The number of pregnancies complicated with cardiovascular disease is increasing due to the improved prognosis of patients with congenital heart disease; the increased number of diseases diagnosed at a young age by genetic examination, such as channelopathy and the increased number of advanced maternal age pregnancies. Through the experience of pregnancy and childbirth, maternal hemodynamics change dynamically, and the risk of cardiovascular complications increases. Therefore, professional involvement is recommended from the time of preconception counseling.
In the clinical management of pregnancy with cardiovascular disease, we will attempt to prevent complications and make an early diagnosis by knowledge of the changes and timing of maternal hemodynamics. In addition, although maternal safety is the highest priority in principle, we should become knowledgeable of teratogenicity and fetal toxicity in maternal drug treatment and the effects of fetal exposure and contrast media use in radiological examinations, and carry out appropriate examinations, as well as carrying out treatments during pregnancy. About 10% of maternal deaths in Japan are caused by cardiovascular disease. Thus, team medical care, in which obstetrics, cardiology and other related departments cooperate, is essential in perinatal medical care in high-risk cases.
Neural tube defects（NTDs）comprising myelomeningocele and anencephaly were proven to be folic acid- preventable in the United Kingdom in 1991. The Ministry of Health and Welfare recommended in 2000 that women planning to conceive should take folic acid supplements 0.4 mg a day from a month before to three months after conception in order to reduce the risk of NTDs in the fetus. The Japan Association of Obstetricians and Gynecologists（JAOG）reported that the prevalence of myelomeningocele has remained unchanged while that of anencephaly has significantly decreased over the past 20 years, where the number of fetuses terminated because of NTDs in the first trimester has not been counted. Subsequently, we attempted to correct the prevalence of NTDs from 2016 to 2018 based on data supplied from 536 hospitals by incorporating the number of above fetuses terminated, which varied from 7.93 to 8.82/10,000 deliveries and was 1.5-fold higher than that of JAOG. Furthermore, the clinical effects of folic acid on the occurrence or recurrence of NTDs, risk factors of NTDs, and awareness and intake of supplements will be analyzed and discussed. We believe that the Japanese government should implement the fortification of grains with folic acid soon, which will certainly decrease the number of fetuses afflicted with NTDs.
This study evaluated retrospectively predictive parameters for massive blood transfusion in postpartum hemorrhage caused by uterine atony. Additionally, we considered benchmarks for the appropriate timing of maternal transfer. Among postpartum hemorrhage cases treated with red blood cells and fresh frozen plasma, we have reviewed 88 cases in which uterine atony was thought to be the main cause of bleeding. MT group, treated with massive blood transfusion of 10 or more units of packed red blood cells, was 46 cases, and non-MT group was 42. There was significant difference between both groups for hemoglobin levels（Hb）, count of platelets（Plt）, fibrinogen levels（Fib）and shock indexs（SI）measured before treatments. Multivariate analysis of bleeding factor led that pre-treatment Fib ≦ 171mg/dL or SI ≧ 1.3 is predictive parameters for massive blood transfusion, odd’s ratio（95％ confidence interval）was 4.81（0.62-0.83）and 7.40（0.61-0.82）respectively. Positive predictive value was 73％ and 82％ respectively. For postpartum hemorrhage caused by uterine atony, pre-treatment Fib ≦ 171mg/dL or SI ≧ 1.3 is the benchmark of maternal transfer to tertiary medical institution.
Extremely low birth weight infants（ELBWI）are reported to be at high risk of hearing impairment and have a high abnormal rate in Auditory Brainstem Response（ABR）test. However, ABR abnormalities are often transient, and in many cases hearing impairment is denied by subsequent follow-up. We examined the developmental outcome of ELBWI who had abnormalities in ABR test before discharge from NICU but did not have hearing impairment. Twenty-two ELBWI were included, with median gestational age of 24.7 weeks and median birth weight of 618 grams. Cerebral palsy was found in 3cases and Autism Spectrum Disorder in 5 of 22 cases.
Developmental Quotient（DQ）of 1 year 6 months old and 3years old and Intelligence Quotient（IQ）of 6 years old were significantly lower than those without ABR abnormalities.
ELBWI with ABR abnormalities but no hearing impairment are at high risk of developmental delay, so intervention such as developmental support should be considered from early stage.
Ketamine hydrochloride has been regulated as a narcotic drug in Japan since 2007, although it has been placed on the WHO Model List of Essential Medicines. To evaluate the efficacy and safety, we reviewed 32 cases in which intravenous anesthesia with ketamine was given in the maternity ward of our hospital between January 2007 and December 2018.
In 22 cases, manual extraction of the placenta was performed. Blood loss was between 130 ml and 2100 ml （mean: 1096 ml）. No women had blood transfusion. The other cases include instrumental placental extraction and/or uterine balloon tamponade（3 cases）, hemostatic treatments for vaginal laceration or hematoma（4 cases）, and repair of the third or fourth degree of perineal laceration（3 cases）. Although 5 women needed blood transfusion, all the women recovered well after the treatments.
The dosage of ketamine was between 30 mg and 80 mg（mean: 43 mg）. It was administered alone in 27 cases, and with other sedative or analgesic agents in 5 cases. Sufficient analgesic/anesthetic effects were obtained in all the cases. There were no cases of acute hypotension, respiratory arrest, aspiration, or subsequent drug addiction. No adverse effects were noted in the infants breastfed by the mothers who were anesthetized with ketamine.
Although ketamine has a favorable efficacy and safety profile in cases of obstetric emergencies, there is a major obstacle in the timely and prompt use of ketamine in Japan. The regulation of ketamine as a narcotic drug should be reconsidered from the viewpoint of maternal lifesaving.
A self-administered questionnaire survey was conducted among 56 pregnant women who smoke and who visited our hospital, which included questions on smoking habits. The average number of cigarettes smoked daily before and during pregnancy was found to be 17.4 and 6.9, respectively. Moreover, 90％ of them reported reducing the number of cigarettes smoked after pregnancy. The average tar content in the cigarettes was found to be 7.7 mg, and many subjects reported smoking low-tar cigarettes. About 90％ of the smoking areas in their homes were in the same room or under a ventilation fan. The drinking rate during pregnancy was about 16％, and less than half of the participants ate breakfast daily. Most stages for quitting smoking in pregnant women were the preparation stages. Perinatal abnormalities were found in approximately 90％ of patients. The most commonly reported reason for difficulty in smoking cessation was "for a change of pace." The knowledge rate about the harmful effects of smoking was high for "preterm birth" and "fetal growth restriction（FGR）" but low for "attention deficit hyperactivity disorder（ADHD）," "obesity/diabetes," and "erectile dysfunction（ED）." The knowledge rate of women who smoked during pregnancy was significantly lower than that of pregnant women who smoked before their pregnancy and those who did not smoke at all（p <0.05）. It can thus be concluded that women who smoke during pregnancy are less informed of the harmful effects of smoking during pregnancy, suggesting the importance of smoking cessation education for pregnant women.
The initial care of neonates of unplanned out-of-hospital births is often done by paramedics, but an educational system related to perinatal care provided for paramedics has yet to be established. The objective of our study was to describe the neonatal outcomes of unplanned out-of-hospital births and to clarify the issues related to perinatal education for paramedics. From January 2008 to December 2018, a total of 40 neonates born out of hospital were transported to our hospital by paramedics. In 23 of those neonates（57.0％）, the paramedics attended the deliveries, whilst the remaining 17（43.0％）were born before the paramedics’ arrival. Although 32 of the neonates underwent only routine neonatal care after birth, five required resuscitations by positive pressure ventilation including one who also needed chest compressions. There were three neonatal deaths, in which two of the neonates had congenital disease and the other was born with severe asphyxia due to cervical umbilical cord winding and difficulty in delivery. An Apgar score by paramedics was registered in only 16 of the cases（40.0％）. Hypothermia, defined as a body temperature below 36 degrees Celsius, was present in 24 of the cases（60.0％）, and it was the most common neonatal complication, revealing the importance of out-of-hospital management of body temperature. Recently, more paramedics have taken classes in perinatal emergency; however, a training course that fully covers out-of-hospital births is still unavailable. To decrease neonatal morbidity and mortality, more practical educational courses on out-of-hospital births for paramedics that focus on the issues identified by this study should be established.
Transportation from remote islands may take a considerable amount of time to the destination due to restrictions on the transportation method depending on the date and time when the transportation is decided.
We examined the application cases, transportation method and transportation time, in addition to outcomes of transporting cases for emergency maternal transportation from Shodoshima island, a remote island in the Seto Inland Sea to central hospitals. During the four years from April 2016 to April 2020, there were 17 cases of pregnant and puerperal women urgently transported from Shodoshima island. The causes of transportation were threatened preterm delivery in 10 cases, premature rupture of membrane（Preterm PROM）in 3 cases, abdominal pain or bleeding during pregnancy in 2 cases, and postpartum hemorrhage in 2 cases. Maternal transportation during the preterm birth period including threatened preterm delivery and preterm PROM was the most common, accounting for 76.5%. As for the transportation method, there ware 13 cases transported by helicopters, 2 cases by ambulance boat and 2 cases by regular ferry transportation. The actual transportation time to the destination hospitals was 17 minutes on average for disaster prevention helicopters, about 70 minutes for ambulance boats, and about 100 minutes for ferry transportation. Transportation by helicopter is very effective in terms of time, but it may not be possible to fly at night or depending on the weather conditions. In cases where preterm birth is predicted, early decision on transportation is required in consideration of the transportation method.
It has been reported that Family-Centered care is important for better neurodevelopmental outcome of NICU babies. Single-family room-NICU is recommended for family to be involved in developmental care of the NICU babies. We optimized NICU design from Open-bay design to semi single-family room-NICU. A questionnaire survey was conducted to clarify the staff’s and parent’s perception due to the environmental change of NICU design, and to research the benefits and risks. As a result, both of staffs and parents answered that parents can stay more comfortably in NICU. They were satisfied the heights of wall, and the breadth per patient. There is a difficulty of observation because of the decreased patient visibility and greater distances between patients. We recommended that closed observation should be required.
Background: The unexpected detection of severe fetal heart defects（FHDs）can impart serious burden on Satogaeri pregnant women and their families. Satogaeri refers to pregnant women leaving their residence and returning to their parents’ residence for receiving perinatal care support.
Objective: This study aims to report the results and issues regarding prenatal diagnosis of severe FHDs in Satogaeri pregnant women at a rural institute.
Method: For over a period of 3 years, beginning in January 2017, 1,662 patients underwent fetal echocardiography at our institute under study. The prenatal diagnosis of FHDs and the outcomes in Satogaeri patients were retrospectively reviewed using electronic medical charts.
Results: Of the 1, 662 patients, 346（21%）were Satogaeri. Additionally, the gestational age of Satogaeri patients was significantly higher than that of the non-Satogaeri patients（mean, 33.5 ± 2.6 weeks vs. mean, 27.2 ± 2.1 weeks, respectively; p < 0.01）. Nine Satogaeri patients had FHDs, of which three had fetal double outlet right ventricle, common arterial trunk, and pulmonary atresia with ventricular septal defect, and were hence referred to the tertiary institute.
Conclusion: Severe FHDs after Satogaeri tend to be diagnosed during late gestation. Therefore, during the limited period until childbirth, changing the delivery institute may be necessary for the Satogaeri patients and their families without proper information about the fetal heart or sufficient psychological support available from practitioners. Thus, prior to Satogaeri, fetal echocardiography, including three-vessel trachea view for evaluating great vessel anomalies associated with many severe FHDs, is strongly recommended.
We evaluated the utilization of special needs education and the complications at school age in children who had an extremely preterm birth. Using a mail questionnaire, we performed an analysis of 91 children born at less than 28 weeks of gestational age at Kitasato University Hospital between 2005 and 2014. Of these, 72 patients belong to regular class, 8 patients need special education in general school, and 11 patients attend special education schools. At the junior high school level, 78.1% of all patients belong to the regular class. The presence of intraventricular hemorrhage（IVH）, periventricular leukomalacia（PVL）, necrotizing enterocolitis-focal intestinal perforation（NEC-FIP）, severe small for gestational age（SGA）, and abnormal ABR significantly reduced the rate of registration for regular classes. Sixty-two patients had some complications; 13 patients had strabismus, and 26 patients（28.6%）needed glasses. In this study, the number of patients requiring special education is almost equivalent to that reported in previously studies. Few children have changed class throughout their school age; therefore, junior high school children, at tend the same class as that in elementary school. The incidence of abnormal ABR at the time of leaving hospital has been to have poor outcomes. We have to carefully observe strabismus because it tends to have a high incidence.
Background: Japanese neonates are considered to have a high risk of developing neonatal jaundice because of their ethnicity and high frequency of carrying the UGT1A1 gene mutation. Although the criteria for initiating phototherapy have been clarified, no studies have examined the rebound in serum total bilirubin levels after phototherapy for neonatal jaundice in Japan.
Objective: To confirm the trend in serum total bilirubin levels after phototherapy and verify the need for follow-up total bilirubin testing.
Methods: This was a retrospective chart review of neonatal jaundice after phototherapy among neonates with ≥36 weeks of gestation, a birth weight of ≥2,500 g, and no other complications who were treated at the newborn nursery of the University of Tsukuba Hospital between January 2010 and August 2014. The clinical features of the patients with repeat phototherapy were compared with those of the patients without.
Results and conclusion: In total, 143 patients were enrolled. Only 4 patients（2.8%）required repeat phototherapy. 3 of the 4 patients were born at 37 weeks of gestation, and 3 of the 4 patients were started on phototherapy at the age of 2 days. No patients required repeat phototherapy on the condition that the serum total bilirubin level at 24 h or 36 h after the end of phototherapy was lower than the level at the end of phototherapy. Under these conditions, subsequent follow-up total bilirubin testing may not be necessary.
There were a few reports concerning adhesive small bowel obstruction（ASBO）in each disease required neonatal laparotomy. In this retrospective study, we reviewed characteristics of primary diseases and ASBO in 81 patients（56 patients required adhesiolysis）who had neonatal laparotomy in our hospital between 1970 and December 2012. The primary diseases were intestinal atresia（IA）including meconium peritonitis in 14 patients （occurrence frequency 5.7%）congenital diaphragmatic hernia（CDH）in 10 patients（6.2%）, congenital abdominal wall anomaly in 9 patients（5.7）, intestinal perforation in 7 extremely low birth weight infants（9.1）, anorectal anomaly in 6 patients（3.5%）, malrotation in 4 patients（3.8%）, and other diseases in 6 patients. Each median age at first adhesiolysis was 0.75 years old in all patients, 1.12 years old in IA, 0.83 years old in CDH, 0.92 years old in abdominal wall anomaly, 0.17 years old in intestinal perforation, 1.71 years old in anorectal anomaly, and 5 years ole in malrotation. More than half of the patients（30 patients）required adhesiolysis in infancy. The median last follow up age was 8.67 years old. The factor occurred ASBO was suspected either pathological condition or laparotomy.
To evaluate the clinical features and perinatal outcomes of monochorionic diamniotic（MCDA）twins with sIUGR Type I.
This single-institution retrospective cohort study enrolled MCDA twins with sIUGR Type I（umbilical artery Doppler with positive diastolic flow）diagnosed before 26 weeks’ gestation. Pregnancies with major structural abnormalities, twin-twin transfusion syndrome（TTTS）diagnosed within 1 week after referral to our center, undetermined chorionicity during the first trimester, and women who gave birth at other hospitals were excluded. The primary outcome was poor prognosis, including spontaneous miscarriage, intrauterine fetal death（FD）, neonatal death. Secondary outcomes were immediate delivery due to fetal compromise, unexpected intrauterine fetal death, and monochorionic-specific complications, including TTTS, twin anemia-polycythemia sequence, and alteration to sIUGR Types II and III.
Among the 57 sIUGR Type I cases, 40 were finally included in this study, after excluding 17 cases. Of the 40 cases, four（10%）had a poor prognosis, including three FD cases and one neonatal death. Immediate delivery due to fetal compromise, unexpected intrauterine fetal death, and monochorionic-specific complications occurred in 10 （25%）, 1（2.5%）, and 17（42.5%）cases, respectively.
Several sIUGR Type I cases have poor prognosis and many cases are high risk; therefore, they should be given particular attention.
To clarify perinatal management issue for pregnant women with epilepsy, we investigated 58 mothers with epilepsy retrospectively among 15, 344 mothers who had delivery at Japanese Red Cross Medical Center from January 2014 to December 2018.
Among 58 mothers, 26 exposed antiepileptic drugs（AEDs）during pregnancy. Compared women with and without AEDs during pregnancy, there was no significant increase in gestational age at delivery, amount of bleeding, birth weight, Apgar score, and cord blood gas analysis. Epileptic seizures occurred in 13 mothers during pregnancy especially in the mothers who discontinued or switched AEDs after awareness of their pregnancy. Most of mothers（94.8%）were breastfeeding with or without formula. Women with epilepsy should be informed about the necessity of AEDs for seizure control and possibility of adverse effect including congenital anomalies.
The effects of ritodrine hydrochloride tablets, which are frequently used for threatened premature labor, on gross fetal movement were investigated using the fetal movement acceleration measurement recorder. A total of 171 singleton pregnant women who delivered at Teikyo University Hospital from April 2010 to August 2019 participated. Sixty-five women（an administered group）were given ritodrine hydrochloride tablets, and the other 106 women（a non-administered group）were not. Fetal movement ratios were counted weekly at home after 28 weeks of gestation. The ratios were compared between the two groups at an earlier（28-31 weeks）and later pregnancy（32-35 weeks）. The ratios were 18.84 ± 9.06% in the administered group and 17.33 ± 7.92% in the non-administered group at the earlier pregnancy（p = 0.90）. As for the later pregnancy, they were 13.87 ± 5.52% and 14.64 ± 6.94%, respectively（p = 0.15）. Administration of ritodrine hydrochloride tablets did not affect fetal movement ratio during pregnancy.
Presepsin levels have been appreciated as an early diagnostic biomarker of sepsis with a high level of evidence, because presepsin levels rise earlier than levels of other well-known markers of inflammation. Presepsin levels are not easily affected by invasive injuries and surgeries, but they reflect treatment responses and the severity of bacterial infection. Five hundred and one pregnant women aged 20 years and over who received medical care from the first trimester of pregnancy and delivered a baby in the Department of Gynecology, Fukui Aiiku Hospital from April 2018 to May 2019 were enrolled in the study. Pregnant women with comorbid conditions and plurifetation, and stillbirth were excluded. Presepsin levels in whole blood, complete blood cell counts and CRP levels were measured during pregnancy period（median gestational ages: 28weeks and 3days） and at delivery times（median gestational ages: 39weeks and 4days）. Presepsin levels at each time showed no significant correlation with complete blood cell counts and CRP levels. Next, we analyzed the effects of prenatal factors associated with presepsin levels at delivery times, there were significant differences in presepsin levels and the rate of change of presepsin levels from pregnancy period to delivery times between pregnant women with and without threatened premature labor, same as only about preterm deliveries. There was a significant inverse correlation between the time from threatened premature labor to delivery and presepsin levels at delivery times. In conclusion, it was suggested that presepsin levels at delivery times may be an indicator of inflammation during pregnancy period that causes threatened premature labor.
With the COVID-19 pandemic, the medical community was required to respond differently from the conventional medical management. Our hospital has reduced the number of medical examinations in pregnant women with no complications and fetal abnormalities. This study aimed to clarify whether there is a difference in perinatal prognosis due to changes in the frequency of medical examination in pregnant women and to evaluate the psychological status of these patients.
Our study included 488 pregnant women who delivered at our hospital. Women without complications requiring regular hospital visits and fetus with no disorders were selected. The patients were divided into Control（n=238） and Case（n=250）groups; multivariate analysis for incidence of maternal and fetal perinatal complications, and delivery and neonatal outcomes was performed. Moreover, psychological evaluation using a questionnaire was performed.
In multivariate analysis, no differences were found among all factors of perinatal outcomes. From the psychological evaluation, 68.0% of questionnaires were collected. Multiparous women tended to be satisfied with the content of our pregnancy management, although in both groups, they felt that examinations were less performed.
The post COVID-19 pandemic prenatal checkup system in our hospital did not affect the perinatal prognosis. Meanwhile, all pregnant women had vague feelings of anxiety due to increased prenatal checkup intervals, and psychological support appeared insufficient. In the post COVID-19 era, conducting more studies on perinatal management may lead to safer outcomes.
Fetal intracranial hemorrhage（ICH）is a rare phenomenon that potentially leads to a life-threating event or postnatal neurological sequelae. Various causes of fetal ICH have been reported, such as coagulopathy, thrombocytopenia and angiopathy induced by transplacental alloantibodies or medication, and fetal congenital disease.
A 25-year-old primigravid woman was referred to our hospital at 31 weeks of gestation in whom ultrasound showed dilatation of the lateral ventricle and echogenic findings around that. Fetal magnetic resonance imaging （MRI）revealed right-sided unilateral ventriculomegaly with associated hematoma, suggesting periventricular venous infarction（PVI）. No plausible cause was identified. Fetal hemorrhagic predisposition could not be ruled out, thus an elective cesarean procedure was done at 37 weeks of gestation and a female with a good Apgar score was successfully delivered. The baby showed a normal appearance and blood test results, while head MRI revealed dilatation of the right lateral ventricle with an intraventricular hematoma and co-lateral decreased white matter volume. Discharge from the neonatal intensive care unit occurred at 15 days of age with no paralysis or seizure symptoms, then at 12 months of age she was diagnosed with mild left hemiplegia. The course of pregnancy in this case was uneventful, though we considered that an intrauterine insult related to respiratory or circulatory instability occurred by an unknown cause, resulting in ICH.
Beckwith-Wiedemann syndrome（BWS）is characterized by macroglossia, overgrowth, and abdominal wall defects. It is caused by the imprinting of multiple genes on chromosome 11p15, and it has been reported to affect 1 in 13,700 newborns. A small percent of BWS cases are caused by trisomy of the chromosomal segment containing 11p15 from the father. Partial monosomy of 18q is characterized by delayed myelination and psychomotor retardation. We report a case of BWS with karyotype 46, XY, der（18）t（11;18）（p15.3; q21.1）. The male patient was born at 38 weeks and 5 days of gestation with a birth weight of 3406 g（+1.5 standard deviation（SD））, a length of 51.5 cm（+1.6 SD）, and a head circumference of 32.5 cm（-0.5 SD）. At birth, he showed low-set ears, widely spaced eyes, macroglossia, atrial septal defect, umbilical hernia, left inguinal hernia, and congenital spinal malformation. At the age of 2 years and 7 months, he presented psychomotor retardation as evaluated by the Kyoto Scale of Psychological Development; he had a Postural-Movement Developmental Quotient （DQ）of 23, Cognitive-Adaptive DQ of 32, Language-Social DQ of 31, and a total DQ of 29. Chromosome G-banding and the analysis of all subtelomeric regions revealed that he had karyotype 46, XY, der（18）t（11;18）（p15.3;q21.1）pat. Previous reports indicated that BWS with unbalanced translocation is characterized by psychomotor retardation and macroglossia. Post-diagnosis follow-up was considered to be important from the viewpoints of psychomotor development, tumor potential, and genetic counseling.
In Japan, phenobarbital（PB）is recommended as the first-line agent and midazolam（MDL）is recommended as the second-line agent for treatment for neonatal seizures. However, there is not sufficient evidence to determine the optimal agent for neonatal seizures for which PB and MDL are not effective. We report a case in which fosphenytoin（fos-PHT）was effective for neonatal seizures. The male infant was born at 40 weeks by vacuum extraction due to a non-reassuring fetal status. His Apgar score was 1 at 1 minute, 4 at 5 minutes, and 5 at 10 minutes, and he was admitted to Neonatal Intensive Care Unit with suspected neonatal asphyxia. On 32 hours after his birth, the amplitude-integrated EEG waveform showed a saw-tooth pattern, and the same type of waveform was observed for approximately 30 seconds on the simultaneously recorded EEG. Following this EEG abnormality, he had clonic seizures in his left upper limb and we had diagnosed neonatal seizures. The seizures persisted after administration of PB and MDL, and only disappeared after the administration of fos-PHT. No side effects such as hypotension or bradycardia due to the fos-PHT were observed. His parents were informed of this off label use of fos-PHT for their infant and agreed to our proposal. We recognized that fos-PHT was effective and safe drug for neonatal seizures.
Although uterine leiomyomas can cause various obstetric complications during pregnancy, few reports have described venous thromboembolism during pregnancy associated with uterine leiomyomas. We present a case of a pregnant woman with a uterine leiomyoma who developed pulmonary embolism（PE）after cesarean section and was successfully treated with intensive care, including cardiopulmonary support.
A 36-year-old primigravida woman with a giant leiomyoma of approximately 14cm in diameter was referred to our hospital. Cesarean section was performed because of arrest of labor. On postoperative day（POD）1, cardiopulmonary arrest occurred during the first ambulation, and we started chest compression. We strongly suspected PE and used tracheal intubation and venoarterial extracorporeal membrane oxygenation（VA-ECMO）（percutaneous cardiopulmonary support［PCPS］）. Contrast-enhanced computed tomography（CT）revealed PE. No thrombus was observed in the pelvic vein, but the inferior vena cava was compressed by the enlarged uterus. The patient was discharged on POD 34 without major complications.
PE should be suspected in patients with postoperative cardiac arrest if they have giant uterine leiomyomas. Early diagnostic imaging using enhanced CT and intensive care with immediate application of VA-ECMO（PCPS） are believed to be crucial for successful treatment in this case. In addition, it is important to evaluate for thrombus, including coagulation function testing, and administer perioperative prophylactic anticoagulation therapy for pregnant women with giant uterine leiomyomas.
The carnitine kinetics of three extremely low birth weight infants who received sivelestat sodium（sivelestat） for severe respiratory distress were investigated. Free carnitine（C0）is consumed during the excretion process of the pivoxyl group present on sivelestat, while pivaloylcarnitine（C5piv）is synthesized and excreted in the urine. C0 was found to decrease with administration of sivelestat, and to remain low for about 2 weeks post administration. C5piv, on the other hand, was found to be elevated in both blood and urine following sivelestat administration while urinary excretion continued for approximately 2 weeks. The effect of sivelestat on carnitine metabolism has previously been mentioned and our results support these findings. It has been suggested that extremely low birth weight infants are more likely to become carnitine deficient and require carnitine supplementation, not only during sivelestat administration, but also afterwards. In addition, the increase of C5piv in blood after sivelestat administration may cause false positive isovaleric acidemia in neonatal mass screening and caution is therefore required.
Psittacosis is a zoonotic disease from bird species caused by Chlamydia psittaci, which infects humans. The disease presents with various symptoms, including mild influenza-like illness, severe multiple organ dysfunction, and disseminated intravascular coagulation（DIC）. We present a case of gestational psittacosis of the placenta with massive perivillous fibrin deposition（MPFD）leading to intrauterine fetal demise at 17 weeks of gestation.
A 31-year-old pregnant woman（15 weeks of gestation, gravida 4, para 3）visited a clinic due to fever, headache, and cough. Two weeks later, she was admitted at a local hospital due to persistent fever and was then transferred to our hospital due to suspected meningitis. Physical examination demonstrated DIC caused by bacterial sepsis. Subsequently, she received intensive care, antibiotics, and anti-DIC treatment. On the second day, she was diagnosed with intrauterine fetal demise and the fetus was delivered by induction on the third day. On the same day, the mother was intubated due to pulmonary edema. Placental histopathologic examination revealed massive perivillous fibrin deposition. As a viral infection was suspected, we requested for further research by the National Institute of Infectious Diseases. They detected C. psittaci DNA only from the placental tissue. In conclusion, gestational psittacosis of the placenta caused maternal bacterial DIC, which resulted in placental dysfunction with MPFD and eventually fetal death. Heightened awareness for pregnant women to avoid contact with bird species should be observed. However, the extent of contact that can be critical remains unclear, and a future study is warranted to shed light on this.
Gastroesophageal intussusception（GEI）is a rare and serious condition in which part of the wall of the stomach slides against the esophageal lumen. Although hyperemesis gravidarum and Valsalva maneuver at the time of delivery are considered to be risk factors for GEI, there are few reports associating it with pregnancy. Herein, we present a case of successful endoscopic treatment for GEI during pregnancy as well as a safe delivery by scheduled cesarean section. A 28-year-old primigravida with a history of ileus of unknown origin presented with frequent vomiting and intermittently increasing left abdominal pain at 31 gestational weeks. At 31+2 weeks’ gestation, a nasal gastric tube was inserted due to persistent bloody vomiting. X-ray examination showed a widely dilated esophagus and MRI examination revealed that the stomach had been sliding against the dilated esophagus, and she was diagnosed with GEI as a result. Her stomach was restored to its original position using upper endoscopy under general anesthesia. Endoscopic findings revealed laceration on the stomach wall but no necrosis or perforation. As we considered abdominal pressure during labor to be a risk factor for GEI recurrence, a scheduled cesarean section was performed at 37+5 weeks’ gestation to prevent GEI recurrence. A female infant weighing 2,290 g was delivered safely. The optimal treatment and delivery mode in pregnant women with GEI has not yet been elucidated due to the small number of reports on GEI occurring during pregnancy. To our knowledge, this is the first report in the world that a case of successful endoscopic reduction of GEI occurring during pregnancy.
Cleidocranial dysplasia（CCD）is a rare autosomal dominant inherited disorder that is characterized by skeletal dysplasia, such as delayed closure of cranial sutures, hypoplastic clavicles, and late erupting secondary dentition. We present a case of CCD in a female full-term infant weighing 2,272 g at birth. She had abnormally large, wide-open fontanelles and hypoplastic clavicles. The diagnosis of CCD was confirmed by array comparative genomic hybridization, which revealed 6p partial deletion involving Runt related transcription factor 2（RUNX2）. Her parents were facing social issues, also had psychiatric disorders, with no caretakers in their family. We held conferences consisting of medical doctors, midwives, nurses, medical social workers, and public health staffs to discuss about the preparation for her discharge. She was discharged at 4 months of age in cooperation with the support team. However, even after the discharge, team support was required to help maintain and evaluate the child care environment by her parents. We anticipate that this interdisciplinary approach for a socially high-risk family will lead to better child-family safety and prevent or minimize abuse on children who need special care, such as those with CCD.
Klippel-Trenaunay-Weber syndrome（KTW）is a congenital disease with the main clinical features of vascular malformations, soft tissue and/or bone hypertrophy, and varicose veins. There are only few reports of pregnancy with KTW woman in Japan. Our case was 25 years old, primipara woman with KTW who got pregnant spontaneously. She admitted in our hospital by threatened preterm labor at 25 weeks of pregnancy. Spinal cord MRI revealed the epidural varicose in the height of second to third thoracic vertebra. We selected cesarean section to avoid epidural varicose injury and to make strict management of blood pressure. At 36+1 weeks of pregnancy, she delivered 3,110g male baby via cesarean section. We did not use unfractionated heparin because she had past medical history of spinal cavernous hemangioma rupture, but she got no venous thromboembolism in her pregnancy course.
Cervical cancer diagnosed during pregnancy occurs in approximately 1.4−4.6 in every 100,000 pregnancies. We report 2 cases that underwent neoadjuvant chemotherapy with carboplatin and paclitaxel（TC therapy） for cervical cancer during pregnancy.
Case 1 was a 40-year-old woman at 24 weeks gestation, who was diagnosed with stage IB1 squamous cell carcinoma of the cervix.
She was treated with 3 courses of TC therapy before Cesarean section at 34 weeks gestation to deliver a baby weighing 1,878 g.
Case 2 was a 34-year-old woman at 13 weeks gestation, who was diagnosed with stage IB1 squamous cell carcinoma of the cervix. She was treated with 5 courses of TC therapy before Cesarean section at 34 weeks gestation to deliver a baby weighing 1,944 g.
Both women underwent radical hysterectomy 3 weeks after their Cesarean sections, and they have had no recurrence for 5 years.
The baby born to Case 1 has shown normal growth and development until 3 years old, at the completion of follow-up. The baby born to Case 2 has shown delayed growth and bone development（approximately −1.9 standard deviation）at 5 years old.
Neoadjuvant chemotherapy during pregnancy remains controversial.
Neoadjuvant chemotherapy with TC therapy could be considered a safe and effective treatment option for cervical cancer during pregnancy. Especially, carboplatin has been reported to be less ototoxic than cisplatin. Neither baby developed hearing loss as a side effect of carboplatin.
We present the case of a pregnant woman, who developed a fracture of the left femoral head. The fracture resolved with conservative management and the patient delivered a healthy baby vaginally at 39 weeks of gestation. We then determined that the fracture had been caused by transient osteoporosis of the hip（TOH）, which mostly affects middle-aged men and pregnant women with hip joint pain in the later stages of gestation. It often resolves spontaneously with rest and restricted weight-bearing. The pathophysiology of TOH in pregnancy is unclear, however several hypotheses have been suggested, including increased pressure in pelvic veins or obturator nerves, and an association with Sudeck’s bone atrophy. We reviewed the Japan Medical Abstracts Society database from January1990 to December 2019 and found 55 cases of TOH in pregnancy. The most prevalent site of involvement was the right femur; this differs from previous reports that mentioned the left femur as the predominant site. The diagnosis of TOH tends to be delayed as radiation exposure is avoided in pregnant women. Magnetic resonance imaging is the most useful diagnostic tool, because it offers specific views of TOH without radiation exposure. Obstetric complications increase the frequency of bone fractures. In case of hip pain or edema during pregnancy, including TOH in the list of diagnostic possibilities will lead to its early diagnosis and improved prognosis.
Here, we report a preterm infant with severe herpes simplex virus type 1（HSV-1）infection with acute retinal necrosis. The case was a male infant weighting 1,321g born at a gestational age of 29 weeks 4 days. His mother had no past medical history of genital herpes, but she had a medical history of oral herpes. She came to our hospital due to membrane rupture at an estimated 29 weeks 1 day of gestation. On day three of admission, the baby was born by emergency caesarean section. He was intubated and received mechanical ventilation due to respiratory distress syndrome and weaned with the aid of continuous positive airway pressure on day five. On day four, skin rashes appeared temporarily but disappeared on the next day. He required theopyline therapy, because apneic spells worsened on day twelve. On day 29, an ophthalmologic examination showed acute retinal necrosis and cranial ultrasonography showed ventricular dilatation. He was diagnosed with HSV-1 infection with positive HSV-DNA tests of the anterior chamber and cerebrospinal fluid. Cerebrospinal fluid HSV-1 antibody was positive, whereas HSV-2 antibody was negative. Although he was treated with 3 weeks intravenous acyclovir that has shifted to oral administration, retinal detachment progressed and brain MRI findings revealed multicystic encephalomalacia on day 63.
A 35-year-old pregnant woman（G1P0, 35 weeks and 3 days）was admitted to our hospital for hematemesis and consciousness disorder. Vital signs during admission were as follows: body temperature, 36.6℃; heart rate, 161 bpm; blood pressure, 182/130 mmHg; respiratory rates, 35 /min, SpO2, 99%（15 L/min with a non-rebreather mask）; and Glasgow coma scale, E4V4M6. The fetal heart rate was 70–100 bpm, indicating a non-reassuring fetal status. The patient had a tongue bite and stomatorrhagia, but no bleeding presented with gastric lavage. Venous blood gas data showed pH, 6.94 and Lac, 170 mg/dL, and the urine protein was 4+. The patient was diagnosed with consciousness disorder due to eclampsia, stomatorrhagia due to tongue bite, and non-reassuring fetal status due to maternal and fetal hypoxia resulting from convulsion. After administering nicardipine and magnesium sulfate, both the maternal vital signs and fetal status improved. However, due to prolonged disorder of maternal consciousness, we performed an emergency cesarean section under general anesthesia. The birth weight of the infant was 2,260 g, and the Apgar scores were 1 at 1 min and 6 at 5 min. The infant was premature and admitted to Neonatal Intensive Care Unit（NICU）. The patient was discharged on day 11 of postoperation, with no further symptoms after discharge. The infant was discharged at 18 days of age, and no physical growth and developmental disorders were observed 1 year and 4 months after birth.
Reversible cerebral vasoconstriction syndrome（RCVS）is caused by transient diffuse segmental constriction of cerebral arteries and characterized by "thunderclap headache," which is acute severe headaches with or without other acute neurological symptoms. We experienced a case of pregnant woman with RCVS at term.
Case: A 28-year-old pregnant woman was transferred to emergency room with a sudden onset severe headache at 37 weeks’ gestation. Although she was suspected to have a subarachnoid hemorrhage because of the "thunderclap headache," she showed no abnormal neurological findings on admission. Immediately after admission, she underwent emergency cesarean section because of the diagnosis of placental abruption with severe fetal bradycardia, and had a live infant. She was diagnosed as RCVS during her postoperative course.
Mid-aortic syndrome（MAS）is a relatively rare disorder characterized by hypertension mainly in the upper region of the body, headache, intermittent claudication, and ischemic symptoms of abdominal organs due to regional stenosis/occlusion of the abdominal aorta. In addition to acquired disorders derived from arteritis, some are due to genetic factors. There are few case reports of pregnancies with MAS, especially in congenital cases. We encountered a case of MAS in the neonatal period leading to abdominal aortic closure before pregnancy. In collaboration with cardiologists, we optimized antihypertensive drug therapy and evaluated the circulatory function. She had late-onset superimposed preeclampsia; however, she delivered vaginally at term. Pregnancy with unrepaired coarctation of the abdominal aorta is considered to have a very high perinatal risk, but adequate management of blood pressure and hemodynamic control before pregnancy may lead to a good perinatal outcome.
Recent efforts have made uterine compression suture（UCS）a feasible option for management of intractable bleeding during cesarean section complicated with abnormal placentation. Reported complications after UCS include infection, necrosis and secondary fertility. However, small bowel obstruction after UCS has never been reported.
Herein, we report a case of small bowel obstruction secondarily developed after cesarean delivery with UCS. A 31 years-old woman with previous history of two cesarean section conceived by in vitro fertilization and embryo transfer. At 38 weeks of gestation, elective cesarean section was performed. During procedure, UCS using Monodiox® was performed for difficult hemostasis due to retained placenta. Eighteen days after cesarean section, small bowel obstruction was diagnosed when she presented with severe abdominal pain. After failed conservative management with placement of ileus tube, laparotomy was chosen, eventually showing that coiling of thread of Monodiox® around small bowel at several portions is the cause of ileus. After removal of the coiled thread, recurrence of ileus was not observed. In summary, in a case managed by UCS, potential risk of secondary development of ileus should be cautioned to achieve early prompt management.
Very long-chain acyl CoA dehydrogenase（VLCAD）deficiency is a congenital abnormality in which mitochondrial fatty acid metabolism is altered. Patients present with myalgia, muscle weakness and rhabdomyolysis in hyper-catabolic states, such as prolonged fasting and intense exercise. There are few reports of VLCAD deficiency in pregnancy, and its management is unclear. In this report, we describe the management of a patient with VLCAD deficiency（myopathic form）during pregnancy. The patient was a 27-year-old, primigravida woman with a history of rhabdomyolysis following appendectomy at the age of 25 years. In early pregnancy, she presented with hyperemesis gravidarum that was successfully treated with administration of glucose-containing infusions. At 39 weeks and 4 days of gestation, the patient went into labor and admitted. She received intravenous glucose（5% solution）continuously from the time of admission until 3 days after the spontaneous vaginal delivery. Although she had a mildly elevated creatine kinase level on the day of delivery, she did not present with myalgia, muscle weakness, or rhabdomyolysis. It was, thus, suggested that timely glucose infusion may have prevented worsening of her symptoms.
We report on a case in which the patient, 39 years old, G1P0, developed idiopathic CSF hypovolemia in late pregnancy. She developed sudden strong headaches at 33 weeks and 4 days gestation, and was hospitalized. The course could not be diagnosed with imaging examination, therefore we examined the clinical condition of the patient which lead to a diagnosis of cerebrospinal fluid hypovolemia, and treated with conservative treatment. The patient’s condition did not improve and we therefore decided to perform a Cesarean delivery using epidural anesthesia at 38 weeks and 2 days gestation. A temporary respiratory depression occurred during anesthesia, but this was improved during the administration of oxygen. Through a postoperative cervical spine MRI examination a potential perforation near C7 was found, confirming the diagnosis. The baby was a female, weighed 3,006g, with an Apgar score of 8 points at 1 minute and 9 points at 5 minutes. The mother’s headache was relieved on the first day after surgery and both mother and child were discharged from the hospital without any problems. There is no specific guidelines for the management of idiopathic cerebrospinal fluid deficiency in pregnancy women. If the symptoms are not relieved with conservative treatment, we need to consider long-term management and delivery style individually.
The patient was a female infant with a history of fetal arrhythmia and she presented with a 2:1 atrioventricular block（AVB）and QT prolongation at birth. Two-to-one AVB due to congenital long QT syndrome was suspected, but the patient started to develop an alternating bundle branch block, and there were concerns that the condition might be due to a conduction disorder. The patient was placed under observation without medication, but at the age of 41 days, she developed a complete atrioventricular block. Thus, a pacemaker was implanted at the age of 70 days. The patient was later identified as carrying a SCN5A variant and was diagnosed with QT-prolongation syndrome due to SCN5A abnormality, accompanied by progressive cardiac conduction defects（PCCD）. This is the world’s first report of an alternating bundle branch block that was observed from the neonatal period, in which cardiac conduction defect progression was continuously confirmed.
Femoral-facial syndrome（FFS）is a rare syndrome characterized by femoral hypoplasia and specific facial features such as upslanting palpebral fissure and micrognathia. FFS has been to be associated with maternal diabetes. We experienced a case of FFS diagnosed in an infant after its birth from a diabetic mother, although skeletal dysplasia was suspected on the basis of severe femoral shortening. The mother was a 37-year-old woman, para 1, who was receiving treatment for type 2 diabetes. Severe femoral shortening and deformity were observed at 19 weeks’ gestation. Based on the low ALP level in the maternal blood test and three-dimensional computed tomographic imaging（3D-CT）at 29 weeks’ gestation, benign hypophosphatasia or type I osteogenesis imperfecta was suspected. A cesarean section was performed at 35 weeks’ gestation. The infant was diagnosed as having FFS on the basis of the specific facial features such as short broad-tipped nose, thin upper lip, long philtrum, and micrognathia, and bilateral femoral hypoplasia and left fibula aplasia. It is important to suspect FFS in a fetus with severe femoral shortening with diabetic mothers and examine its face such as micrognathia.
Group A Streptococci（GAS）causes maternal sepsis during postpartum period and neonatal sepsis. Such infections remain important causes of mortality. We report a case of neonatal GAS infection associated with maternal postpartum endometritis. The neonate was delivered vaginally at 39 weeks of gestation with Apgar score of 8 and 9 at 1 and 5 minutes, respectively. On day 7 after birth, He presented to the hospital with fever and toxic appearance. He was started on antibiotics for neonatal sepsis but resulted in superficial thrombophlebitis due to disseminated intravascular coagulation. The mother also presented in the same period with increasing lochia, fever and uterine tenderness which was diagnosed as postpartum endometritis. GAS was detected in the neonatal blood culture and the maternal vaginal culture. This case demonstrates that when the risk of group B Streptococci infection is low, the need of rapid initiation of antibiotics and screening for multiple organ failure and disseminated intravascular coagulation for better clinical outcome.
Objective: To clarify current situation related to preventive measures for COVID-19 infection among pregnant women and their family.
Methods: November 2020, questionnaire survey was attempted. The subjects were pregnant women visited to the perinatal center and primary obstetric facilities for regular pregnant checkup.
Results: 316 answers were analyzed. 52% of them acknowledged the fact that most frequent route was domestic infection. Face mask was used in 100% of pregnant women and their partner, and enforcement of hand wash in 98% of them both period of emergency status（April-May 2020）and after unbrick of emergency（July 2020）. However, face mask was used only 6% in their home. In pregnant women, self-staying home was performed 89% of them during emergency status and 72% after unbrick of emergency（p<0.01）. Disinfection in the home was performed 52% of them during emergency, and 43% after emergency（p=0.03）.
Conclusion: Pregnant women were likely to do preventive measures for COVID-19 infection during away from home, however unlikely to do them during staying home. Preventive measures in their home to avoid to receive infection from their family, especially continual face mask, should be performed. This information should widely be spread.