Objective: To report our experience with management of fetuses with congenital high airway obstruction syndrome (CHAOS). Methods: We retrospectively reviewed the cases of fetuses who were prenatally diagnosed and postnatally confirmed with CHAOS between 2010 and 2019 at Asan Medical Center, Seoul, Korea. Results: Of 13 fetuses prenatally diagnosed with CHAOS, 7 were lost to follow-up and 6 were postnatally confirmed as having CHAOS. All fetuses, except one were delivered via cesarean section with an ex utero intrapartum treatment (EXIT) procedure. Two patients had coexisting congenital heart diseases requiring several cardiac surgeries following birth. Both of these patients demonstrated developmental delay; however, the remaining 4 had a normal development except for expressive language. Two infants died of respiratory complications, and the remaining 4 were alive at the end of the follow-up period. All 4 live patients underwent tracheostomy with planned reconstruction surgery. Three children are now able to phonate, and 1 can maintain a conservation. Conclusion The proper management of CHAOS using the EXIT procedure results in high survival and low hypoxemia-induced complication rates. Therefore, an accurate prenatal diagnosis is necessary for an appropriate perinatal management.

There have been ongoing social discussions on revision of the abortion law since the Constitutional Court ruled it nonconformity to the constitution on April 11, 2019. Thus, Korean Society of Obstetrics and Gynecology, Korean Society of Maternal Fetal Medicine, Korean College of Obstetricians and Gynecologists, and Korean Association of Obstetricians and Gynecologists organized 'special committee for abortion law' to support social discussion on the revision of the abortion law, and they prepared official opinions of obstetrics for medical issues including doctors' right to refuse abortion. In the future, the committee will continue to adapt to changes of medical environment especially after the revision of the abortion law by collecting its members' feedbacks.

There have been ongoing social discussions on revision of the abortion law since the Constitutional Court ruled it nonconformity to the constitution on April 11, 2019. Thus, Korean Society of Obstetrics and Gynecology, Korean Society of Maternal Fetal Medicine, Korean College of Obstetricians and Gynecologists, and Korean Association of Obstetricians and Gynecologists organized 'special committee for abortion law' to support social discussion on the revision of the abortion law, and they prepared official opinions of obstetrics for medical issues including doctors' right to refuse abortion. In the future, the committee will continue to adapt to changes of medical environment especially after the revision of the abortion law by collecting its members' feedbacks.

OBJECTIVE: This study was conducted to demonstrate the temporal trends in perinatal outcomes of triplet pregnancies over the last two decades. METHODS: The medical records of patients with triplet pregnancies at two Korean tertiary-care hospitals from 1992 to 2012 were retrospectively reviewed in regard to maternal and neonatal outcomes. The study was divided into two periods for analysis: period I (1992–2001) and period II (2003–2012). RESULTS: Over a 21-year period, 65 women with triplet pregnancies and 185 neonates were analyzed. Period II, when compared with period I, was associated with improved maternal outcomes, characterized by a decreased incidence of preeclampsia (31.8% vs. 2.3%, P=0.002) and anemia (68.2% vs. 30.2%, P=0.003) during pregnancy. Regarding neonatal aspects, the composite morbidity of period II was significantly decreased compared with that of period I, as assessed with a generalized estimating equation for logistic regression (26.2% vs. 8.1%, P=0.03). Multivariable analysis revealed that the gestational age at delivery and the period were significantly associated with the composite neonatal morbidity (P<0.001 and 0.007, respectively). CONCLUSION: Improved neonatal morbidity was associated with a higher gestational age at delivery and with the more recent decade.
Anemia ; Female ; Gestational Age ; Humans ; Incidence ; Infant, Newborn ; Logistic Models ; Medical Records ; Pre-Eclampsia ; Pregnancy ; Pregnancy Outcome ; Pregnancy, Triplet ; Premature Birth ; Retrospective Studies ; Triplets

PURPOSE: Although home renovation exposure during childhood has been identified as a risk factor for the development of allergy, there is limited information on the association between prenatal exposure to home renovation and cord blood (CB) IgE response. The aims of this study were to identify the effect of prenatal exposure to home renovation on CB IgE levels, and to investigate whether this exposure interacts with neonatal genes and whether the effect can be modified by maternal atopy. METHODS: This study included 1,002 mother-neonate pairs from the COhort for Childhood Origin of Asthma and allergic diseases (COCOA). Prenatal environmental factors were collected using a questionnaire. The levels of CB IgE were measured by the ImmunoCAP system, and DNA was extracted from CB. RESULTS: Exposure to home renovation during the prenatal period was associated with significantly higher levels of CB IgE only in neonates from atopic mothers, and the effect of renovation exposure on CB IgE levels persisted from 31 months before birth. Furthermore, prenatal exposure to home renovation increased the risk of CB IgE response interacting with polymorphisms of NRF2 and GSTP1 genes only in neonates from atopic mothers. CONCLUSIONS: Maternal atopy modified the effect of prenatal exposure to home renovation on CB serum IgE response as well as the interaction between the exposure and neonatal genes involved in the oxidative stress pathway. These findings suggest that the genetically susceptible offspring of atopic mothers may be more vulnerable to the effect of prenatal exposure to home renovation on the development of allergy.
Asthma ; Cohort Studies ; DNA ; Fetal Blood* ; Gene-Environment Interaction ; Humans ; Hypersensitivity ; Immunoglobulin E* ; Infant, Newborn ; Mothers ; Oxidative Stress ; Parturition ; Polymorphism, Single Nucleotide ; Reactive Oxygen Species* ; Risk Factors

We describe a case of an intrathoracic kidney combined with right congenital diaphragmatic hernia (CDH) that was diagnosed at 32 weeks of gestation. Although it has been well established that a right CDH shows a poorer outcome than a left CDH, our present case showed a good outcome because there was no herniation of other abdominal viscera, except for the right kidney. Our findings in this case indicate that impaction of the intrathoracic kidney may act as a 'shield' against further herniation of other abdominal viscera into the thoracic cavity.
Hernia, Diaphragmatic* ; Kidney* ; Pregnancy ; Thoracic Cavity ; Ultrasonography ; Viscera*

We describe a case of absent aortic and pulmonary valves, diagnosed at 16.4 weeks of gestation. Fetal echocardiography showed cardiomegaly with dilated both ventricles. No valve leaflets were observed in the aorta and pulmonary artery, and a typical to-and-fro flow pattern was noted in both great arteries on color Doppler imaging. Fetal hydrops was also detected. Follow-up ultrasonographic evaluation at 19 weeks demonstrated intrauterine fetal death. Postmortem autopsy revealed the absence of both aortic and pulmonary valve leaflets. To the best of our knowledge, this is the earliest diagnosed case of absent both aortic and pulmonary valves and only the second case to be diagnosed prenatally.
Aorta ; Aortic Valve ; Arteries ; Autopsy ; Cardiomegaly ; Echocardiography ; Fetal Death ; Follow-Up Studies ; Heart Defects, Congenital ; Hydrops Fetalis ; Pregnancy ; Prenatal Diagnosis ; Pulmonary Artery ; Pulmonary Valve*

We describe a case of absent aortic and pulmonary valves, diagnosed at 16.4 weeks of gestation. Fetal echocardiography showed cardiomegaly with dilated both ventricles. No valve leaflets were observed in the aorta and pulmonary artery, and a typical to-and-fro flow pattern was noted in both great arteries on color Doppler imaging. Fetal hydrops was also detected. Follow-up ultrasonographic evaluation at 19 weeks demonstrated intrauterine fetal death. Postmortem autopsy revealed the absence of both aortic and pulmonary valve leaflets. To the best of our knowledge, this is the earliest diagnosed case of absent both aortic and pulmonary valves and only the second case to be diagnosed prenatally.
Aorta ; Aortic Valve ; Arteries ; Autopsy ; Cardiomegaly ; Echocardiography ; Fetal Death ; Follow-Up Studies ; Heart Defects, Congenital ; Hydrops Fetalis ; Pregnancy ; Prenatal Diagnosis ; Pulmonary Artery ; Pulmonary Valve*

Thanatophoric dysplasia (TD) is caused by mutation of the gene that encodes fibroblast growth factor 3 (FGFR3). Owing to the poor prognosis for TD, prenatal diagnosis is critical to optimal perinatal management. We report here a case of TD in twin pregnancy, which was prenatally diagnosed by DNA analysis following amniocentesis at 15 weeks, and was managed by selective fetal termination. Prenatal ultrasonography and molecular analysis to detect TD-specific mutations enable accurate diagnosis of FGFR3-related TD in utero and appropriate obstetrical management at early gestation during twin pregnancy.
Amniocentesis ; Diagnosis ; DNA ; Female ; Fibroblast Growth Factor 3 ; Humans ; Pregnancy ; Pregnancy Reduction, Multifetal ; Pregnancy Trimester, Second* ; Pregnancy, Twin ; Prenatal Diagnosis ; Prognosis ; Thanatophoric Dysplasia* ; Twins* ; Ultrasonography, Prenatal

OBJECTIVE: To report on our experiences with thoracoamniotic shunting and/or the injection of a sclerosing agent (OK-432) to treat fetuses diagnosed with macrocystic congenital cystic adenomatoid malformation (CCAM) of the lung. METHODS: A retrospective study was undertaken in six fetuses with macrocystic CCAM at our institute that had been confirmed by postnatal surgery between August 1999 and January 2012. RESULTS: Six fetuses that had been diagnosed with macrocystic CCAM were analyzed. The median gestational age at diagnosis was 23.5 weeks (range, 19.5-31.0 weeks), and at the time of primary treatment was 24.0 weeks (range, 20.5-31.0 weeks). The mean size of the largest cyst at the initial assessment was 42.5+/-15 mm. Four fetuses were associated with mediastinal shifting, and one also showed fetal hydrops. All fetuses underwent a shunting procedure within the cysts, one case among them was also treated with OK-432. After the completion of all procedures, the mean size of the largest cyst was all decreased (14.2+/-12 mm). The median gestational age at delivery was 38.0 weeks (range, 32.4-40.3 weeks). All of the newborns underwent the surgical resection at a median age of 6 days (range, 1-136 days) and are currently doing well without any complications. CONCLUSION: We suggest that intrauterine decompression therapy to manage fetal macrocystic CCAM is recommendable treatment for good perinatal outcome.
Cystic Adenomatoid Malformation of Lung, Congenital* ; Decompression ; Diagnosis ; Fetal Therapies ; Fetus ; Gestational Age ; Humans ; Hydrops Fetalis ; Infant, Newborn ; Lung* ; Picibanil ; Prenatal Diagnosis ; Retrospective Studies ; Sclerosing Solutions