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.

Successful ex utero intrapartum treatment procedure for prenatally diagnosed severe micrognathia: a case report.

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.

Correlation between regular mouthing movements and heart rate patterns during non-rapid eye movement periods in normal human fetuses between 32 and 40 weeks of gestation.

BACKGROUND: Regular mouthing movements (RMMs) are observed during fetal non-rapid eye movement (NREM) periods. AIM: To determine the correlation between RMM and fetal heart rate (FHR) patterns during NREM periods. STUDY DESIGN: Fetal eye and mouth movements and FHR patterns were observed and recorded. SUBJECTS: 50 normal singleton pregnancies between 32 and 40 weeks of gestation. OUTCOME MEASURES: Changes in the power spectrum ratio of 3-minute blocks of RMM clusters, FHR with RMM clusters (HR+), and FHR without RMM clusters (HR-) were calculated at a frequency band of 0.02 Hz among 3 gestational age groups: group 1, 32-34 weeks gestation; group 2, 35-37 weeks gestation; group 3, 38-40 weeks gestation. We calculated the percentage of cases showing dominant peak ratios of RMM and HR+ in the same frequency band, the maximum correlation coefficient, and its lag time. RESULTS: In group 3, the dominant peaks of both RM and HR+ were present at the same frequency band, 0.06-0.08 Hz; this was not seen in the other groups' relative power spectral patterns. The percentage of cases showing dominant peaks of RMM and HR+ in the same frequency band increased with advancing gestational age. The maximum correlation coefficient in groups 1 (0.28 ± 0.11) and 3 (0.45 ± 0.14) differed significantly (p<0.05). CONCLUSIONS: The correlation between RMM and FHR patterns became stronger, and their rhythmicity was similar, from 38 to 40 gestational weeks, suggesting that a common center starts to govern both patterns at approximately 38 weeks gestation.

Characterization of placental transfer of polychlorinated dibenzo-p-dioxins, dibenzofurans and polychlorinated biphenyls in normal pregnancy.

AIM: Prenatal exposure to dioxins may result in many adverse health effects. However, the mechanisms by which dioxins are transferred from mother to fetus through the placenta are not well understood. The aim of this study was to investigate the differences in dioxin concentrations between maternal blood, the placenta, and cord blood in normal pregnant women, and to identify which individual congeners of these compounds are transferred from mother to fetus through the placenta. MATERIAL AND METHODS: Samples were collected from 19 pregnant Japanese women. Specific congeners of seven polychlorinated dibenzo-p-dioxins (PCDDs), 10 polychlorinated dibenzofurans (PCDFs), and four non-ortho polychlorinated biphenyls (PCBs) were analyzed. RESULTS: The TEQ concentrations of PCDDs, PCDFs, and non-ortho PCBs were 8.03, 3.39, and 3.95 pg TEQ/g lipid, respectively, in the maternal blood; 8.78, 3.61, and 0.87 pg TEQ/g lipid in the placenta; and 4.33, 1.25, 1.08 pg TEQ/g lipid in the cord blood. Among specific congeners, 1,2,3,7,8-PentaCDD and 2,3,4,7,8-PentaCDF exhibited a placenta to maternal blood ratio greater than 1.0, while OctaCDD exhibited the greatest cord blood to placenta ratio. The cord blood to maternal blood ratio of total PCDDs was significantly higher than that of total PCDFs and total non-ortho PCBs. CONCLUSION: The dioxin concentration in cord blood was approximately half of the amount in maternal blood, despite congeners showing a high toxic equivalency factor accumulating in the placenta. PCDDs were transferred more readily than PCDFs and non-ortho PCBs from maternal blood to the fetus through the placenta.

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.

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.

Prenatal three-dimensional images of proximal focal femoral deficiency produced by helical computed tomography.

INTRODUCTION: Proximal focal femoral deficiency (PFFD) is a rare skeletal disorder characterized by failure in development of the subtrochanteric region of the femoral shaft, with varying degrees of shortening of the proximal femur. OBJECTIVE: To investigate the potential of helical computed tomography as a prenatal diagnostic tool for bony abnormalities. CASE: A 37-year-old Japanese woman was referred to our hospital at 32 weeks of gestation for the evaluation of fetal growth restriction with short femurs. An ultrasound examination revealed the fetus to have short femurs bilaterally with normal echogenicity, and a normal facial profile. Assessment by 3D CT confirmed the absence of the femoral heads bilaterally and also revealed bilateral hip dislocations and oligodactyly of the right hand. The baby was delivered by cesarean section at 37 weeks of gestation, whereupon the diagnosis of PFFD was confirmed. CONCLUSION: Helical CT is a useful prenatal diagnostic alternative for bony abnormalities that is superior to the conventional sonographic approach.