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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.

We successfully performed shunting for a fetus with a multilocular macrocystic lung mass with hydrops at 22 weeks' gestation. Complete resolution of hydrops was achieved; however, at 35 weeks' gestation, the fetus developed acute massive pleural effusion. Fetal ultrasound examination revealed that one end of the shunting tube had migrated downward in the thoracic cavity, which led to fluid draining from the lung cyst. The baby was delivered at term and was discharged following neonatal intensive care management. Intrathoracic displacement of the shunt can occur, followed by massive pleural effusion due to drainage of cystic fluid.

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.

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.

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.

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.

Observed/expected lung area to head circumference ratio (o/e LHR) and lung to thorax transverse area ratio (LTR) are the sonographic indicators of postnatal outcome in fetuses with congenital diaphragmatic hernia (CDH), and they are not influenced by gestational age. We aimed to evaluate the relationship between these two parameters in the same subjects with fetal left-sided CDH. Fetuses with left-sided CDH managed between 2005 and 2012 were included. Data of LTR and o/e LHR values measured on the same day prior to 33 weeks' gestation in target fetuses were retrospectively collected. The correlation between the two parameters was estimated using the Spearman's rank-correlation coefficient, and linear regression analysis was used to assess the relationship between them. Data on 61 measurements from 36 CDH fetuses were analyzed to obtain a Spearman's rank-correlation coefficient of 0.74 with the following linear equation: LTR = 0.002 × (o/e LHR) + 0.005. The determination coefficient of this linear equation was sufficiently high at 0.712, and the prediction accuracy obtained with this regression formula was considered satisfactory. A good linear correlation between the LTR and the o/e LHR was obtained, suggesting that we can translate the predictive parameters for each other. This information is expected to be useful to improve our understanding of different investigations focusing on LTR or o/e LHR as a predictor of postnatal outcome in CDH.

It is uncommon to diagnose fetal hepatic hemangioma during the antenatal period. We describe herein two patients with a giant hepatic hemangioma detected antenatally, both with perinatal complications. In Case 1, a fetal intra-abdominal mass, measuring 63 mm × 50 mm × 74 mm, was observed below the right lobe of the liver, and the presumptive antenatal diagnosis of hepatic hemangioma was made at 37 weeks of gestation. Antenatal imaging suggested an intratumoral hemorrhage, but postnatal clinical findings refuted this diagnosis. However, progressive thrombocytopenia and coagulopathy were noted just after birth, resulting in the diagnosis of Kasabach–Merritt syndrome. In Case 2, our ultrasound examination performed at 40 weeks of gestation revealed a mixed solid and cystic hepatic tumor, measuring 99 mm × 54 mm. Further, antenatal sonography revealed cardiomegaly, increased descending-aorta velocity, atrioventricular valvular regurgitation, and a dilated inferior vena cava, suggesting high-output cardiac insufficiency. Giant hepatic hemangiomas can lead to severe complications such as cardiac insufficiency and Kasabach–Merritt syndrome, and these complications may occur during the fetal or early neonatal period. Detailed prenatal evaluation using fetal imaging and cord-blood sampling is important to determine proper antenatal management of patients with giant hepatic hemangiomas and to allow for prompt postnatal treatment.

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 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.

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.

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.

We report a case of severe congenital diaphragmatic hernia accompanied by hydrops fetalis in a fetus conceived by a 33-year-old woman. Fetal magnetic resonance imaging performed at 27 weeks of gestation revealed herniation of the stomach, bowel, and left liver into the thoracic cavity, confirming severe left-sided CDH. Presence of hydrops fetalis at 32 weeks of gestation and its progression over the next 2 weeks was confirmed by fetal echography. Subsequently, fetal echocardiography revealed severe tricuspid regurgitation and reverse flow in the inferior vena cava at 33 weeks, with abnormal left and right ventricular Tei indices, extended left ventricular isovolumetric relaxation time, and increased preload index. Maternal hyperoxygenation (MHO) therapy was initiated at 35 weeks of gestation to increase pulmonary blood flow and promote venous return to the left ventricle. The hydrops was attenuated by MHO for 2 weeks, and fetal echocardiography demonstrated improved cardiac performance. A female infant was born at 37 weeks of gestation and underwent diaphragmatic repair shortly after birth. She survived surgery and was discharged at 4 months of age with no adverse sequelae. This case report suggests that MHO may be a potential therapy for severe congenital diaphragmatic hernia associated with hydrops fetalis.

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.

Hydrops fetalis in association with meconium peritonitis is a rare condition, and the mechanism underlying hydropic changes has not been fully recognized. We present a case of fetal meconium peritonitis with hydrops and coagulopathy. Clinically, the cause of fetal disseminated intravascular coagulation is considered to be a consequence of a systematic inflammatory response based on progressive, but mild fetal anemia without other apparent triggers, thrombocytopenia, elevated white blood cell count and serum C reactive-protein, hypoalbuminemia, and increased vascular permeability. The infant was born at 32 weeks of gestation and survived after postnatal multidisciplinary treatment. Our experience suggests that recognition of this rare condition will enable early diagnosis and better clinical management for fetuses with meconium peritonitis.

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.

Aim 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). Material and Methods 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. Results The expected fetal RLV was derived using the formula RLV (mm3) = 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. Conclusion 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.

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.

Absence of the ductus venosus (ADV) is a rare vascular anomaly; however, the advent of color Doppler sonography has facilitated detailed analysis of the fetal venous system, and prenatal diagnosis has been described in the recent literature. 1-16 The ductus venosus is a short vascular channel with a maximum diameter of 2 mm, which connects the intra-abdominal umbilical vein with the inferior vena cava (IVC). Because of its role as a sphincter in the isthmic portion, the ductus venosus regulates the proportion of umbilical vein return, thus protecting the fetus from excessive placental flow. Congenital absence of this narrow vessel leads to lack of this regulation, and subsequent volume overload can place a substantial burden on fetal myocardial performance with the risk of high-output cardiac failure. If the chronic volume overload is severe, cardiomegaly, polyhydramnios, and fluid accumulation such as pleural effusion, ascites, pericardial effusion, and skin edema may occur. Furthermore, ADV is associated with a high incidence of other anomalies. Because of its high incidence of associated anomalies and hydrops fetalis, fetuses with ADV have a high mortality rate. 1-3 The most frequently reported associated anomaly is congenital heart disease. No reports to date have associated ADV with a congenital abdominal wall defect. Here we describe the case of a fetus with omphalocele with extracorporeal liver in association with ADV, which was diagnosed prenatally. The fetus showed polyhydramnios, cardiomegaly, and unilateral pleural effusion as signs of cardiac impairment; however, this condition did not develop throughout the pregnancy, and the neonate survived.

Objective. We aimed to investigate whether the preload index of the inferior vena cava (PLI-IVC) is of diagnostic value in predicting hydropic changes in fetuses with Ebstein anomaly. Methods. Five cases of prenatally diagnosed Ebstein anomaly, which were managed at our institution between 1999 and 2008, were retrospectively reviewed. The PLI-IVC was calculated as the ratio between the reversed flow velocity from the right atrium and the forward velocity of the IVC. Results. The PLI-IVC was high in all the cases. In 2 cases, PLI-IVC values tended to increase gradually before hydropic changes were recognized. In the cases without hydrops, PLI-IVC values exhibited a nonlinear trend throughout gestation and did not show any apparent increase. Conclusions. The upward trend of the PLI-IVC rather than the maintenance of a high value can be considered a sign of cardiac failure. The blood flow pattern in the IVC should be carefully monitored in fetuses with Ebstein anomaly for the early identification of fetal impairment.

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.

Background The objectives in this report are to present data on echocardiographic imaging of fetuses with congenital structural heart diseases using wide-band Doppler and to discuss the practical application of this unique display modality in the field of fetal echocardiography. Methods We performed fetal echocardiography using wide-band Doppler technology and examined fetuses that were at various gestational ages between January 2004 and December 2007. Results Satisfactory blood flow signals could be obtained using the wide-band Doppler; this has increased the understanding of fetal cardiovascular hemodynamics. Conclusions Wide-band Doppler appears a color flow mapping method that can contribute toward better understanding of fetal cardiovascular hemodynamics; it is also expected to provide greater accuracy in fetal echocardiography.

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.

Background Advanced Dynamic Flow (ADF), the software loaded in Toshiba's Aplio series of ultrasound apparatus (Toshiba Medical Systems, Tokyo, Japan), is useful for the detailed examination of small vessels such as fetoplacental or umbilical cord vasculature because of its high resolution and low blooming. The purpose of this study was to investigate the accuracy with which the fetoplacental vasculature is depicted by three-dimensional ultrasonic angiography using ADF. Materials and Methods Approximately 100 placentas, umbilical cords and fetuses, with and without abnormalities, at various gestational ages were investigated over a period of 1 year. We performed freehand scanning with the sector-scanning bracket using a three-dimensional magnetic position sensor. Results The umbilical cord insertion, placental vasculature, and superficial anastomosis in a monochorionic placenta were clearly depicted. Fetal vessels such as those of the portal and hepatic venous system could be clearly reconstructed. Conclusion ADF is suitable for the accurate depiction of fetoplacental vasculature for the three-dimensional reconstruction of vessels. The technique of three-dimensional ultrasonic angiography using ADF is reliable for visualizing fetal, umbilical and placental vasculature.

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.

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.

Observed/expected lung area to head circumference ratio (o/e LHR) and lung to thorax transverse area ratio (LTR) are the sonographic indicators of postnatal outcome in fetuses with congenital diaphragmatic hernia (CDH), and they are not influenced by gestational age. We aimed to evaluate the relationship between these two parameters in the same subjects with fetal left-sided CDH. Fetuses with left-sided CDH managed between 2005 and 2012 were included. Data of LTR and o/e LHR values measured on the same day prior to 33 weeks' gestation in target fetuses were retrospectively collected. The correlation between the two parameters was estimated using the Spearman's rank-correlation coefficient, and linear regression analysis was used to assess the relationship between them. Data on 61 measurements from 36 CDH fetuses were analyzed to obtain a Spearman's rank-correlation coefficient of 0.74 with the following linear equation: LTR = 0.002 × (o/e LHR) + 0.005. The determination coefficient of this linear equation was sufficiently high at 0.712, and the prediction accuracy obtained with this regression formula was considered satisfactory. A good linear correlation between the LTR and the o/e LHR was obtained, suggesting that we can translate the predictive parameters for each other. This information is expected to be useful to improve our understanding of different investigations focusing on LTR or o/e LHR as a predictor of postnatal outcome in CDH.

A co-existing right congenital diaphragmatic hernia and omphalocele is rare. We present images of a fetus diagnosed with this rare combination of anomalies. Early neonatal death occurred immediately after full-term birth due to severe respiratory insufficiency. In this case, disturbance of chest wall development due to the omphalocele rather than the diaphragmatic hernia was considered as the main cause of lung hypoplasia. Our experience suggests that caution should be exercised for severe respiratory insufficiency in a neonate with an omphalocele and diaphragmatic hernia, even in the absence of an intra-thoracic liver, one of the indicators of poor outcome for congenital diaphragmatic hernia.

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.

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

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.

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.

however, a hiatal hernia is usually diagnosedafter symptoms present during infancy or childhood. To our knowledge, prenatalsonographic detection of a hiatal hernia in a fetus with asplenia syndrome has notbeen reported previously. Here we present our experience and describe the prenataldiagnosis of a congenital hiatal hernia in a fetus with asplenia syndrome.

Noncompaction of the ventricular myocardium (NCVM) is a rare form of cardiomyopathy with a poor prognosis, and it is characterized by prominent trabeculations and deep intertrabecular recesses. This disease is speculated to occur because of arrest of the normal myocardial development process during early embryogenesis. In symptomatic infants, cardiac transplantation may be the only current curative option. Although NCVM is a congenital disorder, there are only a few previous reports on its status during fetal life. Here we report a case of nonimmune hydrops fetalis caused by this rare disease; in this case, digoxin administration to the mother could improve the fetal hemodynamics, and evaluation of the circulatory state of the fetus by using the preload index of the inferior vena cava (PLI) was proven to be sensitive and useful.

Objective. The purpose of this report is to describe the in utero sonographic appearance of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) and examine the role of vesicocentesis. Methods. Two cases of the sonographic appearance of MMIHS were reviewed. We performed vesicocentesis in 2 fetuses with MMIHS and performed vesicoamniotic shunting in 1 of them. The sonographic findings after these procedures were compared with those before puncture. Results. In both cases, ultrasound examination showed a massively enlarged fetal bladder and bilateral hydroureteronephrosis with a normal amount of amniotic fluid. Fetal urinary sodium and chloride concentrations were within normal limits in both cases; this suggested normal fetal renal function. Generally, it is difficult to detect the dilated fetal bowel in MMIHS, probably because of the disturbance caused by a large bladder. In our cases, however, the ultrasound examination clearly showed fetal bowel dilatation after these procedures; this made the antenatal diagnosis of MMIHS more definite. Conclusions. The sonographic features after vesicocentesis may be helpful in confirming the antenatal diagnosis of MMIHS. Key words: fetal urinalysis; megacystis-microcolon-intestinal hypoperistalsis syndrome; sonographic appearance; vesicocentesis.