Journal of Japan Society of Perinatal and Neonatal Medicine
Online ISSN : 2435-4996
Print ISSN : 1348-964X
Current issue
Journal of Japan Society of Perinatal and Neonatal Medicine
Displaying 1-31 of 31 articles from this issue
Review
  • Masahiko Nakata
    2026Volume 62Issue 1 Pages 1-8
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     The Perinatal Committee of the Japan Society of Obstetrics and Gynecology(JSOG)has revised the conventional diagnostic criteria for fetal growth restriction(FGR)and established a new conceptual framework and diagnostic standards. This revision is driven by recent advancements in the evaluation of maternal and fetal blood flow for managing FGR. In Japan, where assessment has traditionally relied solely on estimated fetal weight(EFW), distinguishing between "small for gestational age"(SGA)fetuses─who are constitutionally small─and "fetal growth restriction"(FGR)─where growth is pathologically limited by various factors─will significantly improve perinatal management. Furthermore, the adoption of new EFW reference values facilitates a transition toward management using percentiles, which is the international standard. This review aims to serve as a catalyst for optimizing FGR management strategies in Japan.

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  • Hiroshi Kubota, Yoko Fukushima
    2026Volume 62Issue 1 Pages 9-19
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Retinopathy of prematurity(ROP)remains one of the leading causes of avoidable childhood blindness. In addition to prematurity at birth, poor systemic conditions following delivery contribute to the severity of the disease. Specifically, various methods of oxygen administration and respiratory management have been extensively studied to reduce the incidence of ROP.

     In the field of ophthalmology, the introduction of anti-vascular endothelial growth factor(anti-VEGF)agents in the 2010s(e.g., ranibizumab or aflibercept)has revolutionized treatment. These agents have become a first-line option alongside conventional retinal photocoagulation due to their superior therapeutic efficacy and the potential for reduced incidence of high myopia compared to laser treatment. It is presumed that the number of advanced cases progressing to retinal detachment has significantly decreased in Japan, owing to the combined impact of improved neonatal care and expanded therapeutic choices.

     However, this paradigm shift in treatment modalities has brought new, significant challenges to light. Unlike traditional retinal photocoagulation, anti-VEGF therapy carries the risk of interfering with normal retinal vascular development, as VEGF is essential for physiological angiogenesis. Recent clinical observations have highlighted the emergence of issues that were not prevalent in the era of photocoagulation, such as late-onset recurrence and sustained impairment of normal vascular progression, which necessitate lifelong monitoring. Furthermore, initiatives have begun to develop diagnostic support technologies(such as AI-based automated screening systems)in anticipation of a shortage of specialists proficient in ROP clinical practice. This section outlines the current status of ROP, incorporating the latest clinical findings regarding these complex long-term sequelae.

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  • Miho Watanabe
    2026Volume 62Issue 1 Pages 20-24
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Fetal therapy is a medical strategy aimed at improving survival and functional outcomes in fetuses with life-threatening or severe congenital conditions by intervening before birth. Over the past decades, advances in fetal physiology, imaging technologies, and medical device development have significantly contributed to the evolution of this field, with a clear trend toward minimally invasive approaches.

     Fetal therapy represents a typical example of translational research, in which clinical challenges drive basic and preclinical investigations that are subsequently translated into clinical applications. At the same time, it is supported by a strict ethical framework, as interventions involve both the fetus and a healthy pregnant mother. Therefore, fetal therapy is indicated only when maternal safety is ensured and a clear benefit to the fetus is expected.

     In Japan, fetal therapy has been gradually introduced and has achieved steady progress through clinical research and insurance coverage of selected procedures. However, several challenges remain, including limited case volumes, insufficient collaboration between academia and industry, the need for structured training systems, and the development of transition models that ensure continuity of care from the fetal period to adulthood.

     Fetal therapy should not be regarded solely as a technical intervention but rather as the starting point of lifelong medical care. Its future development will depend on the integration of technological innovation, evidence generation, multidisciplinary collaboration, and ethical considerations.

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Originals
  • Masaya Yamoto, Kaori Yamoto, Hiromu Miyake, Akiyoshi Nomura, Koji Fuku ...
    2026Volume 62Issue 1 Pages 25-32
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Intestinal hypoperistalsis, considered an allied disorder of Hirschsprung disease, remains diagnostically challenging. This retrospective study analyzed 21 patients treated between 1987 and 2024 to evaluate diagnostic processes, clinical outcomes, and propose a novel diagnostic approach. Diagnostic methods included imaging studies, acetylcholinesterase staining of rectal mucosa, full-thickness biopsy of the rectum, and full-thickness biopsy of the digestive tract. Additionally, five patients underwent molecular analysis including whole exome or whole genome sequencing. Histopathological evaluation resulted in definitive diagnoses of hypoganglionosis in three patients and immaturity of ganglia in one patient, while four cases were suspected hypoganglionosis. Among 13 patients with normal ganglia, four were diagnosed with chronic intestinal pseudo-obstruction(CIIP), three with megacystis microcolon intestinal hypoperistalsis syndrome(MMIHS), and six remained unclassified. Genetic analysis revealed ACTG2 mutation in one CIIP case, leading to reclassification as visceral myopathy, and ACTA2 mutation in one unclassified case, reclassified as multisystemic smooth muscle dysfunction syndrome(MSMDS). Most patients(n=18)required central venous nutrition, and 16 underwent gastrostomy and/or enterostomy. Mortality occurred in five patients(24%), primarily from liver or renal failure or sepsis. Associated systemic malformations, including VACTERL association and Prune-belly syndrome, were noted. These findings highlight the limitations of histopathology alone for accurate diagnosis. Incorporating early genetic analysis targeting smooth muscle- and neuron-related genes can enhance diagnostic precision, guide clinical management, avoid unnecessary surgeries, and enable appropriate genetic counseling. A diagnostic algorithm integrating both pathological assessment and genetic testing is proposed to improve diagnostic accuracy and facilitate personalized treatment in clinical practice.

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  • Tomoe Kodama, Minako Yokoyama, Yoshiro Tachizaki, Motoshi Kunii, Maki ...
    2026Volume 62Issue 1 Pages 33-38
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Objectives: To assess the vaccination status of the respiratory syncytial virus prefusion F protein-based(RSVpreF)vaccine at our hospital, to identify issues related to improving vaccination coverage, and to evaluate the safety of the RSVpreF vaccine.

     Methods: Pregnant women who delivered at our hospital between August 2024 and July 2025 were assigned to an RSVpreF-vaccinated group or an unvaccinated group, and their perinatal outcomes were compared. In addition, a questionnaire survey was administered to collect information on vaccination status and reasons for receiving or declining the vaccine.

     Results: Of the 284 pregnant women, 151(53.2%)received the RSVpreF. No statistically significant differences were observed in the perinatal outcomes between the vaccinated and unvaccinated groups. The results of the survey indicate that the most common reason for receiving the RSVpreF was a recommendation from a physician. In contrast, the most common reason for not receiving the vaccine was “high cost”.

     Discussion: The RSVpreF coverage rate at our hospital was moderately satisfactory. To further improve vaccine uptake, healthcare professionals must provide clear and comprehensive information regarding the benefits of the vaccine, and economic support measures, such as public subsidies from governmental authorities, are essential. Therefore, its inclusion in the routine vaccination schedule is anticipated.

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  • Kaoru Wakabayashi, Norikazu Watanabe, Akito Endo, Risako Yamaguchi, Ta ...
    2026Volume 62Issue 1 Pages 39-45
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Objective: To evaluate whether a pre-labor scoring system based on maternal- and pregnancy-related factors can predict the likelihood of emergency cesarean section during an attempted vaginal delivery.

     Methods: This retrospective study included singleton pregnancies at ≥ 36 weeks gestation, where vaginal delivery was attempted at our institution between July 2020 and December 2024. Clinical records up to December 2023 were reviewed to compare the factors between successful vaginal deliveries and emergency cesarean deliveries. A scoring system to predict the likelihood of emergency cesarean sections was developed based on pre-labor factors and validated using data from 2024 cases.

     Results: Among the 775 cases analyzed, 590(76.3%)had vaginal deliveries and 183(23.7%)underwent emergency cesarean delivery. Multivariate analysis identified maternal aged ≧ 40, nulliparity, male fetus, hypertensive disorder of pregnancy, hyperglycemic disorders, fetal growth restriction, maternal height of < 150 cm and infertility treatment as independent predictors. A scoring system assigning 1 point to each of the factors showed a positive correlation with emergency cesarean rates. This trend was confirmed by an additional 220 cases in 2024.

     Conclusion: A simple pre-labor score based on eight factors may help predict the risk of emergency cesarean section during attempted vaginal deliveries.

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  • Nahoko Shirato, Miwa Sakamoto, Aiko Sekine, Tatsuko Ishii, Takahiro Ya ...
    2026Volume 62Issue 1 Pages 46-54
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Newborn mass screening, which checks all newborns for potential congenital disorders, is expanding its target diseases as the Expanded Newborn Mass Screening Test. Therefore, we investigated the implementation status in Japan and the awareness of this test among obstetric medical institutions(obstetrics)and pediatricians involved in prenatal consultations for genetic medicine(pediatrics). In March 2024, a self-administered survey was conducted targeting 856 obstetrics facilities and 611 pediatricians. The expanded screening was performed at 77% of facilities(including planned implementation), with half of these achieving implementation rates of 90% or higher. 76% of facilities charged patients the full cost for the expanded screening, with the peak cost being ¥10, 000. Among facilities also using public funding, approximately 30% had public funding contributions of ¥5,000 or less. Implementation rates were higher when public funding was the sole source. Approximately 60% of obstetricians and over 70% of pediatricians desired public funding for the program. Regarding testing starting in the fetal period, 70% of obstetricians and 92% of pediatricians viewed it positively. Furthermore, when abnormalities were detected via expanded screening, 94% of both specialties felt genetic counseling was necessary.

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  • Atsushi Naitoh, Nobuyuki Katsumata, Yuuki Maebayashi, Tamao Shinohara, ...
    2026Volume 62Issue 1 Pages 55-62
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     In 2018, the Yamanashi Prefecture established a neonatal resuscitation training system in collaboration with the Comprehensive Perinatal Center, Nursing Association, and Medical Affairs Division. The program aims to ensure that all medical staff and emergency technicians at childbirth facilities across the prefecture are certified in Neonatal Cardiopulmonary Resuscitation. Between April 2018 and March 2024, the program hosted 29 A courses(361 participants), 26 B courses(183 participants), 81 S courses(431 participants), and 6 P courses(51 participants). Following the initiation of the project, the certification rates by profession increased as follows: obstetricians/gynecologists, 43% to 79% ; pediatricians, 51% to 56% ; midwives, 78% to 94% ; nurses, 39% to 68% ; and emergency medical technicians, 4% to 21% . The overall certification rate increased from 36% to 64% . Facility achievement levels were classified into four steps. Step 1 involved training and certifying personnel within the facility; Step 2 focused on developing in-house instructors; Step 3 involved the hosting of certification workshops at the facility; Step 4 involved achieving self-sufficiency in certification renewals within the facility. All eight delivery hospitals reached Step 4. Among the eight delivery clinics, all achieved Step 3, and four advanced to Step 4. Among the prefecture’s 10 fire headquarters facilities, nine achieved Step 1, five advanced to Step 2, and one reached Step 3. Establishing a prefectural neonatal resuscitation training system helped ensure equitable dissemination of neonatal resuscitation skills across different facilities and healthcare professions.

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  • Hiroki Araki, Satomi Arai, Momoka Nakarai, Rie Okawara, Taisuke Hayash ...
    2026Volume 62Issue 1 Pages 63-68
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     [Purpose]To examine the impact of the introduction of sutureless enterostomy for neonatal gastrointestinal perforation on stoma care for fragile newborns weighing less than 500g at birth and to identify key care points.

     [Method]We analyzed surgical procedures and clinical data for infants weighing less than 500g at birth who underwent stoma creation for gastrointestinal perforation between 2009 and 2021.

     [Results]During the study period, we cared for 20 cases(11 boys and 9 girls). Twelve cases were treated using the conventional method, and eight were treated using the sutureless method. Although there were no recorded differences in complications in the skin surrounding the stomal openings between the two groups, after the conventional method, skin barrier use for bowel diversion through the stoma was initiated 4.18 days(0-25 days) postoperatively, whereas after the sutureless method, skin barrier use began 24.5 days(13-56 days) postoperatively, these differences did not reach significant. After the sutureless method, Bowel diversion through the stoma was observed early postoperatively.

     [Discussion]In fragile patients, the stoma sutureless method requires time for local stoma stabilization after surgery. More careful stoma care is required.

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  • Yusuke Ishibashi, Chikako Morioka, Yusuke Noguchi, Eisuke Fukama, Mich ...
    2026Volume 62Issue 1 Pages 69-75
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Although the safety of epidural analgesia during labor has been well established, evidence regarding its impact on short-term neonatal outcomes in Japan remains limited. This study aimed to clarify the impact of epidural analgesia on short-term outcomes in term neonates.

     We retrospectively analyzed 582 term neonates for whom vaginal delivery was attempted at our institution between January and December 2024. Of 959 total births, cases involving intrauterine fetal death, elective Cesarean section, preterm or post-term delivery, multiple pregnancy, or expedited delivery due to non-reassuring fetal status(NRFS)already present at hospital admission were excluded. Among the eligible cases, 255 mothers received epidural analgesia and 327 did not. The outcomes of this study were defined as delivery outcomes including instrumental delivery, emergency Cesarean section, NRFS, as well as short-term neonatal outcomes including 1- and 5-min Apgar scores, umbilical arterial blood pH(UApH), the need for resuscitation, and neonatal admission. Statistical analyses were conducted using nonparametric tests and logistic regression.

     The epidural group showed a significantly higher incidence of instrumental delivery and NRFS, while the rate of emergency Cesarean section did not differ between the groups. The median UApH was slightly lower in the epidural group(7.28 vs. 7.30), but no significant differences were observed in other neonatal outcomes.

     Epidural analgesia was associated with an increased rate of instrumental delivery but did not adversely affect short-term neonatal outcomes. These findings suggest that epidural analgesia can be safely implemented in term deliveries with appropriate intrapartum management and neonatal care.

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  • Hiroyuki Yoshio, Isamu Hokuto, Moe Yorozu, Yoko Tateishi
    2026Volume 62Issue 1 Pages 76-84
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     We examined the incidence of hypoglycemia in low risk neonates born in a breastfeeding promotion facility and the related issues of supplementation. A total of 424 term neonates without any abnormalities at birth, who were all at rooming-in with their mothers soon after birth, were enrolled and their blood glucose levels were measured after 24 hours of life around their minimum weight dates. Hypoglycemia was diagnosed as less than 45 mg/dL and 43 cases(10.1%)were found based on this definition. The mean blood glucose level ±SD was 39.8 ±4.35 mg/dL(26〜44), and all cases were asymptomatic except for one with transient tremor. The most common onset was, with 25 cases(58.1%), on day2(48〜72 hours after birth), and in 33 cases(76.7%), the minimum blood glucose level and the minimum weight date were the same. Logistic regression analysis revealed that hypoglycemia was not associated with maximum percent weight loss from birth weight but associated with early term, cesarean section and supplementation of sugar water(5% glucose solution)alone for 24 hours just before the minimum blood glucose levels, and their ORs(95% CI)at full term, vaginal delivery and breastfeeding with expressed milk were 3.51(1.60-7.70), 3.01(1.23-7.35)and 4.56(2.06-10.10), respectively. Early term infants, especially born by cesarean section, around the time of minimum weight when breast milk production is not sufficient, have a more increased risk of hypoglycemia than term infants born by vaginal delivery. It is suggested that in order to reduce the risk, it is useful to use a combination of expressed breast milk as much as possible or appropriate formula milk.

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  • Yumiko Suzuki, Takao Kobayashi, Sota Iwatani, Aika Matsushima, Toshihi ...
    2026Volume 62Issue 1 Pages 85-92
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Congenital diaphragmatic hernia(CDH)is a life-threatening condition with severe pulmonary and circulatory failure due to pulmonary hypoplasia. Although advances in prenatal diagnosis allowed for early planned intensive care, leading to improved survival rates in CDH, undiagnosed cases continue to be observed in clinical settings. Here, we investigated the prenatal diagnosis rate of isolated CDH cases managed at our center from January 2015 to December 2024 and described their clinical characteristics. In this retrospective single-center cohort study, among 34 newborns with isolated CDH, 3 cases(9%)had not been diagnosed prenatally. All undiagnosed cases were outborn and presented with right-sided CDH. In several of these cases, the clinical course of respiratory failure was notable for its delayed onset and exacerbation during manual ventilation. Given the limitation of prenatal diagnosis, CDH should be considered as a potential cause of postnatal respiratory failure.

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Case reports
  • Fumiya Nomura, Tomomi Shiga, Koyuki Uemura, Miwa Masuda, Kana Hayashi, ...
    2026Volume 62Issue 1 Pages 93-98
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Uterine prolapse during pregnancy is rare, requiring careful perinatal management to minimize the risk of complications during pregnancy and delivery. Here, we describe our experience with a pregnancy complicated by uterine prolapse associated with spina bifida and cervical elongation.

     The patient was 35 years old, with 1 pregnancy, 0 deliveries, and conceived by spontaneous conception. She had congenital spina bifida. Pelvic organ prolapse quantification(POP-Q)stageIV uterine prolapse was observed since early pregnancy and was managed with a ring pessary. At 12 weeks of gestation, the ring pessary became difficult to manage, and the patient was managed with FemiCushion female prolapse support. After 32 weeks of gestation, cervical lengthening up to 13 cm was observed and it no longer returned to the vagina. However, with continued use of the FemiCushion, no pregnancy complications were observed. Due to fetal dysfunction, a cesarean section was performed under general anesthesia at 41 weeks 0 days of gestation. The postoperative course was uneventful, but the POP-Q stageIV uterine prolapse remained.

     Severe uterine prolapse during pregnancy is rare, and no methods for perinatal management have been established. The FemiCushion may become one of the new management options for pregnancies complicated by uterine prolapse.

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  • Yuki Noboribayashi, Hiroki Kakita, Nami Nakamura, Yukinobu Nomura, Mar ...
    2026Volume 62Issue 1 Pages 99-103
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Currently, there are no established guidelines regarding the use of continuous transcutaneous oxygen saturation(SpO₂)monitoring in healthy newborns. However, the Japan Society for Neonatal Health and Development has proposed SpO₂-based screening for critical congenital heart disease. At our institution, we routinely conduct 24-hour SpO₂ monitoring in healthy newborns. Here, we report two cases of congenital chylothorax that were diagnosed based on histogram analysis derived from continuous SpO₂ monitoring.

     Case 1: A male infant was born by cesarean section at 38 weeks and 0 days of gestation with a birth weight of 3, 126 g. His Apgar scores were 9 at 1 minute and 9 at 5 minutes, and his initial respiratory status was normal. However, from day of life(DOL)4, the proportion of time with SpO₂ ≥96% began to decline gradually. On DOL 7, he was admitted to the neonatal intensive care unit(NICU), where congenital chylothorax was diagnosed. He was managed with a medium-chain triglyceride(MCT)formula and supplemental oxygen. His respiratory condition improved, and he was discharged on DOL 31.

     Case 2: A female infant was born by cesarean section at 36 weeks and 5 days of gestation, weighing 2, 666 g at birth. She received brief continuous positive airway pressure(CPAP)support but remained clinically stable. On DOL 5, the proportion of time with SpO₂ ≥96% decreased to 26% . She was subsequently admitted to the NICU and diagnosed with congenital chylothorax. Management with MCT formula and oxygen therapy led to clinical improvement, and she was discharged on DOL 19.

     Congenital chylothorax, which can be exacerbated by feeding, is a disorder that may not present with an early postnatal decline in SpO₂. Our findings suggest that continuous SpO₂ monitoring with histogram evaluation could be effective for its detection.

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  • Ryota Yamaguchi, Kento Kato, Yuna Tanaka, Yohei Kaishi, Kazuya Yoshimo ...
    2026Volume 62Issue 1 Pages 104-110
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Myotonic dystrophy(MyD)is an autosomal dominant genetic disorder caused by a CCTG repeat expansion, which can present with conditions such as polyhydramnios. When MyD is suspected, taking a family history, conducting genetic testing, and providing genetic counseling are essential. This report presents two cases in which MyD was diagnosed following the administration of ritodrine for threatened preterm labor.

     Case 1: A 30-year-old woman, gravida 1, para 0. After initiating ritodrine treatment for threatened preterm labor and polyhydramnios, she exhibited an elevated CK level. At 36 weeks, she experienced complete rupture of membranes and delivered vaginally. However, the baby had no muscle tone and was stillborn due to severe asphyxia. MyD was suspected based on the mother’s family history. With a CCTG repeat expansion of over 200, MyD was diagnosed, and genetic counseling was provided.

     Case 2: A 33-year-old woman, gravida 1, para 0. After initiating ritodrine treatment for placenta previa and threatened preterm labor, she exhibited an elevated CK level. MyD was suspected due to her "hammer-like" facial appearance and grip myotonia. After consulting with neurology, she underwent a cesarean section at 37 weeks, and the baby had a favorable outcome. With a CCTG repeat expansion of over 300, MyD was diagnosed, and genetic counseling was provided.

     Genetic counseling for MyD plays a crucial role in ensuring a safer subsequent pregnancy and improving perinatal outcomes for the child.

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  • Kazuki Suzuki, Shinya Hirabuki, Akira Takanashi, Hirotaka Ueno, Ai Usu ...
    2026Volume 62Issue 1 Pages 111-115
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     The perinatal period is associated with an increased risk of developing invasive Group A Streptococcus(GAS)infections, with streptococcal toxic shock syndrome(STSS)being a leading cause of maternal mortality from perinatal infections in Japan. Here, we report a fatal case of STSS that originated from lactational mastitis 12 days after cesarean delivery. A 37-year-old primigravida underwent emergency cesarean at 38 weeks of gestation due to severe preeclampsia and peripartum cardiomyopathy. She was discharged on postoperative day 10 following an uneventful recovery. However, two days later, she was readmitted to our hospital with severe bilateral mastitis, hypotension, and renal dysfunction. Neither the uterus nor cesarean-section wound showed any signs of infection. Despite intensive treatment, including hemodynamic support, her condition rapidly deteriorated, with fulminant tissue destruction affecting both breasts and extremities. Surgical debridement was not possible due to her critical state, and she died of multiple organ failure on postpartum day 36. STSS is a life-threatening complication of invasive GAS infection. In postpartum cases, potential sites of infection include the uterus, vagina, cesarean-section wound, and, less commonly, the breast or other locations. Prompt diagnosis, antibiotic therapy, intensive supportive care, and timely surgical intervention are crucial for improving survival outcomes.

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  • Maiko Takazakura, Aya Toyofuku, Kenichi Nishimatsu, Tomo Yamagata, Tak ...
    2026Volume 62Issue 1 Pages 116-120
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Patients with congenital antithrombin(AT)deficiency have an extremely high risk for the onset of thrombosis, which is at least 50 times higher than that in healthy individuals, and are generally recommended to undergo anticoagulation therapy and AT replacement from an early stage of pregnancy. The patient was a 34-year-old primiparous woman. She had a history of using oral contraceptives and undergoing surgery, but no past medical history or family history of thrombosis. A blood test at 34 weeks of gestation for the management of preeclampsia revealed a marked decrease in AT activity to 45% . Since she had no past medical history or family history of AT deficiency, we suspected that she had acquired AT deficiency, and induced delivery while administering AT replacement and anticoagulation therapy. The AT activity at 2 months after delivery remained low(55%), and her mother’s AT activity was also low. Thus, as congenital AT deficiency was suspected, we performed a genetic test, which resulted in a diagnosis of Type 2 HBS(heparin binding site defect)AT deficiency. Type 2 HBS is known to have a markedly lower risk for the onset of thrombosis than other subtypes. In future pregnancies, management will be performed with AT concentrate supplementation alone, without anticoagulant therapy. Genetic tests should be performed proactively to stratify patients with suspected congenital AT deficiency according to risk for the onset thrombosis.

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  • Takahiro Mitani, Mizuho Kadooka, Tokumasa Suemitsu
    2026Volume 62Issue 1 Pages 121-127
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Spontaneous hepatic rupture during pregnancy is rare but life-threatening, with an incidence of 1 in 40,000 to 250,000 pregnancies. It is most commonly associated with HELLP syndrome(hemolysis, elevated liver enzymes, low platelet count)and hypertensive disorders of pregnancy(HDP). We report a case of hepatic rupture in the absence of HELLP syndrome and HDP.

     A 36-year-old nulliparous woman at 36 weeks and 4 days had normal blood pressure(135/86mmHg)and no symptoms. Routine laboratory tests revealed abnormal liver function: hemoglobin, 12.2g/dL; platelet count, 148, 000/μL; AST, 98U/L; LDH, 272U/L.

     At 37 weeks and 2 days, she developed sudden epigastric pain, lost consciousness, and was transported to our facility. On arrival, she was hypotensive(BP, 60/30mmHg)with pulse 97 bpm. Laboratory evaluations revealed hemoglobin, 8.0 g/dL; platelet count, 45, 000/μL; AST, 187 U/L; LDH, 330 U/L. Ultrasound confirmed intrauterine fetal demise. Contrast-enhanced CT revealed hepatic rupture with hemoperitoneum, leading to hemorrhagic shock.

     Emergency laparotomy revealed a 10-cm subcapsular hematoma in the right hepatic lobe with active bleeding. Perihepatic packing was performed, followed by selective transcatheter arterial embolization. On postoperative day 2, second laparotomy confirmed hemostasis. Although extensive hepatic infarction was observed, liver function gradually improved, and the patient was discharged on day 19 postoperatively.

     This case illustrates that hepatic rupture can occur, even in the absence of HDP or HELLP syndrome. Early recognition based on subtle clinical and laboratory findings, along with prompt surgical and interventional management, is essential for maternal survival in hepatic rupture.

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  • Shunsuke Nomura, Yukako Kawasaki, Mituhide Nagaoka, Satomi Inomata, Ke ...
    2026Volume 62Issue 1 Pages 128-133
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Malan syndrome is a rare overgrowth disorder caused by heterozygous point mutations or deletions in NFIX, first described in 2010. Fewer than 90 cases have been reported, and perinatal or early infantile features remain poorly characterized, except the observation that ~ 15% of affected infants are large for gestational age.

     We describe a male infant born at 39 weeks and 5 days with a birth weight of 2,870g(−0.6 standard deviation[SD]), length of 46.3 cm(−1.6 SD), and head circumference of 35.3cm(+1.5 SD). He exhibited a bell-shaped thorax, proximal joint contractures, and poor feeding, necessitating transfer to our neonatal unit. Brain magnetic resonance imaging revealed downward displacement of the cerebellar tonsils. Whole-exome sequencing of the proband and parents identified a point mutation in exon 2 of NFIX, confirming Malan syndrome. The cerebellar tonsillar herniation aligns with previous reports linking Malan syndrome and Chiari type I malformation. In conclusion, proximal joint contractures have not been described in Malan syndrome and, together with Chiari I malformation, may provide useful diagnostic insights for distinguishing this condition from related disorders.

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  • Masato Shishido, Takeo Mukai, Chiharu Irisa, Atsushi Ito, Yoshihiko Sh ...
    2026Volume 62Issue 1 Pages 134-140
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     This case presents a neonate diagnosed with left congenital diaphragmatic hernia(CDH)complicated by double outlet right ventricle(DORV)and pulmonary atresia(PA). CDH with DORV is an exceptionally rare condition with a poor prognosis. We developed a comprehensive prenatal management strategy, including potential administration of extracorporeal membrane oxygenation(ECMO), through repeated multidisciplinary conferences. After birth, we promptly initiated ECMO and performed CDH repair on the same day, leading to successful resuscitation and survival. This case highlights that strategic prenatal preparation and a multidisciplinary approach can directly contribute to survival even in CDH with complex congenital heart disease. Further accumulation of case series is warranted to establish standardized criteria for ECMO indication and management in CDH with severe cardiac anomalies.

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  • Tatsuhito Furui, Kota Umemura, Maino Kan, Masafumi Kuroyanagi, Jotaro ...
    2026Volume 62Issue 1 Pages 141-145
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     A 26-year-old primigravida(G1P0)was referred to our hospital at 7 weeks of gestation with pregnancy complicated by ovarian tumors. Transvaginal ultrasound revealed bilateral ovarian masses, and MRI at 10 weeks demonstrated mature cystic teratomas measuring 5cm on the right and 7cm on the left. Considering the risk of torsion, rupture, and labor obstruction, surgical intervention was deemed necessary. Both tumors were incarcerated in the pouch of Douglas, and to minimize uterine mobilization and reduce the impact on pregnancy, bilateral ovarian cystectomy was performed via vNOTES(vaginal natural orifice transluminal endoscopic surgery). The operation lasted 61 minutes with an estimated blood loss of 57mL. The postoperative course was uneventful, and the patient was discharged on postoperative day 3. The pregnancy progressed without complications, resulting in a term vaginal delivery. Reports of vNOTES during pregnancy are extremely rare; however, in cases with incarceration in the pouch of Douglas, this approach may minimize maternal-fetal impact and serve as a useful surgical option. With careful case selection, vNOTES may represent a potential treatment strategy for ovarian tumors complicating pregnancy.

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  • Momoka Yumoto, Rina Aoyama, Mariko Tamura, Ikuko Yamada, Risa Arai, Ta ...
    2026Volume 62Issue 1 Pages 146-150
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Acute respiratory distress syndrome(ARDS)is a form of permeability pulmonary edema that develops secondary to various underlying conditions. We report a case of severe ARDS of unknown etiology that occurred after an emergency cesarean section at 25 weeks of gestation, in which the mother was successfully saved.

     The patient was a 30-year-old woman, gravida 4, para 0, with three prior spontaneous miscarriages. The current pregnancy was achieved by frozen blastocyst transfer. She was of Indian nationality and obese, with a pre-pregnancy body mass index(BMI)of 27, but had no other comorbidities. At 25 weeks and 3 days of gestation, she was admitted due to bulging membranes, and an emergency cesarean section was performed following preterm labor.

     Immediately after surgery, she developed rapid-onset hypoxemia requiring endotracheal intubation. Chest radiography showed bilateral pulmonary infiltrates, and severe hypoxemia was confirmed with a PaO2/FiO2 ratio of 60, leading to the diagnosis of severe ARDS. Her condition improved promptly with mainly supportive and intensive management.

     Reports of ARDS developing in pregnant women without comorbidities, triggered solely by the surgical stress of cesarean delivery, are extremely rare. In this case, the surgical invasion itself was considered a potential precipitating factor for ARDS. Rapid recovery was achieved with prompt intensive care.

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  • Wakako Nakamura, Akihito Moroi, Akira Naomi
    2026Volume 62Issue 1 Pages 151-157
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Tubal abscesses during pregnancy are uncommon, but delayed diagnosis and treatment can lead to serious complications for both mother and fetus. We report a case of a woman at 13 weeks’ gestation who developed a tubal abscess that progressed to generalized peritonitis and ultimately resulted in miscarriage. The patient was a 31-year-old woman, at 13 weeks and 4 days gestation, she presented with right lower abdominal pain and was transferred from a local hospital with suspected ureteral calculi. Transabdominal ultrasound revealed a 15 mm cystic lesion in the right adnexa, and CT scan suggested abscess formation. As peritoneal irritation symptoms were localized, antimicrobial therapy was initiated first, with a plan for exploratory laparoscopy the following morning. The next morning, abdominal pain worsened, and emergency exploratory laparoscopy was performed. Purulent ascites and enlargement of the right fallopian tube were observed, and a right salpingectomy was performed. Postoperatively, rupture of membranes and labor contractions occurred, resulting in spontaneous stillbirth. Haemophilus influenzae was detected in vaginal discharge, the abscess, and placental scrapings. Acute abdominal conditions during pregnancy carry a significant risk of premature labor or miscarriage and warrant consideration for more urgent, aggressive early surgical intervention compared to non-pregnant patients.

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  • Ryo Inaoka, Yutaka Yasuda, Naruhito Asano, Takanari Arai
    2026Volume 62Issue 1 Pages 158-163
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Acute fatty liver of pregnancy(AFLP)is a life-threatening condition in late pregnancy. We report a case in which antithrombin III activity(AT III)fell marke/dLy before the Swansea criteria were fulfilled, enabling early diagnosis and timely delivery. A 35-year-old gravida 2, para 1 at 37+5 weeks was admitted for evaluation of fever. Thirty days before admission she developed rhinorrhea and cough; seven days prior she noted thirst, polydipsia, and polyuria. On admission she had febrile infection that improved with antimicrobial therapy. On hospital day 5, liver enzymes worsened; on day 6, only five Swansea items were positive. However, AT III activity was 35%. Given the prodromal symptoms and the marked AT III reduction, AFLP was diagnosed clinically and emergency cesarean delivery was performed. After delivery, symptoms, liver tests, and AT III normalized rapidly. Chest imaging identified pneumonia as the infectious focus. Postpartum screening of the newborn and metabolic evaluation of the mother revealed no lipid-metabolism disorders. The clinical course suggests that, on the background of physiologic late-pregnancy free fatty acid(FFA)mobilization, inflammatory stressors(e.g., infection or medication)promoted FFA release and reactive oxygen species(ROS), increasing mitochondrial burden and precipitating AFLP. Decline in AT III activity may precede bilirubin elevation and prolongation of prothrombin time(PT)or activated partial thromboplastin time(APTT), offering an earlier diagnostic window than cross-sectional application of the Swansea criteria. Attention to longitudinal trajectories of symptoms an/dLaboratory markers may facilitate earlier recognition and timely delivery.

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  • Yoshifumi Kojima, Takashi Shibata, Tatsuya Nakamura, Hiroki Kato, Taka ...
    2026Volume 62Issue 1 Pages 164-168
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Early-onset group B streptococcal(GBS)infection is associated with a high risk of neonatal morbidity and mortality. Guidelines recommend GBS screening within five weeks of delivery. We report a case of a term neonate who developed early-onset GBS infection despite negative maternal screening. The patient was a primigravida in her 30s with hypertensive disorders of pregnancy who underwent labor induction at 39 weeks. Eleven hours after premature rupture of membranes, the mother developed a fever of 38℃. Although her condition did not meet Lencki’s criteria, emergency cesarean section was performed because clinical chorioamnionitis was suspected. The infant was delivered four hours after the onset of maternal fever but died 11 hours later due to respiratory failure. GBS was isolated from the amniotic fluid and the infant’s blood. Autopsy findings confirmed GBS infection.

     Improving the sensitivity of GBS screening and recognizing fetal tachycardia as a diagnostic clue in suspected chorioamnionitis may facilitate earlier initiation of appropriate antibiotic therapy and improve outcomes.

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  • Kazuki Yokota, Shunsuke Fukazu, Kana Takahashi, Chisato Kasuya, Dai Ko ...
    2026Volume 62Issue 1 Pages 169-173
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     We encountered a case of placental abruption, where sexual intercourse was considered a contributing factor to its onset. A 33-year-old woman who was pregnant for the second time with one prior delivery developed severe hypertension early in her pregnancy. She started to take antihypertensive drugs, which kept her blood pressure stable. At 34 weeks of gestation, she experienced genital bleeding after sexual intercourse. The intercourse was not significantly different from previous experiences, and there was no direct external force applied to the abdomen. She stopped having sexual intercourse immediately after recognizing a small amount of genital bleeding, abdominal tightness, and abdominal pain. However, the abdominal symptoms persisted, and two hours later, she experienced heavy genital bleeding. Her blood pressure was 136/100mmHg, and she reported reduced fetal movements along with frequent abdominal tightness. Examination revealed a firm abdomen and persistent heavy genital bleeding, and abdominal ultrasound showed placental thickening. Suspecting placental abruption, an emergency cesarean section was performed on the same day. Couvelaire sign and a placental hematoma were observed.

     Although the patient had chronic hypertension, the course of events suggested that sexual intercourse might have contributed to the placental abruption. Pregnant women with risk factors for placental abruption, such as chronic hypertension, may be at increased risk of developing placental abruption following sexual intercourse. Therefore, appropriate counseling and caution should be provided to these patients.

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  • Anju Maeda, Misa Nunode, Seiichiro Nao, Nagisa Ishikawa, Hiroshi Ota, ...
    2026Volume 62Issue 1 Pages 174-177
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Uterine synechiae is a membranous or rope-like structure traversing the uterine cavity, often incidentally detected after the second-trimester by ultrasound. It is known that uterine synechiae is associated with perinatal complications such as preterm premature rupture of membranes(pPROM)or placental abruption. A 40-year-old woman(gravida 3, para 1)had undergone hysteroscopic myomectomy for a submucosal myoma. During 18 weeks of gestation, a growing intrauterine mass was detected. Ultrasound examination revealed a septum-like structure in the uterine cavity, which was covered by amniotic membrane and chorion, and no continuity with the placenta was observed. MRI was performed and confirmed the diagnosis of uterine synechiae. At 26 weeks of gestation, she developed pPROM without clinical signs of infection or placental abruption. Conservative management was adopted to prolong gestation. After that, oligohydramnios was observed, but the fetal growth was good and there was no increase in uterine contractions. At 35 weeks, a cesarean section was performed, and a 2,174 g male infant was delivered. Both maternal and neonatal courses were uneventful. The placental pathology showed no chorioamnionitis or funisitis. Uterine synechiae occurs at about 1% and is usually considered to have a good prognosis, but some cases have been reported that lead to PROM and placental abruption, so we must take care in careful perinatal management.

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  • Yasufumi Hoshimoto, Hyuga Matsumoto, Tomoki Nobumasa, Maaya Ono, Yukik ...
    2026Volume 62Issue 1 Pages 178-183
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     The effects of systemic lupus erythematosus(SLE)on the uterus are not well understood. We report a case of SLE-complicated pregnancy that resulted in intrauterine fetal death(FD), characterized by marked fibrosis and thinning of the myometrium, followed by massive genital bleeding necessitating hysterectomy. The patient was a 34-year-old, gravida 3 para 0. She had received medications including oral steroids for SLE. Pregnancy was achieved via in vitro fertilization and embryo transfer(IVF-ET), but FD occurred at 15 weeks gestation. Repeated cervical dilation and administration of uterotonic agents were attempted, but no uterine contractions were elicited and we could not attain delivery. Therefore, at 17 weeks gestation, a curettage was performed to remove the fetus, and the placenta was preserved. Five months after the procedure, massive genital bleeding occurred, necessitating an emergency hysterectomy. Histological examination of the excised uterus revealed severe myometrial atrophy with minimal smooth muscle and extensive fibrosis. Previous reports have occasionally noted uterine myometrial thinning and contractile dysfunction in pregnancies by SLE, suggesting a need for further research.

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  • Rurimi Kawamura, Misa Nunode, Atsushi Daimon, Yoko Nagayasu, Takumi Sa ...
    2026Volume 62Issue 1 Pages 184-189
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     Non-invasive prenatal genetic testing(NIPT)has high sensitivity and specificity for aneuploidy in chromosomes 13, 18, 21. However, false positive and false negative can occur. Therefore, when fetal structural anomalies are detected by ultrasound imaging, even if NIPT results are negative, we need to consider chromosomal or genetic disorders as a diagnosis. We report the case of a 25-year-old woman(gravida 1, para 0) who underwent a NIPT at a non-certified facility at 11 weeks of gestation, and the result showed no aneuploidy in chromosomes 13, 18, 21. At 19 weeks, she was introduced to our hospital after another hospital suspected fetal omphalocele. The fetal ultrasound revealed multiple anomalies: choroid plexus cyst, ventricular septal defect, omphalocele, and overlapping fingers. These results strongly indicated fetal chromosomal abnormality. Amniocentesis was performed and confirmed trisomy 18. The patient chose to terminate the pregnancy, the baby (a girl, 230g)was delivered at 21 weeks and 3 days of pregnancy. The fetal ultrasound can detect structural abnormalities and features suggestive of chromosomal or genetic disorder which not covered by NIPT. Therefore, it is possible to comprehensively assess the fetus, and provides essential information for diagnosis and supports informed decision-making.

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  • Eri Nagasaki, Taro Ikeda, Makoto Hashimoto, Shumpei Goto
    2026Volume 62Issue 1 Pages 190-194
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     The patient was a 9-day-old boy. Hydronephrosis was not noted during the fetal period. The patient was hospitalized at our facility for detailed testing and treatment due to feeding problems and poor weight gain. Diagnostic imaging performed at admission revealed a left megaureter. As intestinal pressure caused by the left megaureter was thought to be the cause of the feeding problems, emergency urinary drainage was performed when the patient was 10 days old. Although there was some resistance when inserting a 3Fr open-end ureteral catheter tube from the left ureteral orifice using cystoscopy, we could place the catheter in the left ureter. Based on intraoperative findings, the patient was diagnosed with left ureterovesical junction obstruction. Postoperative urinary excretion was good, and clear improvement was noted in the left megaureter. Following the surgery, the patient fed well and gained weight. On postoperative day 9, the left ureteral stent was removed, and there has been no recurrence of left hydronephrosis or hydroureter since then. In the neonatal period, most cases of emergency urinary drainage are managed by nephrostomy; however, for patients with obstructive megaureter, the less invasive option of transurethral ureteral stent placement is also considered to be viable.

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  • Sae Matsumoto, Nanayo Sasagasako, Yukiko Mikami, Masae Yamada, Aya Har ...
    2026Volume 62Issue 1 Pages 195-200
    Published: 2026
    Released on J-STAGE: May 10, 2026
    JOURNAL FREE ACCESS

     A 24-year-old primigravida woman was found to have a subchorionic hematoma at 14 weeks of gestation. She presented with watery vaginal discharge and was diagnosed with previable preterm premature rupture of membranes at 16 weeks. Expectant management was continued in the absence of infection or any abnormal uterine bleeding. Ultrasonography at 18 weeks revealed a massive subchorionic hematoma(Breus’ mole). The hematoma gradually regressed after 23 weeks and was undetectable by 27 weeks, with normalization of amniotic fluid volume after 24 weeks. She was readmitted due to threatened preterm labor at 28 weeks, and delivered vaginally at 30 weeks and 3 days of gestation. We hereby report the case in which a Breus’ mole complicated with PROM, identified in the second trimester, decreased in size with expectant management and resulted in the delivery of a live infant.

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