus, it may be useful to use modified short-term tocolysis.

 In the context of the increasing fertility treatments and consolidating of medical resources, the number of multiple pregnancies delivered at perinatal centers is recently increasing. However, few reports focused on the rate of multiple births delivered at perinatal centers. In this study conducted at a tertiary perinatal center, Hyogo Prefectural Kobe Children’s Hospital Perinatal Center, 9,559 newborns who delivered between 1995 and 2020, and their mothers were investigated. During the study period, the rate of mothers with more than 35 years of life increased from 10.4 to 34.2%. Concurrently, the rate of pregnancy with fertility treatments, including timing of intercourse, artificial insemination with husband, ovulation induction, and assisted reproductive technologies, also increased from 2.3 to 26.3%. As a result, the rates of multiple pregnancies and births were increased from 7.7 and 15.3% to 22.3 and 37.2%, respectively. At our tertiary perinatal center, multiple pregnancies and births are significantly increasing during recent decades.

 When neonatal transport to our center is required on weekdays during daytime, NICU doctors and nurses ride together in the neonatal transport doctor car. At nighttime and on holidays and weekends, staff from the maternity clinic use fire department ambulances for transport. We compared 238 cases that were transported and admitted to the NICU between January 2018 and December 2021. Cases were compared in terms of the time from birth to request, chief complaint, perinatal information, and treatment in the NICU, according to whether the transport occurred on a weekday during daytime, or at nighttime or on a weekend or holiday. Regardless of the time of request, among neonatal transports up to 12 hours of age, approximately 80% of the chief complaints were asphyxia and respiratory failure. Moreover, despite the severity of the illness, more cases were admitted at nighttime and on weekends and holidays than on weekdays during daytime. Therefore, many cases could not be transported by the neonatal transport doctor car. Neonatal transport by doctor car has the benefits of enabling an early start of treatment and safe transport of newborns. There is an urgent need to establish a system that allows newborns to be transported 24 hours a day by doctor cars.

 Obese pregnant women are at high risk for maternal and infant complications and consideration of prenatal care and delivery method is important. In this study, we retrospectively compared obese and severely obese groups to clarify the characteristics of prenatal management in obese pregnant women. We studied 164 women who had delivery from 2017 to 2020, classifying pregnant women with BMI 35 or above as severe obese and BMI between 30 and 35 as obese at 30th week pregnancy. The severely obese group had significantly higher risk of having hypertension and HDP and socio-economic problems compared to the obese group. There was no significant difference between the severe obese group and the obese group in the outcome of vaginal delivery cases. For cesarean sections, induction time of anesthesia, operation time were significantly longer in the severe obese group, and general anesthesia and sedentary regional anesthesia were significantly more common in the severely obese group. There was no significant difference in the infant outcome and the reason of NICU hospitalization. Comparing the severe obese group with the obese group, we see higher risk with higher BMI, suggesting that the management of pregnancy and delivery in obese women should take into account not only obstetric but also social, medical, and anesthetic risks.

 Objectives: To examine the current status and complications of trial of labor after cesarean delivery（TOLAC）in Aomori Prefecture and to review the criteria for TOLAC.

 Methods: Pregnant women who delivered singleton babies at two of the five perinatal maternity centers in Aomori Prefecture that offer TOLAC were included in the study. Their decision to go through with TOLAC, the final delivery method, maternal adverse events during vaginal birth after cesarean delivery（VBAC）, and the prognosis of the child were determined. Furthermore, information on the mothers and babies was compared between the VBAC and cesarean groups.

 Results: Of 441 expectant mothers, 159（36.1%）requested TOLAC, with the process being successful for 87 of them（TOLAC success rate: 92.6%）. The number of weeks of labor and the birth weights of the newborn were significantly higher in the VBAC group. Furthermore, there were no neonatal deaths or babies requiring multidisciplinary care for issues that could have been related to TOLAC.

 Discussion: This study was conducted because repeated cesarean sections increase the risk of not only placenta previa and placenta accreta, but also hysterectomy, bowel and bladder injury, and maternal death. The two centers in this analysis that performed TOLAC had a high success rate and intend to continue this practice in the future.

 Objective: To investigate the effect of cervical pessaries on uterine cervical length in women diagnosed with preterm labor with short cervix（≦ 25 mm at <30 weeks of gestation）.

 Methods: This is a retrospective observational study. From November 2018 to January 2022, we compared cervical length measured by transvaginal ultrasonography at every 1 week after the start of treatment between 2 groups of singleton fetuses without bulging fetal membranes who started treatment at <30 weeks of gestation:（1） inpatient pessary group whose members received at least 1 day of inpatient management during pessary use and （2）control group managed with bed rest without pessary use. Hospitalization or outpatient management was at the attending physician’s discretion. The Mann-Whitney test was used for statistic analysis, and P<0.05 was considered significant.

 Results: There were 9 women treated with pessary, 6 in the inpatient pessary group and there were 31 in the control group. Cervical length before treatment was not different between the pessary（16.5 mm of median）and the control group（14 mm）. Cervical length in the pessary group was significantly（P<0.05）longer after treatment than that in the control group at 2（26 vs 16 mm）, 3（23 vs 14.5 mm）, and 4 weeks（23.5 vs 15 mm）after the start of treatment.

 Conclusion: Pessary may have a temporary effect of increasing cervical length for pregnant women with short cervix, although the effect on the preterm delivery rate must be studied further.

 The aim of this study was to clarify the current status and need for simulation-based medical education（SBME）in neonatal medicine in Japan. A questionnaire survey of educational staff at neonatal care facilities accredited by the Japan Society of Perinatal and Neonatal Medicine was conducted to gather the opinions of instructors. In addition, a web-based questionnaire survey of ≤10-year post-graduate physicians engaged in neonatal care was conducted to collect the views and experiences of learners. Valid answers were received from 206 facilities（44% response rate）and 101 individuals. Most instructors（95%）and learners（84%）agreed that, in general, SBME was very or somewhat beneficial for training young neonatal care professionals. However, our research also revealed that the existing SBME in Japan was limited to a few topics such as tracheal intubation, artificial respiration, chest compressions, and resuscitation（NCPR®）. The lack of simulators─either because facilities do not own them or they are not available in the market─and the inability of facilities to raise costs, including time, manpower, and funds, were mentioned as factors that prevented the implementation of SBME on various topics. Therefore, not only are SBME programs on various topics warranted but also a sustainable implementation scheme must be established to enhance the training of young neonatal care professionals and improve the quality of neonatal care in Japan.

 Objective: In 2019, we designed the neonatal intensive care unit（NICU）and pediatric intensive care unit（PICU）to be adjacent to each other during renovations. This study is a retrospective observational analysis that compares management patterns, morbidity, and mortality among infants undergone cardiac surgeries before and after the renovation.

 Methods: We conducted a retrospective review of the medical records of neonates admitted to the University of Tokyo Hospital NICU who underwent cardiac surgery and received treatment in the PICU during hospitalization. Data were collected on infants born in two different time periods: “Before”（2017 and 2018）and “After”（2020 and 2021）. The “Before” group comprised patients managed in the previous ward, while the “After” group included patients managed in the new ward. Maternal and neonatal characteristics were compared between the groups.

 Results: Most of the maternal and neonatal backgrounds were similar between the groups, and in-hospital mortality did not differ significantly between the two groups（p = 0.73）. Patients in the “After” group had non-cardiac anomalies more frequently compared with the “Before” group（p = 0.006）. Furthermore, there was a significantly higher rate of patient exchange between the NICU and PICU in the “After” group（p < 0.001）. The length of hospital stay was also longer in the “After” group（p <0.001）.

 Discussion: Safe transfer between the NICU and PICU was advantageous to the management of infants with complications, but resulted in a longer stay in the NICU.

 Conclusion: Management pattern can be influenced by ward arrangement in infants underwent cardiac surgeries.

 ［Objective］To clarify the important points in the perinatal management of pregnant cases after repair surgery of complete transposition of the great arteries（TGA）. ［Material and Methods］We review the obstetric and cardiac outcomes of pregnancies after TGA repair those who were managed at our hospital between 2019 and 2022. We used the medical records retrospectively ［Results］There were 4cases and 5pregnancies. The TGA repair procedures were atrial repositioning in 2 cases, arterial repositioning in 1 case, and Rastelli’s surgery in 1 case 2 pregnancies. There were 1 pregnancy with NYHA class II and 4 with class I before pregnancy, and 3 pregnancies required medication before pregnancy. Four pregnancies were delivered at term, and one with NYHA II delivered preterm at 34 weeks of gestation due to maternal arrhythmia. Perinatal complications included 2 pregnancies with 1, 800ml intrapartum bleeding（same case）and 1 pregnancies with SGA of -1.5SD. Patients with right ventricle as systemic ventricle required hospitalization for management during the second trimester of pregnancy and received postpartum multidisciplinary treatment, while those with left ventricle as systemic ventricle preserved cardiac function. ［Conclusion］Perinatal cardiac function and complications differed depending on the postoperative systemic ventricles. Multidisciplinary team is important for the care of pregnancy with repaired TGA since before pregnancy to postpartum, more careful management is required especially in cases right ventricle as systemic ventricle.

 Although listeriosis during pregnancy is rare（3 in 100,0000）, it is a serious disease that can cause fetal demise or preterm birth. In 19 years, there were 5 cases of listeriosis during pregnancy at our hospital（20 in 100,0000）. During outbreaks of COVID-19, 2020 - 2022, listeriosis during pregnancy were recognized every year. Two of the cases resulted in intrauterine fetal death（at 20 and 23 weeks of gestation）and three were born by emergency cesarean section due to non-reassuring fetal status（at 29, 33 and 39 weeks of gestation）. No abnormal long-term prognosis has been observed at present among those three. The chief complaints were fever and abdominal pain. Diagnosis confirmed by detection of listeria on bacterial culture. Antibiotics including ampicillin were administered after the results of bacterial culture were obtained, and the mothers recovered in all cases. A probable causative food might be raw milk in one case. However, it was difficult for others to identify the cause. It is important to educate women about diet during pregnancy and to remind them to seek immediate medical attention if they have symptoms such as fever or abdominal pain.

 Thanatophoric dysplasia is a severe skeletal dysplasia. Recent advancements in neonatal respiratory management have resulted in long-term survival of individuals with thanatophoric dysplasia. It is now recognized that this disease is not always fatal. However, there are currently no standardized guidelines or statements for the management of neonates with this disorder, and each medical institution manages the patients on a case-by-case basis. In this paper, we present a case of thanatophoric dysplasia in our hospital who was managed by multidisciplinary medical staffs during the perinatal and postnatal periods. Extensive treatment was carried out immediately after birth, and the patient survived for 5 months. The extensive review of the previous literatures on the perinatal management of thanatophoric dysplasia in Japan revealed that patients contributed by genetic specialists received relatively extensive treatments after birth. In addition, patients who were intensively treated survived for at least three months. This report may help us understand the management of this rare genetic disorder with nearly lethal manifestations in Japan.

 Agnathia-otocephaly complex has been regarded as a lethal condition, although less severe and non-lethal variations of the isolated type have been documented. Ensuring airway patency during the acute phase of this condition is paramount, and some cases with indications for Ex-Utero Intrapartum Treatment（EXIT）have been previously reported. In this report, a 37-year-old primiparous woman was referred to our hospital at 27 weeks of gestation and was diagnosed with an isolated type of agnathia-otocephaly with no other abnormalities. Magnetic resonance imaging and microbubble tests revealed delayed fetal lung maturation but not entirely immature. With the patient’s consent, an emergency cesarean section with EXIT was performed owing to clinical chorioamnionitis at 35 weeks of gestation; however, the neonate died 12 hours after delivery because of hypoplastic lungs. The methods for evaluating lung maturation are inconsistent; thus, the indication for invasive EXIT procedure warrants careful consideration, and the procedure necessitated robust teamwork. As our hospital only has few physicians experienced in performing EXIT, the present case marked our inaugural execution of the procedure. Consequently, we accomplished the prenatal diagnosis and execution of the EXIT procedure through coordinated medical care by multidisciplinary and interinstitutional teams.

 Mitochondrial disease is a disorder of mitochondrial function that presents with a variety of clinical symptoms. In the present case, mitochondrial disease was a maternal complication, and MELAS was strongly suspected. Magnesium sulfate was administered at 23 weeks’ gestation for threatened preterm labor. At 29 weeks’ gestation, uterine contractions with pain were observed, leading to administrations of betamethasone and β2 stimulator. However, increasing abdominal pain and prolonged bradycardia（lowest pulse: 80 bpm）were subsequently noted, and an emergent cesarean section was carried out. The patient delivered a female, with a birth weight of 1,452 g, Apgar scores of 3/6 points, and umbilical artery blood pH of 6.873. There were no intraoperative findings that could have caused fetal dysfunction, and postoperative blood tests of the mother showed a high lactic acid level, hyperglycemia, and acidosis. The patient was subsequently diagnosed with MELAS. It is possible that diabetic ketoacidosis was concurrent with lactic acidosis due to MELAS. In pregnancies complicated with MELAS, physicians should be aware of the possible sudden onset of acidosis.

 A majority of neonates with gastroschisis have an uncomplicated course and good postoperative recovery. However, the outcomes of complex gastroschisis, which involves other intestinal anomalies such as atresia, necrosis, perforation, or volvulus, remain poor. A 26-year-old woman was referred to our institute at 18 weeks of gestation due to the management of fetal gastroschisis. At 28 weeks of gestation, ultrasonography revealed partial intestinal dilatation and wall thickening. At 30 weeks of gestation, the fetal intestines had the appearance of a mass with increased amniotic fluid echo density. A male neonate was subsequently delivered at 32 weeks of gestation following preterm membrane rupture. The birth weight was 1,824 g and Apgar scores were 7（1 min） and 8（5 min）. A clumped intestine was visible outside the anterior abdominal wall to the right of the umbilicus. The mass had severe bowel matting and was covered by a thick peel. The intestine had multiple atresia, and the necrotic small intestines were removed. At 1 year and 2 months, the infant had a body weight of 2.4 kg and could not tolerate enteral feeding due to development of short bowel syndrome. Although no management strategy has yet been established for fetuses with complex gastroschisis, the presence of a clumped intestine on fetal ultrasonography could be indicative of severe intestinal injury in fetuses with gastroschisis. This ultrasound finding may serve as a valuable aid in making the decision to terminate the pregnancy, considering both gestational age and the well-being of the fetus.

 We experienced a case of pregnancy with venous thromboembolism, which worsened in a short period after changing the drug. 32 years old, 4 gravidity 1 parity, abortion at 26, vaginal delivery at 29, and she developed deep vein thrombosis of the vena cava and bilateral common iliac vein at 5 weeks of gestation at age 30. She took oral edoxaban tosilate hydrate and opted for artificial abortion. The inferior vena cava lesion remained. Therefore, oral administration was continued. There was no thrombotic predisposition. This time, a spontaneous pregnancy was established, and we changed the anticoagulation therapy to subcutaneous injection of calcium heparin 10,000 units/day at 10 weeks gestation. D-dimer, which had been under 1.0µg/mL before pregnancy, elevated to 9.6µg/mL at 12 weeks gestation, and pulmonary thromboembolism and aggravated inferior vena cava thrombosis were detected by contrast-enhanced CT. We started continuous intravenous sodium heparin, but her platelet count decreased to 54,000/µL and heparin-induced thrombocytopenia was suspected. We changed to edoxaban tosilate hydrate and had an abortion. The pregnancy was complicated by venous thrombosis. The informed consent was required not only to allow and continue the pregnancy but also to change the drug.

 Neonatal hemochromatosis（NH）, a disease with poor prognosis, is characterized by iron deposition in the liver and other non-reticuloendothelial organs such as the pancreas by maternofetal alloimmune mechanisms, resulting in severe liver damage. Although diagnostic criteria have been proposed, diagnosis remains difficult. Our patient was a preterm, very low birth weight baby, born at 25 weeks 6 days of gestation with a birth weight of 1,056 g. The infant developed metabolic acidosis, hyperlactacidemia, anemia, and disseminated intravascular coagulation immediately after birth, which did not respond to treatment. The patient died of multiple organ failure 43 h after birth. The autopsy revealed a massive hepatocellular necrosis. Iron deposits were seen not only in the hepatocytes but also in the pancreas. Furthermore, C5b-9, activated by anti-fetal liver IgG antibodies in maternal blood, was positive in hepatocytes by immunohistochemistry; therefore, NH was diagnosed. It is not easy to diagnose NH before birth, as tests for NH diagnosis are scarce in very preterm infants. Hence, autopsy is important because NH is also included in the differential diagnosis of death from multiple organ failure of unknown cause.