Max dD/Dt: A Novel Parameter to Assess Fetal Cardiac Contractility and a Substitute for Max dP/Dt.

Aortic pulse waveforms are composed of a forward wave from the heart and a reflection wave from the periphery. We focused on this forward wave and suggested a new parameter, the maximum slope of aortic pulse waveforms (max dD/dt), for fetal cardiac contractility. Max dD/dt was calculated from fetal aortic pulse waveforms recorded with an echo-tracking system. A normal range of max dD/dt was constructed in 105 healthy fetuses using linear regression analysis. Twenty-two fetuses with suspected fetal cardiac dysfunction were divided into normal and decreased max dD/dt groups, and their clinical parameters were compared. Max dD/dt of aortic pulse waveforms increased linearly with advancing gestational age (r = 0.93). The decreased max dD/dt was associated with abnormal cardiotocography findings and short- and long-term prognosis. In conclusion, max dD/dt calculated from the aortic pulse waveforms in fetuses can substitute for max dP/dt, an index of cardiac contractility in adults.

The outcomes and prognostic factors of fetal hydrothorax associated with trisomy 21.

OBJECTIVES: To determine the characteristics, outcomes, and prognostic factors of fetal hydrothorax (FHT) with trisomy 21. METHODS: A nationwide survey was conducted on FHT fetuses with trisomy 21 delivered after 22 weeks' gestation between January 2007 and December 2011 at perinatal centers. RESULTS: The 91 cases of FHT with trisomy 21 included 28 (30.8%) diagnosed in utero and 63 (69.2%) diagnosed after birth. The natural remission rate was 6.6% (6/91). Thoracoamniotic shunting was performed in 14.3% (13/91) of cases. The survival rates of the hydropic, nonhydropic, and total cases were 47.0% (31/66), 84.0% (21/25), and 57.1% (52/91), respectively. The crude odds ratio for death was 8.2 (p = 0.003) for fetuses diagnosed at 26-30 weeks of gestational age (vs ≥30 weeks), 5.9 (p = 0.003) for hydrops, 4.0 (p = 0.04) for bilateral pleural effusion, 0.68 (p = 0.42) for associated cardiovascular anomalies, and 2.1 (p = 0.26) for thoracoamniotic shunting (vs no fetal therapy). CONCLUSIONS: The prognosis of FHT with trisomy 21 was not very poor, but it was still worse than that of primary FHT. Hydrops, an early gestational age at the diagnosis and bilateral effusion, but not associated anomalies, were risk factors for death. Fetal therapy showed no survival benefit for FHT with trisomy 21. © 2017 John Wiley & Sons, Ltd.

The prognostic factors and outcomes of primary fetal hydrothorax with the effects of fetal intervention.

OBJECTIVES: This study aims to determine the prognostic factors and outcomes of primary fetal hydrothorax (FHT) and investigate the effects of fetal therapy. METHODS: A nationwide survey was conducted on fetuses with primary FHT delivered after 22 weeks of gestation between January 2007 and December 2011 at perinatal centers. RESULTS: Among the 287 cases of primary FHT, the survival rates for those with and without hydrops were 58.0% (113/195) and 97.8% (90/92), respectively. The survival rates in the no-therapy, thoracocentesis, and thoracoamniotic shunting (TAS) groups in the hydropic cases and the non-hydropic cases were 59.7% (40/67), 51.5% (35/68), and 63.3% (38/60) and 98.1% (53/54), 96.3% (26/27), and 100% (11/11), respectively. The crude relative risk for death was 2.1 (p = 0.005) for fetuses diagnosed at 26 to 30 weeks of gestational age (vs ≥30 weeks), 2.3 (p = 0.001) for both skin edema and ascites, and 3.1 (p = 0.02) for bilateral pleural effusion. TAS was associated with a significant risk reduction for death in hydropic cases [adjusted relative risk 0.61, p = 0.01 (vs no fetal therapy)]. CONCLUSIONS: Hydrops and an early gestational age at diagnosis (<30 weeks of gestation), skin edema with ascites, and bilateral effusion predicted a poor prognosis in primary FHT cases. TAS was associated with a higher survival rate. © 2016 John Wiley & Sons, Ltd.

Prenatal diagnosis of abnormal umbilical cord insertion: a rare case of furcate insertion.

Furcate insertion (FI) is an extremely rare abnormality of umbilical cord insertion. One of the complications associated with FI is hemorrhage from the umbilical vein at the site of FI of the umbilical cord, which can cause sudden intrauterine fetal death. Because of its rarity, no prenatal diagnosis of FI has been reported. A 31-year-old woman at 34 weeks' gestation was referred to us for suspected abnormal cord insertion. Ultrasonography showed normal fetal growth and amniotic fluid volume, with no fetal anomalies. Although the umbilical cord contained three vessels inserted at the center of the placenta, the umbilical vessels separated from the cord substance before their insertion to the placenta. Based on these findings, the patient was diagnosed with FI. During labor at 37 weeks' gestation, fetal heart rate was normal and a healthy neonate was delivered. At macroscopic examination, the umbilical cord was inserted in the middle of the placenta, and the placental parenchymal tissue just under the cord insertion was deficient and had been changed to white, elastic hard tissue. Pathological examination of the white tissue revealed fibrin deposition and focal infarction. Although FI is a very rare condition, prenatal diagnosis can be achieved through detailed color Doppler ultrasound studies. Therefore, taking precautions and keeping in mind the poor fetal outcome associated with FI are preferred.

Management of congenital diaphragmatic hernia with transposition of the great arteries.

Congenital diaphragmatic hernia (CDH) is a relatively rare malformation, but it has a high mortality rate. Its association with congenital heart disease lowers survival rate due to severe acidosis and desaturation caused by persistent pulmonary hypertension of the neonate. We describe herein a case of CDH with transposition of the great arteries and intact ventricular septum, in a patient who was prenatally diagnosed, managed with extracorporeal oxygenation (ECMO) support and successfully treated with both CDH repair and Jatene procedure, with no respiratory or circulatory sequelae. In conclusion, precise prenatal estimation is essential, and ECMO is a useful therapeutic option in these complex cases.

Neurological outcomes in Chiari type II malformations and their correlation to morphological findings and fetal heart rate patterns: a retrospective study.

BACKGROUND: Correlations among Chiari type II malformation (CMII) morphological findings, the proportion of fetal heart rate patterns corresponding to the quiet phase (QP), and neurological outcomes have yet to be investigated. FINDINGS: The correlations among the morphological findings (i.e., the degree of ventriculomegaly, myelomeningocele levels, and degree of cerebellar herniation), proportion of time spent in QP, and developmental quotients (DQs) were analyzed in 22 children. The proportion of time spent in QP was compared between children with poor neurological outcomes (n = 9) and those with good outcomes (n = 13). Pearson's correlations and the Mann-Whitney U-test were used to assess for statistical significance; P < 0.05 was considered statistically significant. No significant differences were observed between the DQs and morphological findings, but the DQs and the proportion of time spent in QP were significantly correlated (r = 0.287, P = 0.01). The proportion of time spent in QP was significantly different between children with poor outcomes and those with good outcomes (median, 11% [range, 0-32%] vs. 28% [range, 2-55%]; P = 0.006). CONCLUSIONS: The proportion of fetal heart rate patterns corresponding to the QP might be a useful predictor of neurological outcomes in 2-year-old children with CMII.

Rare fetal complications associated with placental mesenchymal dysplasia: a report of two cases.

Placental mesenchymal dysplasia (PMD) is a rare disease that may be difficult to distinguish from molar pregnancy. The disease is associated with major fetal complications, including Beckwith-Wiedemann syndrome, fetal growth restriction and intrauterine fetal death. Rarely, fetal hematological disorders and liver tumors also may occur. Two patients were referred to our hospital during their second trimesters because of suspected molar pregnancies. Fetal karyotyping and maternal serum human chorionic gonadotropin level determinations led to the PMD diagnoses. In one case, the maternal clinical course was normal, but the neonate suffered from disseminated intravascular coagulation and needed a platelet transfusion. In the second case, the PMD decreased during pregnancy, but a gradually increasing fetal liver tumor appeared. The tumor was diagnosed as mesenchymal hamartoma, based on ultrasound and magnetic resonance imaging studies. The neonate was delivered without cardiovascular compromise. Due to the difficulty of immediate surgical treatment, expectant management, with close follow-up, was chosen.

Doppler sonographic evaluation of arteriovenous shunt flow in a fetus with dural sinus malformation.

Dural sinus malformation (DSM) is a rare congenital malformation characterized by a dilated dural sinus pouch. We present a case of prenatally diagnosed DSM and propose a parameter to predict poor fetal outcome. Detailed ultrasonography at 26 weeks of our patient showed an intracranial cyst in the left posterior fossa. Color Doppler study indicated an arteriovenous shunt within the cyst with increased blood flow velocity. Based on these findings, fetal DSM with arteriovenous shunt was diagnosed. Because of fetal hydrops with high-output cardiac failure and maternal pregnancy-induced hypertension, labor was induced at 32 weeks and resulted in stillbirth. In conclusion, based on the present case, we can deduce that color Doppler study is useful for prenatal diagnosis of DSM with arteriovenous shunt and that a high-flow velocity to the cystic lesion is a possible predictor of hydropic change in such fetuses.

Clinical utility of augmentation index as a new parameter of peripheral circulation in human fetuses.

BACKGROUND: Augmentation index (AI) is calculated from the central arterial pressure or pulse waveform and known as a parameter to evaluate arterial vascular function in adults. Patients with deterioration of peripheral circulation will demonstrate increased AI values as well as those with cardiovascular risks. It is because increased AI is caused by the early timing of the reflection wave from the periphery. On the other hand, in fetuses, although arterial pressure waveforms are not available, pulse waveforms of fetal descending aorta are recordable by using an echo-tracking system. Therefore in this study we aimed to evaluate the utility of fetal AI calculated from aortic pulse waveforms for detecting the altered peripheral circulation in human fetuses. STUDY DESIGN: Fetal AI was calculated from pulse waveforms in the descending aorta using an echo-tracking system. In a cross-sectional study of 105 normal fetuses, the reference range was constructed using linear regression analysis. Retrospectively, 36 growth-restricted fetuses were divided into 2 subgroups, normal (n=21) and increased AIx (n=15), based on the 90th percentile value of normal fetuses. Clinical parameters were compared using Fisher's exact test or Mann-Whitney U test. RESULTS: Fetal AI decreased linearly with advancing gestational age (r(2)=0.820). The incidences of umbilical artery absent/reversed end-diastolic flow, brain-sparing effect, and oligohydramnios were significantly higher in the increased AI group than the normal AI group. CONCLUSION: Fetal AI has a possibility to detect deteriorated peripheral circulation in the fetal body as well as fetoplacental circulation.

Successful management of cervico-isthmic pregnancy delivered at term.

A 29-year-old woman was diagnosed with a cervico-isthmic pregnancy based on ultrasound findings at 8 weeks of gestation. At 30 weeks of gestation, placenta previa was confirmed. During cesarean section at 37 weeks, the placenta did not spontaneously detach from the uterus; therefore, we decided to leave it in the uterus to avoid major hemorrhage. Blood loss was 775 mL and a healthy infant was delivered. After the operation, weekly methotrexate injection was initiated. Shortly after the eighth course of injection, massive vaginal bleeding suddenly occurred and bilateral uterine artery embolization was performed to control it. After the procedure, the retained placental tissue was removed and the patient was discharged with good general condition. Although a cervico-isthmic pregnancy constitutes a high-risk pregnancy, fertility-sparing management without a hysterectomy or blood transfusion was possible by not removing the placenta manually during the operation.

Ultrasound evaluation of fetal brain dysfunction based on behavioral patterns.

To identify fetuses at high risk of poor neurological outcomes using a novel ultrasound evaluation system. We assessed an ultrasound evaluation system based on our previous findings, consisting of screening for decreased or lack of fetal movements, abnormal patterns of fetal heart rate, congenital CNS malformations, polyhydramnios of unknown cause, and a "brief ultrasound evaluation" of fetal brain functions, including movement of extremities, breathing movements, ultradian rhythm, REM period, and NREM period. We then assessed the correlation between fetal brain functions and neurological outcomes in infancy (MR, CP, and low Developmental Quotient). During screening, we prospectively evaluated 4978 fetuses receiving prenatal and intrapartum management between January 2000 and December 2009 in our hospital that were later delivered between 32 and 41 weeks' gestation and identified 93 cases as suspicious for impairment. Of the 93 fetuses, 26 underwent the second step of brief ultrasound examination at 35-40 weeks' gestation. Our findings revealed that this method was adequately sensitive (80%) and specific (88%) in identifying neurological impairment. We concluded that this method was mainly useful in the clinical setting for establishing the first indication for fetal CNS examination for functional impairment, rendering it suitable for clinical application.

A retrospective chart review of the perinatal period in 22 pregnancies of 16 women with Moyamoya disease.

Moyamoya disease (MMD) is characterized by severe stenoses of the arteries in the circle of Willis, which predispose the patient to brain ischemia and intracranial hemorrhage. We performed a retrospective chart review of 22 pregnancies in 16 patients with MMD at the Kyushu University Hospital. An uncomplicated Cesarean delivery was performed in nearly all patients. In the 20 pregnancies in patients with pre-existing MMD, two had transient ischemic symptoms in the postpartum period. Two patients not previously known to have MMD developed transient ischemic symptoms postpartum. One of these patients was initially diagnosed and managed as pre-eclampsia. For patients diagnosed with MMD, a good perinatal outcome can be expected with appropriate management. Neurological events, however, may still occur postpartum even in well-managed patients. MMD may mimic the signs and symptoms of other neurological or psychiatric disorders, thereby complicating diagnosis and management.

Measurement of the fetal isovolumetric contraction time in the fetus with a left ventricular aneurysm.

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.

Cervical length predicts placental adherence and massive hemorrhage in placenta previa.

AIM: To evaluate the relationship between cervical length (CL) and obstetrical outcome in women with placenta previa. MATERIAL AND METHODS: Eighty uncomplicated, singleton pregnancies with an antenatally diagnosed previa were categorized based on CL of over 30mm (n=60) or 30mm or less (n=20). A retrospective chart review was then performed for these cases to investigate the relationship between CL and maternal adverse outcomes. RESULTS: The mean CL was 38.5±5.4mm and 26.9±3.2mm and the mean gestational age at measurement was 29.2±2.7 and 28.5±2.7weeks of gestation for the longer and shorter CL groups, respectively. The median estimated blood loss at cesarean section (CS) was significantly higher in the shorter CL group (1302mL vs 2139mL, P=0.023) as was the percentage of patients with massive intraoperative hemorrhage (60.0 vs 18.3%, P=0.001). In the shorter versus longer CL patients, emergent CS before 37weeks (23.3 vs 50.0%, P=0.046) and the percentage of patients with placental adherence (6.7 vs 35.0%, P=0.004) were both significantly more frequent in the shorter CL group. The shorter CL was a risk factor both for massive estimated blood loss (≥2000mL) (odds ratio 6.34, 95% confidence interval 1.91-21.02, P≤0.01) and placental adherence (odds ratio 6.26, 95% confidence interval 1.23-31.87, P≤0.05) in the multivariate analysis. CONCLUSION: CL should be included in the assessment of a placenta previa given its relationship to emergent CS, cesarean hysterectomy, intraoperative blood loss and placental adherence.

Grayscale and Doppler sonographic evaluation of response to in utero treatment of hydrops fetalis caused by extralobar pulmonary sequestration.

Pulmonary sequestration is defined as nonfunctional lung tissue that lacks communication with the bronchial tree and that is supplied by an anomalous systemic vessel. In comparatively rare cases, pulmonary sequestration may lead to hydrothorax or hydrops fetalis, which is nearly universally fatal. In this report, we describe a case of pulmonary sequestration with hydrops fetalis, which was successfully treated by thoracoamniotic shunting. A sonographic Doppler study in this case suggested that the underlying mechanism of the hydropic change in a fetus with extralobar pulmonary sequestration may have differed from that in fetuses with primary hydrothorax not associated with a structural anomaly.

Stercoral perforation of the colon during pregnancy.

A 39-year-old Japanese woman was referred to our hospital for severe abdominal pain at 22 weeks and 2 days of gestation. Abdominal computed tomography (CT) suggested perforation of the gastrointestinal tract and emergency surgery was conducted. There was a fibrous adhesion between an enlarged uterus and the sigmoid colon. There was a 5.0-cm perforation near the adhesion in the posterior wall of the sigmoid colon. We performed a partial resection of the sigmoid colon and Hartmann's procedure with copious intraperitoneal lavage. Five hours following the laparotomy, uterine contractions could not be controlled and the patient delivered vaginally. The neonate died almost immediately after delivery. We conclude that although stercoral bowel perforation is rare, poor prognosis after perforation emphasizes the need to carry out a CT scan for patients who present with undiagnosed severe abdominal pain and compatible medical history, even if the patient is pregnant.

Prognosis and long-term neurodevelopmental outcome in conservatively treated twin-to-twin transfusion syndrome.

BACKGROUND: Amnioreduction remains a treatment option for pregnancies with twin-to-twin transfusion syndrome (TTTS) not meeting criteria for laser surgery or those in which it is not feasible. Amnioreduction is a relatively simple treatment which does not require sophisticated technical equipment. Previous reports of conservative management have indicated that major neurodevelopmental impairment occurs in 14.3-26% of survivors. The purpose of this study was to investigate long-term neurodevelopmental outcome in conservatively treated TTTS. METHODS: During the nine-year study period from January 1996 to December 2004, all pregnancies with TTTS who were admitted to our center were investigated. TTTS was diagnosed by using standard prenatal ultrasound criteria, and staged according to the criteria of Quintero et al. We reviewed gestational age at diagnosis, gestational age at delivery, the stage of TTTS at diagnosis, and diagnosis to delivery interval. Neonatal cranial ultrasound findings were reviewed and the neurodevelopmental outcomes were evaluated. RESULTS: Twenty-one pregnancies with TTTS were included. Thirteen pregnancies (62%) were treated with serial amnioreduction. The mean gestational age at delivery was 28 weeks (22-34 weeks). The perinatal mortality rate was 42.9%. Twenty survivors were followed up until at least 3 years of age. The mean age at follow-up was 6.3 years (3-12 years). Six children (30%) had neurodevelopmental impairment. Four children (20%) had major neurodevelopmental impairment and two children (10%) had minor neurodevelopmental impairment. Children with neurodevelopmental impairment were delivered before 29 weeks of gestation. CONCLUSIONS: Our study showed a high rate of perinatal mortality and a high rate of major neurodevelopmental impairment in conservatively treated TTTS. The long-term outcomes for the survivors with TTTS were good when survivors were delivered after 29 weeks of gestation.

Successful Management of Pregnancy Complicated by Klippel-Trenaunay Syndrome Using MR Angiography-Based Evaluation.

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.

Congenital abdominal aortic aneurysm with porencephaly: a case report.

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.

Three cases of acute fatty liver of pregnancy: postpartum clinical course depends on interval between onset of symptoms and termination of pregnancy.

We report our experience with three cases of acute fatty liver of pregnancy. Case 1 complained of hydrodipsia 4 days before delivery. Case 2 presented with nausea, vomiting and dizziness 6 days before delivery. Case 3 developed loss of appetite and general fatigue with jaundice 10 days before delivery. They underwent termination of pregnancy after diagnosis was made. Case 3 still developed hepatic encephalopathy, and finally she required liver transplantation. We hypothesise that the interval between the onset of symptoms and termination of pregnancy is an important factor for acuity of the disorder and patient morbidity or mortality.