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Background/purpose: Few large multicenter surveys have been performed on sacrococcygeal teratomas (SCTs) describing both the prenatal and postnatal courses. The aim of this study was to review and report on the prenatal surveillance and postnatal outcome of a large cohort of fetuses with SCTs in Japan., Methods: A nationwide retrospective cohort study was conducted on 97 fetuses prenatally diagnosed with SCTs between 2000 and 2009. The prenatal course, perinatal data, and postnatal outcome were reviewed., Results: Eleven pregnancies were terminated before 22 weeks of gestation. Of the 86 remaining fetuses, 3 died in utero, and 83 were delivered. Three infants died before surgery, and 8 infants died after excisional surgery. The overall mortality was 26%, with a mortality excluding terminations of 16%. The gestational age at delivery was younger than 28 weeks in 5, 28 to 31 weeks in 13, 32 to 36 weeks in 27, and 37 weeks or more in 37 cases, with mortality rates of 60%, 38%, 11%, and 0%, respectively. The tumor component was predominantly cystic in 54 and predominantly solid in 32 cases, with mortality rates of 2% and 33%, respectively., Conclusions: The overall mortality of prenatally diagnosed SCTs excluding terminations was 16%. Early delivery and predominantly solid component tumors were associated with an increased risk of mortality., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Perinatal management of congenital diaphragmatic hernia (CDH) and gastroschisis (GS) remains nonstandardized and institution specific. This analysis describes practice and outcome variation across a national network., Methods: A national, prospective, disease-specific database for CDH and GS was evaluated over 4 years. Centers were evaluated individually and defined as low (low-volume center [LVC]) or high (high-volume center [HVC]) volume based on case mean., Results: Congenital diaphragmatic hernia. Two hundred fifteen liveborn cases were studied (mean, 14.3 cases/center) across 15 centers (8 LVCs and 7 HVCs). Significant interinstitutional practice variation was noted in rates of termination (0%-40%) and cesarean delivery (0%-61%). Centers demonstrated marked variation in ventilation strategies, vasodilator and paralytic use, timing of surgery, and rates of primary closure. Overall survival was 81.4% (LVC, 76.9%; HVC, 82.4%; P = .43). Gastroschisis. Four hundred sixteen cases were investigated (mean, 26 cases/center; range, 6-72) across 16 centers (10 LVCs and 6 HVCs). Cesarean delivery rates varied widely between centers (0%-86%) as did timing of closure (early vs delayed, 1%-100%). There was no difference in length of stay, days on total parenteral nutrition, and overall survival (94.3% vs 97.2%; P = .17) between LVCs and HVCs., Conclusions: The existence of perinatal practice and outcome variation for GS and CDH suggests targets for improved delivery of care and justifies efforts to standardize treatment on a national basis., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: The termination of pregnancy because of fetal abnormalities in Japan has not been described. The aim of the present study was to analyze the current status and to evaluate the medical and ethical relevance in our institution for negative treatment decision-making for fetuses demonstrating neonatal surgical disease with a prenatal diagnosis., Materials and Methods: The medical records of 209 fetuses with a prenatal diagnosis from 1999 to 2008 were retrospectively reviewed. The cases with a negative treatment policy were analyzed according to the potential for survival. The negative treatment policies were defined as those in which the pregnancy was not actively continued, including elective termination of pregnancy and palliative or limited treatment that are primarily provided after birth., Results: The selected treatment policies were active in 162 cases and negative in 46 cases. Thirty-three cases with negative policies were in the second-half period of pregnancy. The potential for survival was high in 5 cases, moderate in 11 cases, and nonviable in 30 cases. Eight of the nonviable cases underwent either limited or palliative treatment, whereas the remaining 38 fetuses were aborted., Conclusions: The negative treatment policies in the nonviable fetuses were considered to be medically and ethically relevant. However, the number of cases with negative policies increased over the last 5 years and is therefore associated with complex ethical issues., (Copyright © 2010 Elsevier Inc. All rights reserved.)

The impact of neural tube defects on neonatal surgery has been declining in Scotland over the past two decades. The Scottish statistics for neural tube defects were studied from 1971 to 1988. The incidence of neural tube defects in Scotland has declined from 5.50 to 1.10 per 1,000 births over this period (3.00 to 0.58 per 1,000 births for spina bifida and 2.50 to 0.52 per 1,000 births for anencephaly). Antenatal maternal alpha-fetoprotein (AFP) screening was introduced to Scotland on a wide scale in 1976. The number of terminations for anencephaly peaked in 1980 (85), and for spina bifida in 1981 (70), and both have since declined. The Scottish birth rate has been about 67,000 per year over this period. The declining incidence of neural tube defects is not explained by the effect of antenatal screening and terminations alone. A downward trend was apparent before 1976, and although antenatal screening has had a considerable impact on anencephaly births (peak terminations 89% in 1983), it has had only a modest impact on spina bifida births (peak terminations 53% in 1984). We conclude that the natural decline in incidence of neural tube defects is the major factor in the observed decline in neonatal surgical admissions for these defects.