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A 35-year-old primigravid woman with chronic idiopathic intestinal pseudo-obstruction presented to our institution. Except for an enlarged fetal bladder, her pregnancy was almost uneventful until she developed pre-eclampsia requiring emergent cesarean section at 34 weeks gestation. After delivery, intractable uterine atony developed with blood loss reaching 3500 mL within 15 min. Following a B-Lynch suture, the bleeding attenuated but uterine atony persisted; lochia persisted for 3 months post-partum. The infant was diagnosed with megacystis microcolon intestinal hypoperistalsis syndrome after birth. The mother's clinical course and previous reports suggested that atonic bleeding was associated with the pathology of chronic idiopathic intestinal pseudo-obstruction; the infant's disease was considered to be maternal-related disease. Clinicians should be vigilant in pregnant patients with chronic idiopathic intestinal pseudo-obstruction especially with these complications.

Gastric cancer involving the placenta during pregnancy is rare; however, we present 1 such case in this report. A 31-year-old Japanese woman was referred at 26 weeks of gestation for the evaluation of a swollen left supraclavicular lymph node. Biopsy revealed poorly differentiated adenocarcinoma, and esophagogastroduodenoscopy with biopsy of the stomach confirmed the diagnosis of gastric cancer. Her epigastric and back pain became more pronounced and her general status worsened, and we performed a cesarean delivery at 29 weeks. Microscopic examination of the placental specimen revealed poorly differentiated adenocarcinoma cells diffused into the intervillous space. Postpartum chemotherapy consisted of S-1 plus oxaliplatin. Unfortunately, this treatment was ineffective, and the patient died 3 months after delivery. The infant did well, without clinical or laboratory manifestations of metastasis. In patients with advanced gastric cancer during pregnancy, it is important to perform a microscopic examination of the placenta to evaluate for metastatic involvement.

Objective: To determine whether vaginal progesterone (VP) reduces the rate of preterm birth in pregnant women after abdominal trachelectomy (AT) for early-stage cervical cancer / Study Design: This is an interventional study with a historical cohort. For the interventional study participants who had singleton pregnancies after AT between October 2016 and September 2020, the administration of vaginal progesterone was started between 16+ and 19+6 weeks of gestation and discontinued at 34 weeks of gestation or at the time of delivery, rupture of membranes, or massive uterine bleeding. We investigated obstetric and neonatal outcomes among the study participants and compared them with outcomes of the historical control group participants, included women with singleton pregnancies after AT who were managed without VP at our institution between January 2007 and September 2016, using Fisher’s exact test and the Mann–Whitney U test The main outcomes were the gestational age at delivery and incidence of preterm birth before 37 weeks and 34 weeks of gestation. / Result: Twelve pregnancies in ten women were included in the VP group. In contrast, 19 pregnancies in 17 women were included in the historical control group. The incidence of preterm birth at

Objective: To investigate the outcome of pregnancies with small baby, including both small for gestational age (SGA) and late fetal growth restriction (FGR) fetuses, undergoing induction of labor (IOL) with Dinoprostone, Misoprostol or mechanical methods. Study design: Medline, Embase and Cochrane databases were searched. Inclusion criteria were non-anomalous singleton pregnancies complicated by the presence of a small fetus, defined as a fetus with estimated fetal weight (EFW) or abdominal circumference (AC) 3rd centile with normal cerebroplacental Dopplers). Quality assessment of each included study was performed using the Risk of Bias in Non-randomized Studies-of Interventions tool (ROBINS-I), while the GRADE methodology was used to assess the quality of the body of retrieved evidence. Meta-analyses of proportions and individual data random-effect logistic regression were used to analyze the data. Results: 12 studies (1711 pregnancies) were included. In the overall population of small fetuses, composite adverse intra-partum outcome occurred in 21.2 % (95 % CI 10.0−34.9) of pregnancies induced with Dinoprostone, 18.0 % (95 % CI 6.9−32.5) of those with Misoprostol and 11.6 % (95 % CI 5.5−19.3) of those undergoing IOL with mechanical methods. Cesarean section (CS) for non-reassuring fetal status (NRFS) was required in 18.1 % (95 % CI 9.9−28.3) of pregnancies induced with Dinoprostone, 9.4 % (95 % CI 1.4−22.0) of those with Misoprostol and 8.1 % (95 % CI 5.0−11.6) of those undergoing mechanical induction. Likewise, uterine tachysystole, was recorded on CTG in 13.8 % (95 % CI 6.9−22.3) of cases induced with Dinoprostone, 7.5 % (95 % CI 2.1−15.4) of those with Misoprostol and 3.8 % (95 % CI 0–4.4) of those induced with mechanical methods. Composite adverse perinatal outcome following delivery complicated 2.9 % (95 % CI 0.5−6.7) newborns after IOL with Dinoprostone, 0.6 % (95 % CI 0–2.5) with Misoprostol and 0.7 % (95 % CI 0–7.1) with mechanical methods. In pregnancies complicated by late FGR, adverse intrapartum outcome occurred in 25.3 % (95 % CI 18.8−32.5) of women undergoing IOL with Dinoprostone, compared to 7.4 % (95 % CI 3.9−11.7) of those with mechanical methods, while CS for NRFS was performed in 23.8 % (95 % CI 17.3−30.9) and 6.2 % (95 % CI 2.8−10.5) of the cases, respectively. Finally, in SGA fetuses, composite adverse intrapartum outcome complicated 8.4 % (95 % CI 4.6−13.0) of pregnancies induced with Dinoprostone, 18.6 % (95 % CI 13.1−25.2) of those with Misoprostol and 8.7 (95 % CI 2.5−17.5) of those undergoing mechanical IOL, while CS for NRF was performed in 8.4 % (95 % CI 4.6−13.0) of women induced with Dinoprostone, 18.6 % (95 % CI 13.1−25.2) of those with Misoprostol and 8.7 % (95 % CI 2.5−17.5) of those undergoing mechanical induction. Overall, the quality of the included studies was low and was downgraded due to considerable clinical and statistical heterogeneity. Conclusions: There is limited evidence on the optimal type of IOL in pregnancies with small fetuses. Mechanical methods seem to be associated with a lower occurrence of adverse intrapartum outcomes, but a direct comparison between different techniques could not be performed.

BackgroundMother-infant bonding is an emerging perinatal issue. While emergency cesarean deliveries are associated with a risk of bonding disorders, the mode of anesthesia used for emergency cesarean deliveries has never been studied in this context. We aimed to investigate the impact of administering general anesthesia and neuraxial anesthesia to women undergoing cesarean deliveries on mother-infant bonding.MethodsThis was a retrospective, propensity score-matched multivariable analysis of 457 patients who underwent emergency cesarean deliveries between February 2016 and January 2019 at a single teaching hospital in Japan. The Mother-Infant Bonding Scale (MIBS) scores at hospital discharge and the 1-month postpartum outpatient visit were evaluated in the general anesthesia and the neuraxial anesthesia groups. A high score on the MIBS indicates impaired mother-infant bonding.ResultsThe primary outcome was the MIBS score at hospital discharge in propensity score-matched women. After propensity score matching, the median [interquartile range (IQR)] MIBS scores were significantly higher in the general anesthesia group than those in the neuraxial anesthesia group at hospital discharge [2 (1–4) vs. 2 (0–2); P = 0.015] and at the 1-month postpartum outpatient visit [1 (1–3) vs. 1 (0–2); P = 0.046]. In linear regression analysis of matched populations, general anesthesia showed a significant and positive association with the MIBS scores at hospital discharge [beta coefficient 0.867 (95% confidence interval [CI] 0.147–1.59); P = 0.019] but not at the 1-month postpartum outpatient visit [0.455 (−0.134 to 1.044); P = 0.129].ConclusionGeneral anesthesia for emergency cesarean delivery is an independent risk factor associated with impaired mother-infant bonding.

Although fibrosis is one of the most prominent pathologic features of preeclamptic (PE) placentas, its mechanism remains largely unknown. Consistent with previous reports, we observed overexpression of collagen; actin, α2, smooth muscle, aorta; connective tissue growth factor; and fibronectin in PE placentas compared with control ones. To investigate the mechanism of fibrosis in PE placentas, placental fibroblasts were isolated from PE placentas or normal pregnancies at delivery. The expression of fibrosis-related factors in fibroblasts was evaluated by real-time RT-PCR, Western blotting, enzyme-linked immunosorbent assay, and gene microarrays. An in vitro collagen gel contraction assay was also performed. Fibroblasts isolated from PE placentas showed higher expression levels of fibrosis-related factors compared with those from control ones. Global gene expression profiling of PE fibroblasts was contrasted with that of control ones and indicated an intimate association with transforming growth factor-β1 (TGFB1) signaling. Furthermore, the PE fibroblasts expressed abundant phosphorylated SMAD family member 2 and showed higher expression levels of target genes of TGFB1 signaling compared with the control ones. The PE fibroblasts also had a greater ability to contract compared with the control ones. Contractility also depended on TGFB1 signaling. Our results suggest that TGFB1 signaling is activated in the fibroblasts in PE placentas and that these active fibroblasts contribute to fibrosis.

Objective We aimed to evaluate the outcomes of growth-restricted fetuses with absent end-diastolic velocity in the umbilical arteries (UA-AEDV), and investigate the relationship between Doppler flow velocity waveforms in the ductus venosus (DV) and the clinical features. Materials and methods This was a retrospective study of growth-restricted fetuses diagnosed with UA-AEDV delivered at our institution between 2013 and 2015. The time from diagnosis of UA-AEDV to delivery, postnatal survival, and developmental prognoses were the primary outcomes. The time lag between the occurrence of UA-AEDV and an abnormal increase in the DV pulsatility index (DV-PI) were investigated. We also examined the correlation between the DV-PI values immediately before birth and umbilical cord arterial pH at birth. Results The median gestational age at birth among the 18 subjects was 28+2 (24+0–34+6) weeks, and the observation period between the first detection of UA-AEDV and delivery ranged from 0 to 35 days with a median of 8 days. Among the 18 infants, 15 (83%) survived, among whom 2 were diagnosed with a developmental disability. Gestational age at delivery was significantly lower in the poor outcome group. A positive correlation (correlation coefficient, 0.68) was observed between the umbilical artery pH and the last measured DV-PI. Conclusion The time interval from initial detection of UA-AEDV to delivery is highly variable, and it is reasonable to manage these growth-restricted fetuses with UA-AEDV expectantly with careful surveillance for fetal well-being. Specifically, Doppler DV analysis is clinically valuable for their evaluation.

Trachelectomy was developed as a fertility-sparing surgery for early-stage cervical cancer in patients of childbearing age. The purpose of this study is to evaluate oncologic and obstetric outcomes and complications after abdominal trachelectomy. We began to perform abdominal trachelectomy in 2005. Our institutional review board approved this clinical study, and fully informed consent was obtained from each patient. The medical records of patients who underwent trachelectomy were retrospectively reviewed. We performed 151 abdominal trachelectomies (89 radical trachelectomies, 48 modified radical trachelectomies, and 14 simple trachelectomies). The median age of the patients was 33 years, and the median postoperative follow-up period was 61 months. Although one patient experienced recurrence at the preserved cervix, none died after treatment. A total of 61 patients attempted to conceive after trachelectomy, and 21 pregnancies were achieved in 15 women. Hence, the pregnancy rate among patients who attempted to conceive was 25%. Fifteen babies were delivered by cesarean section between gestational weeks 23 and 37. Six babies were delivered at term. Six cases of preterm premature rupture of the membranes occurred. Varices appeared around the uterovaginal anastomotic site in five patients. Our data indicate that the oncologic outcome was excellent but infertility treatment was necessary to achieve the majority of conceptions. Additionally, preterm premature rupture of the membranes and premature delivery were frequently observed. An improved pregnancy rate and prevention of complications during pregnancy are issues that should be addressed in future studies.

Although some cases describing delayed birth of the second twin have been published recently, delay of delivery beyond 36 weeks seems scarce. We report a case of delayed-interval delivery wherein prolongation of the second twin's delivery until term with a subsequent favorable infantile outcome. In this case, the stillbirth of the first twin occurred at 25 weeks' gestation. Prophylactic tocolysis was performed with ritodrine and magnesium sulfate, and a McDonald cerclage was performed 2 days after delivery of the first twin. Ampicillin and gentamicin were also administered for the purpose of prevention of intrauterine infection. No clinical sign of chorioamnionitis was found thereafter, and full term uneventful delivery was achieved. With this experience, we believe that delayed-interval delivery can be effective in prolonging gestation and should be an option if the first twin developed an extreme preterm delivery.

Pregnancy after mechanical valve replacement involves high risk. Maternal valve thrombosis and hemorrhagic complications are associated with lethal outcomes; therefore, strict anticoagulant therapy is needed. Our patient was 26-year-old primiparous woman. She had undergone aortic valve replacement with a mechanical valve at 4 years of age and had used warfarin 3 mg per day since then. Because of her desire for a baby, she stopped warfarin and conceived spontaneously. She was referred to our hospital. After being informed of her choices, unfractionated heparin (UFH) administration was started. She experienced mild heart failure with sacroiliitis, bacteremia, and hematuria during pregnancy. She delivered her newborn at 37 weeks. Blood loss at delivery was 220 g. Administration of UFH was restarted 4 h after delivery and 3 mg of warfarin was administered from postpartum day (PPD) 6. Hemostatic suturing was required for vaginal bleeding on PPD7. A therapeutic dose of warfarin was achieved on PPD9. Although warfarin use is recommended as anticoagulant therapy for pregnant woman with mechanical valves, the safety and efficacy of UFH have not yet been clarified because of its limited use. More cases are needed to clarify this.

Introduction. Although nonabsorbable woven tape has been widely used for cervical cerclage, technical difficulties that can occur with an effaced cervix because of the thickness of the tape, and the risks of local infection are two major concerns. This study investigated perinatal outcomes of pregnancies involving an emergency cervical cerclage using absorbable monofilament polydioxanone sutures, which is a narrow thread and protects against bacterial infection. Materials and Methods. We performed a chart review of patients who underwent emergency McDonald cerclage with polydioxanone sutures at our institution between 2007 and 2015. Gestational age at delivery, duration between cerclage and delivery, and neonatal prognosis were evaluated as primary outcomes. Results. Among the 23 patients (18 singleton and five twin pregnancies) evaluated, ultrasound-indicated (progressive cervical length shortening) were eight (35%) and physical examination-indicated (fetal membranes that prolapsed into the vagina or dilated cervix) were 15 patients (65%). The median gestational age at cerclage was 22+3 weeks (range, 17+5 to 25+3 weeks). Postoperative spontaneous abortion occurred in only one patient. The median gestational age at delivery was 32+5 weeks (range, 20+5 to 40+6 weeks). Extremely preterm delivery before 28 weeks of gestation occurred in four (17%) cases. Full-term delivery was achieved in 10 (42%) cases. The duration between cerclage and delivery ranged from 5 to 136 days (median, 77 days). Except for one case of spontaneous abortion, all newborns survived till hospital discharge. Conclusions. Although our series included some patients at high risk for spontaneous abortion and preterm delivery, satisfactory prolongation and favorable neonatal outcomes were achieved for most patients by using absorbable monofilament sutures, thus suggesting the efficacy of this type of suture for emergency cervical cerclage.

Objectives Although the efficacy of thoracoamniotic shunting (TAS) for fetal hydrothorax is well-recognized, the coexistence of hydrops fetalis is still a clinical challenge. The preoperative determinants of shunting efficacy are not fully understood. In this study, we aimed to investigate the perinatal and postnatal outcomes of hydrops fetalis with pleural effusion treated by TAS using a double-basket catheter, and to discuss the preoperative factors predictive of patients who will benefit from TAS. Study design We conducted a retrospective study in hydropic fetuses with pleural effusion treated by TAS between 2007 and 2015. We extracted information regarding postnatal survival and pretherapeutic sonographic findings, including skin-edema thickness, pleural-effusion pocket size, and Doppler readings. Results Twelve subjects underwent TAS at a median gestational age of 29+5 weeks (range, 25+5–33+2 weeks). Skin edema disappeared or regressed in 7. Three experienced early neonatal death and the other 9 ultimately survived after a live birth at a median gestational age of 33+4 weeks (range, 29+1–38+2 weeks). All surviving children, except for 1, had a pretherapeutic pleural-effusion pocket greater than the precordial-edema thickness. All 3 children that died had precordial-edema thickness equal to or greater than the size of the pleural-effusion pocket. Conclusions We achieved a high survival rate (75%) using the double-basket technique. A greater pretherapeutic width of skin edema compared with the pleural-effusion pocket is possibly suggestive of a treatment-resistant condition and subsequent poor postnatal outcome.

We aimed to investigate whether the lung-to-thorax transverse area ratio (LTR) immediately before birth is of diagnostic value for the prediction of postnatal short-term outcomes in cases of isolated left-sided congenital diaphragmatic hernia (CDH). We retrospectively reviewed the cases of fetal isolated left-sided CDH managed at our institution between April 2008 and July 2016. We divided the patients into two groups based on LTR immediately before birth, using a cut-off value of 0.08. We compared the proportions of subjects within the two groups who survived until discharge using Fisher's exact test. Further, using Spearman's rank correlation, we assessed whether LTR was correlated with length of stay, duration of mechanical ventilation, and supplemental oxygen. Twenty-nine subjects were included (five with LTR 0.08, and 24 with LTR ≥ 0.08). The proportion of subjects surviving until discharge was 40% (2/5) for patients with LTR 0.08, as compared with 96% (23/24) for those with LTR ≥ 0.08. LTR measured immediately before birth was negatively correlated with the postnatal length of stay (Spearman's rank correlation coefficient, rs = -0.486), and the duration of supplemental oxygen (rs = -0.537). Further, the duration of mechanical ventilation was longer in patients with a lower LTR value. LTR immediately before birth is useful for the prediction of postnatal short-term outcomes in fetuses with isolated left-sided CDH. In particular, patients with prenatal LTR value less than 0.08 are at increased risk of postnatal death.

Aim Fetal borderline ventriculomegaly represents a frequent dilemma in perinatal management. The present study aimed to evaluate the clinical significance of fetal borderline ventriculomegaly in a low-risk Japanese population and to identify the risk factors for associated anomalies. Methods Data of cases of fetal borderline ventriculomegaly detected at 26−28 weeks of gestation by routine ultrasonographic screening of low-risk singleton pregnancies between 2006 and 2012 were retrospectively collected. Ventricular width, in utero progression, associated anomalies, chromosomal abnormalities, and perinatal and postnatal outcomes were assessed. The ventricular width, in utero progression and other perinatal characteristics were compared between the isolated and non-isolated groups. Results Among the total 6020 singleton low-risk pregnancies, we noted that 42 had borderline ventriculomegaly. Six (14%) of these cases had other defects by subsequent detailed examination. Ventriculomegaly resolved or regressed in 35 (83%) and progressed in four (10%) cases, of which three were associated with other anomalies. The median ventricular width was 12.8 mm (range, 10.0–14.7) in the six non-isolated cases and 10.5 mm (range, 10.0–13.3) in the 36 isolated cases; the differences were statistically significant. A ventricular width of 12 mm or more and in utero progression were more frequently observed in non-isolated cases than in isolated cases. Conclusion Fetal borderline ventriculomegaly frequently resolves in utero. A ventricular diameter of more than 12 mm and in utero progression are risk factors for additional anomalies. After the initial diagnosis of borderline ventriculomegaly, the pregnancy should be carefully followed up to determine whether the ventricle size is resolved, remains stable or increases.

Aim To assess the feasibility and practicality of expectant management for pregnancies with fetal growth restriction (FGR) at term without evidence of placental dysfunction. Methods We reviewed the records of pregnancies with an estimated fetal weight ≤ 1.5 SD below the mean at 37 weeks of gestation. We excluded elective cesarean deliveries and pregnancies that, at 37 weeks, were complicated by oligohydramnios, decreased fetal cerebroplacental ratio, or pregnancy-related hypertensive disorders. Prior to May 2013, we performed routine labor induction for FGR at term; after that time, we used routine expectant management. The rate of delivery by cesarean or instrumental assist and the rate of neonatal morbidity were compared between the groups. Results The gestational age at delivery and the neonatal birthweight were higher in the expectant management policy group (39+4 vs 38+1 weeks; 2405 vs 2205 g). The cesarean rate (7/77 vs 7/73) and the instrumental delivery rate (5/77 vs 6/73) did not differ. Neonatal hypoglycemia and hyperbilirubinemia were significantly less frequent (10/77 vs 21/73; 7/77 vs 20/73) in the expectant management policy group. Seven patients in the expectant management policy group underwent emergency cesarean delivery; five of these (71%) had required labor induction because of progression to oligohydramnios. Conclusions Expectant management policy for FGR at term can reduce neonatal morbidity without increasing maternal risk or the cesarean rate. Caution should be used, however, during labor if oligohydramnios develops during expectant management.

In this report, we present a case of twin-twin transfusion syndrome in the presence of the thick dividing wall of each sac in dichorionic twins. The dichorionic diagnosis was based on the presence of the lambda sign at the first-trimester ultrasound evaluation. In addition to fetoscopic and pathological investigation, DNA typing confirmed that the twin set was monochorionic and monozygotic. This case illustrates that although extremely uncommon, the presence of lambda sign does not exclude monochorionic pregnancy.

Aim The objective of this study was to evaluate the significance of maternal toxoplasmosis, rubella, cytomegalovirus (CMV) and herpes simplex virus (TORCH) screening in cases of fetal growth restriction (FGR). Material and Methods The medical records of women carrying fetuses with FGR who underwent TORCH screening over a 10-year period were retrospectively reviewed for maternal and congenital TORCH infection. Women carrying fetuses with FGR routinely underwent serologic TORCH tests and systematic ultrasound evaluation for congenital abnormalities. If a congenital CMV infection was suspected, amniotic fluid, placenta or neonatal urine was used for CMV DNA detection by polymerase chain reaction. Results In 319 patients, no cases of maternal or congenital infection with toxoplasma, rubella, or herpes simplex virus were found. Conversely, six cases (1.8%) were diagnosed with congenital CMV infection, two of which had no structural abnormalities other than FGR. Conclusions A complete maternal TORCH screening for cases of FGR appears to be unnecessary. Although a maternal CMV test can be considered, the incidence of congenital CMV infection was found to be low in FGR cases.

Aim: The aim of this study was to evaluate the perinatal outcome of monochorionic diamniotic (MD) twin gestations managed from early gestation onward at a single center. Material and Methods: This was a retrospective single-cohort study, spanning 6 years, of 219 MD twin pregnancies who received prenatal care before 14 weeks of gestation and delivered at our center. The subjects were all under the same perinatal management protocol. The incidence of fetal or neonatal death, major neurological complications at 28 days of age, twin–twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence, and discordant birth was evaluated. Laser surgery was offered for TTTS at less than 26 weeks; however, selective feticide was not performed. Results: Pregnancy termination was selected in two cases. Miscarriage occurred in two (1%) of the cases and preterm delivery occurred in 91 (42%). In regard to perinatal outcome at 28 days of age, 195 (89%) women possessed two live infants and 205 (94%) possessed at least one live infant. The overall survival rate was 92% (403/438). The prevalence of TTTS was 17 cases (8%), seven of whom underwent laser surgery. Four cases of twin anemia-polycythemia sequence were diagnosed postnatally (2%); discordant birth was diagnosed in 24 (12%). Major neonatal neurological abnormalities were noted in six cases (2%). Conclusions: The incidence of perinatal complications in 219 sets of MD twins managed from early gestational age to the neonatal period in one perinatal center was demonstrated. The incidence of TTTS was 8%; the survival rate was 89% at 28 days of age.

Tomoyuki Yoshitomi, MD, Nobuhiro Hidaka, MD, PhD, Yasuo Yumoto, MD, PhD,Kotaro Fukushima, MD, PhD, Kiyomi Tsukimori, MD, PhD, Norio Wake, MD, PhDDepartment of Obstetrics and Gynecology, Graduate School of Medical Sciences,Kyushu University, Fukuoka, JapanReceived 23 October 2010; accepted 16 August 2011ABSTRACT: Pulmonary sequestration is defined asnonfunctional lung tissue that lacks communicationwith the bronchial tree and that is supplied by ananomalous systemic vessel. In comparatively rarecases, pulmonary sequestration may lead to hydro-thorax or hydrops fetalis, which is nearly universallyfatal. In this report, we describe a case of pulmonarysequestration with hydrops fetalis, which wassuccessfully treated by thoracoamniotic shunting. Asonographic Doppler study in this case suggestedthat the underlying mechanism of the hydropicchange in a fetus with extralobar pulmonary seques-tration may have differed from that in fetuses withprimary hydrothorax not associated with a structuralanomaly.

We have summarized the care management of pregnant women with complete atrioventricular block (CAVB) by reviewing data from the published literature as well as our own experience in 32 pregnancies. Obstetrical management of women with a permanent pacemaker implanted prior to conception has been sufficiently reported thus far, and the management of such patients is considered to be of low risk. Since CAVB usually does not cause any specific obstetrical problems during pregnancy, prepregnancy prophylactic placement of a permanent pacemaker is not indicated in all asymptomatic patients. However, when asymptomatic women without pacemakers become pregnant, there is a subset that ultimately develops heart failure during pregnancy. Therefore, close surveillance of pregnant patients with CAVB is warranted. The current increase in the use of permanent pacemakers in young women with symptomatic CAVB will certainly limit the need for intrapartum temporary pacing in patients who do not require a pacemaker before pregnancy. In fact, most women with CAVB, who do not require a permanent pacemaker before delivery, can be safely managed during labor without temporary pacing. However, the clinical symptoms and cardiac function of patients should be carefully followed after delivery, even when pregnancy and delivery are uneventful. (PACE 2011; 34:1161–1176)

Trisomy 16 mosaicism detected at midtrimester amniocentesis is rare and indicative of true fetal mosaicism. We report a case of mosaic trisomy 16 diagnosed by amniocentesis in which the sonographic findings included fetal pulmonary artery stenosis, a single umbilical artery, and early onset fetal growth restriction. The pregnancy was legally terminated. A review of previous reports suggests that abnormalities of outlet tracts are rarely encountered in fetuses with trisomy 16 mosaicism revealed via amniocentesis. © 2008 Wiley Periodicals, Inc. J Clin Ultrasound, 2009

Aims: To identify the clinical characteristics of fetal abdominal wall defects managed at a single institution and to provide information regarding the most likely clinical course of the affected fetuses. Methods: A retrospective review was conducted of 44 fetuses prenatally diagnosed with abdominal wall defects at Kyushu University Hospital between 1990 and 2006. Results: A total of 11 cases of gastroschisis and 33 cases of omphalocele were found. Preterm delivery was observed in eight of 11 fetuses (72.7%) with gastroschisis and in 14 of 27 fetuses (51.9%) with omphalocele who were not artificially aborted. Intrauterine growth restriction (IUGR) was identified in four of 11 fetuses (36.4%) with gastroschisis and in 13 of 27 fetuses (48.1%) with omphalocele who were not aborted artificially. There was a high rate of associated structural anomalies with omphalocele (26/33). Karyotypic abnormalities were observed in eight of 29 tested fetuses with omphalocele (27.6%). The prognosis for a chromosomally normal fetus with omphalocele appeared to largely depend on the associated structural abnormalities and the gestational age at delivery. Despite the frequent presence of IUGR, the outcome of neonates with gastroschisis was good. Conclusions: The possibility of preterm delivery and IUGR should be considered during the management of both omphalocele and gastroschisis. For the management of omphaloceles, chromosomal abnormalities and respiratory insufficiency after birth must also be considered. Timely prenatal diagnosis and the subsequent identification of associated anomalies will improve patient care and will enable clinicians to provide appropriate counseling about the expected course.

Background: Fetal urinary ascites is a condition that is rarely encountered. A review of previous reports suggests that the clinical course of the affected fetuses is highly variable. Methods: In this report, we describe a case in which urinary ascites was observed after vesicocentesis in a fetus with megacystis caused by posterior urethral valve. Results: The urinary ascites was transient, and the fetal outcome was good after a successful vesicoamniotic shunting operation. Conclusions: In utero bladder rupture or injury and the subsequent appearance of urinary ascites may be well tolerated by the fetus. Further, spontaneous resolution can be expected during fetal life; therefore, it appears to be preferable to opt for conservative management in fetuses with urinary ascites. Urinary ascites, particularly after vesicocentesis, should be observed with extreme caution.

We present a case of a pregnant woman with hereditary antithrombin III deficiency and deep vein thrombosis of the left lower extremity managed by perinatal unfractionated heparin injection with antithrombin III replacement as well as by intrapartum placement of a temporary inferior vena cava filter. A massive thrombus of the right common iliac vein occurred during the intrapartum period despite antithrombin III replacement. An inserted filter could prevent fatal pulmonary embolism in this patient. Hereditary antithrombin III deficiency increases risk of thrombosis during pregnancy. However, discussion on intrapartum management, when anticoagulants are contraindicated due to possible hemorrhage, is minimal. Our experience suggests that thrombosis can occur and develop during the intrapartum period in an antithrombin III deficient woman despite antithrombin III replacement. It may be advisable to manage deliveries with temporary inferior vena cava filters to prevent fatal pulmonary embolism.

Prenatal sonographic findings of lymphangiomas characteristically appear as thin-walled, multiseptate hypoechogenic masses. In our case, a retroperitoneal hypoechogenic mass was detected at 26 weeks of gestation by sonography. Serial sonographic examinations demonstrated multiple septations at 29 weeks of gestation, which in retrospect was a classical finding of retroperitoneal lymphangioma. MRI at 38 weeks of gestation revealed a multilocular mass, which was homogeneous, low on T1-weighted and high on T2-weighted images, in the left retroperitoneal cavity. These findings were compatible with the diagnosis of a lymphangioma. This case shows the change in characteristic imaging features, from a unilocular to multilocular pattern, of a lymphangioma with regard to gestational age. It is important to observe the size and extension of such tumors in order to determine the prospect for neonatal prognosis, as well as to make decisions on the delivery timing and style.

Although the role of interventional radiology in the field of obstetrical hemorrhage has been widely reported upon recently, the rate of procedure-related complications has not been fully determined. We present the case of a patient who developed an external iliac artery thrombosis, a rarely reported complication associated with prophylactic common iliac artery balloon occlusion (CIABO). After CIABO, we found that the dorsalis pedis artery of the right foot was weak and the foot was cold, despite the fact that the patient had no complaints. Computed tomography demonstrated a linear thrombus in the right external iliac artery. We managed the patient conservatively using a heparin drip without the need for thromboembolectomy. Our experience suggests that it is important to consider the risk of thrombosis formation after CIABO. Physical examination post-procedure is key to identifying this complication early.

In the recent years, the association between congenital intestinal atresia and umbilical cord ulceration has been demonstrated; however, this complication is rarely encountered. To the best of our knowledge, only 14 cases on this association have been published previously. We encountered a case of acute intrauterine hemorrhage from an umbilical cord ulcer in a fetus with duodenal atresia that resulted in early neonatal death. There is little information available on the association between congenital intestinal atresia and umbilical cord ulceration. Greater understanding may alter the obstetric management. Although the prevention of sudden perinatal death is considered to be difficult, frequent fetal monitoring and detailed ultrasonographic examinations may facilitate the early detection of this condition, thereby enabling the rescue of the affected fetuses. Further investigation is required to establish the management protocol.

We encountered a patient with a fetal cytomegalovirus infection manifesting as pancytopenia and thoracic hypoplasia. The fetal anemia was treated by transfusion via the umbilical cord, and did not progress after 22 weeks' gestation. The neutropenia resolved spontaneously, and only thrombocytopenia was persistent at birth. The severe thoracic hypoplasia led to pulmonary hypertension and required intensive postnatal respiratory management. Our experience suggests that pancytopenia is a possible manifestation in fetuses infected with cytomegalovirus. This may be transient, resolving spontaneously during fetal life; however, caution should be taken with blood counts, particularly platelet counts, after delivery. In addition, clinicians should carefully follow the thoracic volume in cytomegalovirus-infected fetuses and consider the possibility of postnatal severe respiratory insufficiency.

β-thalassaemia are inherited blood disorders, which are characterised by reduced or no production of haemoglobin beta chains resulting in varied phenotypes, with affected individuals ranging from s...

Although prenatal diagnosis of congenital diaphragmatic hernia is not a rare event, detection of intrathoracic kidney in association is extremely rare. We present the sonographic findings in such a case. The postnatal outcome after successful respiratory management and surgical repair was good.

BACKGROUND We aimed to investigate which factors in the clinical profile of mothers with idiopathic thrombocytopenic purpura (ITP) can predict neonatal risk of thrombocytopenia. METHODS Data was retrospectively collected from all pregnant women with ITP who presented to our institution between 2001 and 2013. Neonatal offspring of these women were classified into 2 groups based on the presence or absence of neonatal thrombocytopenia (platelet count

Objective: We aimed to investigate the clinical characteristics and postnatal outcomes of fetuses with congenital diaphragmatic hernia (CDH) and additional anomalies. Materials and methods: We reviewed the charts of fetuses with CDH managed between 2005 and 2013. Patients were divided into complex and isolated groups based on the presence of additional anomalies. We analyzed the respective polyhydramnios, liver herniation, stomach position, lung to thorax transverse area ratio (LTR), and prognoses of the two groups. The survival rates of both groups were assessed based on the LTR as well as on stomach and liver positions. Results: CDH was diagnosed in 65 fetuses, and additional anomalies were found in 23. The incidences of liver herniation, polyhydramnios, and death were significantly higher, and LTR was significantly lower, in the complex group. The mortality rate of fetuses with a LTR Conclusions: The prognosis of complex CDH is poor. This may result from both the associated anomalies and the severity of CDH itself. Even in complex CDHs, intrathoracic liver and LTR values are useful in estimating postnatal outcome.

We present a case of fetal severe micrognathia in which successful airway stabilization was achieved by an ex utero intrapartum treatment procedure. In this case, it was anticipated that the infant would have a vulnerable airway at birth based on in utero sonographic findings, including an extremely hypoplastic jaw, worsening polyhydramnios and absence of stomach visualization. Early sonographic recognition was helpful in preparing the parents and physicians for the possibility of airway emergencies during the perinatal period. When a severely hypoplastic mandible accompanied by polyhydramnios and absent stomach visualization is noted on ultrasound, clinicians should consider the indication for ex utero intrapartum treatment. A multidisciplinary team with technically skilled medical providers should be coordinated to perform the procedure.

Persistent truncus arteriosus is an uncommon congenital cardiac anomaly. In most patients, this condition is not diagnosed prior to birth. We report a case in which this uncommon cardiac anomaly was diagnosed prenatally using wide-band Doppler imaging. When diagnosing fetal truncus arteriosus, sonologists should carefully search for the origin of the main pulmonary artery and for its 2 branches. Our experience suggests that wide-band Doppler imaging facilitates the prenatal diagnosis of truncus arteriosus. © 2008 Wiley Periodicals, Inc. J Clin Ultrasound, 2009

Fetal congenital cystic adenomatoid malformation (CCAM) can progress to nonimmune hydrops, and the mortality rate of CCAM with hydrops is reported to be nearly 100%. We describe three microcystic CCAM cases in which the fetal condition improved after maternal betamethasone therapy. The median gestational age at steroid administration was 23 5/7 weeks' gestation. The CCAM decreased in size in all cases. Our series showed a 100% hydrops resolution rate (2/2) and a 100% survival rate (3/3). Our experience suggests the efficacy of betamethasone treatment on fetuses with microcystic CCAM who have fluid collection or are at risk of developing hydrops.

Fetal borderline ventriculomegaly represents a frequent dilemma in perinatal management. The present study aimed to evaluate the clinical significance of fetal borderline ventriculomegaly in a low-risk Japanese population and to identify the risk factors for associated anomalies.Data of cases of fetal borderline ventriculomegaly detected at 26-28 weeks of gestation by routine ultrasonographic screening of low-risk singleton pregnancies between 2006 and 2012 were retrospectively collected. Ventricular width, in utero progression, associated anomalies, chromosomal abnormalities, and perinatal and postnatal outcomes were assessed. The ventricular width, in utero progression and other perinatal characteristics were compared between the isolated and non-isolated groups.Among the total 6020 singleton low-risk pregnancies, we noted that 42 had borderline ventriculomegaly. Six (14%) of these cases had other defects by subsequent detailed examination. Ventriculomegaly resolved or regressed in 35 (83%) and progressed in four (10%) cases, of which three were associated with other anomalies. The median ventricular width was 12.8 mm (range, 10.0-14.7) in the six non-isolated cases and 10.5 mm (range, 10.0-13.3) in the 36 isolated cases; the differences were statistically significant. A ventricular width of 12 mm or more and in utero progression were more frequently observed in non-isolated cases than in isolated cases.Fetal borderline ventriculomegaly frequently resolves in utero. A ventricular diameter of more than 12 mm and in utero progression are risk factors for additional anomalies. After the initial diagnosis of borderline ventriculomegaly, the pregnancy should be carefully followed up to determine whether the ventricle size is resolved, remains stable or increases.

We aimed to investigate whether the ratio of magnetic resonance imaging (MRI)-measured right lung volume (RLV) to ultrasonography-estimated bodyweight (RLV/BW) and observed-to-expected (o/e) RLV are of diagnostic value in predicting postnatal outcomes of left congenital diaphragmatic hernia (CDH).We included 32 CDH patients and 34 control subjects. Manually outlined fetal right lung areas on MRI were multiplied by the slice thickness and added to determine the entire volume. The association between RLV and RLV/BW with gestational age in the controls was examined using regression analysis. RLV/BW and o/e RLV were compared between surviving and non-surviving neonates with CDH.The expected fetal RLV was derived using the formula RLV (mm(3)) = 1.717 × (gestational weeks)(2.82). In the controls, RLV/BW was nearly constant during the third trimester. The 27 survivors with CDH had a median RLV/BW of 10.7 and a median o/e RLV of 60.0, whereas the five non-surviving neonates had a median RLV/BW of 4.3 and a median o/e RLV of 22.6; the differences were statistically significant.Assessment of fetal lungs by MRI volumetry is reliable for clinical use. RLV/BW and o/e RLV are potential predictors of postnatal outcomes of left CDH.

Congenital megalourethra is a rare urogenital malformation that is usually associated with lower urinary tract obstruction. In association with hypoplasia or deficiency of the corpus spongiosa and/or corpus cavernosum, a cystic dilatation of penile urethra is generally observed. We present a case of congenital megalourethra diagnosed by fetoscopy and ultrasonography at 17 weeks of gestation. Fetoscopic investigation demonstrated a balloon dilatation of the penis with a complete obstruction of the urethral meatus, which could distinguish a fusiform type congenital megalourethra from other disorders. Autopsy subsequent to the termination of pregnancy revealed a complete deficit of both corpus spongiosa and corpus cavernosum.

The aim of this study was to evaluate the perinatal outcome of monochorionic diamniotic (MD) twin gestations managed from early gestation onward at a single center.This was a retrospective single-cohort study, spanning 6 years, of 219 MD twin pregnancies who received prenatal care before 14 weeks of gestation and delivered at our center. The subjects were all under the same perinatal management protocol. The incidence of fetal or neonatal death, major neurological complications at 28 days of age, twin-twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence, and discordant birth was evaluated. Laser surgery was offered for TTTS at less than 26 weeks; however, selective feticide was not performed.Pregnancy termination was selected in two cases. Miscarriage occurred in two (1%) of the cases and preterm delivery occurred in 91 (42%). In regard to perinatal outcome at 28 days of age, 195 (89%) women possessed two live infants and 205 (94%) possessed at least one live infant. The overall survival rate was 92% (403/438). The prevalence of TTTS was 17 cases (8%), seven of whom underwent laser surgery. Four cases of twin anemia-polycythemia sequence were diagnosed postnatally (2%); discordant birth was diagnosed in 24 (12%). Major neonatal neurological abnormalities were noted in six cases (2%).The incidence of perinatal complications in 219 sets of MD twins managed from early gestational age to the neonatal period in one perinatal center was demonstrated. The incidence of TTTS was 8%; the survival rate was 89% at 28 days of age.

We present a case of an antenatally diagnosed congenital aneurysm of the left ventricle in which fetal cardiac contractility was evaluated by measuring the fetal isovolumetric contraction time (ICT). The workup of the fetus at 26 weeks' gestation led to the identification of a left ventricle aneurysm. Initially, the value of ICT of the left ventricle indicated adequate cardiac function. However, the fetal ICT was gradually prolonged, suspecting deteriorated cardiac contractility. Following an uncomplicated term delivery, a postnatal echocardiogram showed normal cardiac function. It is considered that because of the hypokinesis of the wall of the left ventricular aneurysm, the ICT did not fully predict cardiac function in this setting.

Incarceration of the gravid uterus is a rare but serious complication of pregnancy. The often required cesarean section is technically complicated, and preoperative planning is critical. An important initial surgical step is to lift the fundus out of the pelvis prior to creating the hysterotomy in order to facilitate adequate visualization of the pelvic anatomy.A 38-year-old primigravida with uterine incarceration from a large anterior leiomyoma underwent cesarean delivery at 29 weeks' gestation. In this case, a failure of the fundal height to increase was not appreciated as the anterior leiomyoma was palpated to represent the fundus. Intraoperatively the uterus was unable to be repositioned because of the leiomyoma. However, the surgery did proceed smoothly primarily due to the highly detailed images obtained on pelvic magnetic resonance imaging.Although uterine incarceration is rare, knowledge of this condition is important. Magnetic resonance imaging is a useful tool in that it enables the detailed evaluation of the pelvic anatomy in cases with suspected uterine incarceration.

Takayasu arteritis predominantly affects young women of reproductive age; therefore, the management of pregnancies with this disease is of great importance in clinical obstetrics. However, only a limited number of such cases have been reported in the English literature. Our aim in this study was to investigate the clinical features of pregnant women with Takayasu arteritis managed at a tertiary center in Japan. We conducted a retrospective study of 26 pregnancies (18 deliveries) in 10 women with Takayasu arteritis, who were managed at Kyushu University Hospital between 1996 and 2010. Data were collected retrospectively by reviewing the maternal medical records. The mean age of these patients at delivery was 29.3 ± 5.2 years. Six patients had lesions above and below the diaphragm, and four patients showed isolated supradiaphragmatic disease. Echocardiography was performed in all patients, and aortic regurgitation was observed in six. Five pregnancies resulted in spontaneous abortion, and three pregnancies were legally terminated at the patients’ request. The remaining 18 pregnancies resulted in live births. Of the 18 live babies born, growth restriction was observed in 2 babies. Superimposed pregnancy-induced hypertension was observed in two cases, and other severe complications, such as cerebral hemorrhage or cardiac failure did not occur. Although Takayasu arteritis is a potentially severe condition during pregnancy, successful pregnancy is possible if extreme caution is followed. Blood pressure should be strictly controlled and the delivery should be planned for favorable maternal and fetal outcomes.

Klippel-Trenaunay syndrome (KTS) is a rare congenital disease, and extensive cutaneous hemangiomas and abnormal venous vessels are characteristic. In our case, to manage her pregnancy with KTS, whole-body MRA was performed before delivery. A 29-year-old woman was referred at 28 weeks because of prominent vulvovaginal varicosities due to KTS. At 35 weeks, hypertrophy and multiple venous varicosities of her leg as well as massive vulvovaginal varicosities became prominent with a normal coagulation profile. Systematic MRAs revealed hemangiomas and varicosities in the right leg, the lower abdomen, and the pubic region, while no obvious AVM was detected around the bronchial tube and spine. We decided to deliver her baby by cesarean section at 37 weeks under general anesthesia, and a healthy baby was delivered. No blood transfusion was required. Prophylaxis against thrombosis was performed after the operation. She was discharged with her baby. Her vulvovaginal varicosities shrunk considerably one month later.

An abdominal aortic aneurysm is a rare disease in the paediatric population and is mainly caused by intrauterine infection, connective tissue diseases, such as Ehlers-Danlos syndrome and Marfan’s syndrome, and iatrogenic trauma due to umbilical artery catheterization. Although several cases have been reported in the English literature, they were rarely diagnosed prenatally. Vascular obstruction in utero is also believed to be the major cause of porencephaly. Recently, gene mutations have been reported as the cause of both the above-mentioned diseases. We present a prenatally diagnosed case of congenital abdominal aortic aneurysm with porencephaly.

Cardiac hypertrophy in the recipient fetus of twin-twin transfusion syndrome (TTTS) has been reported previously. We encountered an unusual set of monochorionic twins in which one twin had cardiac hypertrophy without TTTS while the other fetus had selective growth restriction. In this case, the diagnosis of selective growth restriction was made at 17 weeks of gestation, and right ventricular hypertrophy was identified in the co-twin at 21 weeks; however, no signs of TTTS were observed. At 29 weeks we concluded that the fetal circulation had deteriorated based on echocardiographic findings that included hydrops fetalis and an elevated preload index. Emergency cesarean section was performed. Our experience suggests that hypertrophic cardiomyopathy-like change in a monochorionic twin pregnancy may arise in settings outside of TTTS, including growth restriction of a co-twin. We believe our case will assist the discussion surrounding the etiology of cardiac hypertrophy in monochorionic twins.

Sacrococcygeal teratomas have been diagnosed prenatally on sonograms as masses of cystic, solid, or mixed echogenicity from the sacral area and protruding through the perineum or buttocks. However, a cystic sacrococcygeal teratoma may be misdiagnosed as an anterior sacral meningomyelocele, especially when presenting as a posterior cystic mass. We report a case in which three-dimensional power Doppler imaging was helpful for making a correct prenatal diagnosis of a type 1 cystic sacrococcygeal teratoma, which mimicked a meningomyelocele.

Aims: To investigate the association between the presence of liver herniation and perinatal course and outcome of fetal omphalocele. Methods: Cases of fetal omphalocele managed at our hospital between 1990 and 2006 were retrospectively reviewed and grouped according to the location of the liver. Results: Thirty-three fetal omphalocele cases were diagnosed. The chromosomal status of 29 of 33 fetuses was determined. The rate of chromosomal abnormalities in cases with an extracorporeal liver was significantly lower (2/18) than in the intracorporeal group (6/11) (Ps0.028). In chromosomally normal cases, four with extracorporeal liver resulted in early neonatal death compared to none with intracorporeal liver. Five of the 21 chromosomally normal fetuses showed an abnormal volume of amniotic fluid. All five cases had extracorporeal liver and two of them resulted in neonatal death. Conclusions: Fetuses with an extracorporeal liver had a lower rate of chromosomal abnormalities than those in the intracorporeal liver group. However, in chromosomally normal cases, it appeared that extracorporeal livers might be associated with more life-threatening anomalies, amniotic fluid volume abnormalities, and a higher rate of mortality than in the group with an intracorporeal liver. Upon diagnosis of fetal omphalocele, a careful search for liver location should be conducted before counseling.