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      Feasibility and outcomes of fetoscopic endoluminal tracheal occlusion for severe congenital diaphragmatic hernia: A Japanese experience

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          Abstract

          Aim

          To present the feasibility, safety and outcomes of fetoscopic endoluminal tracheal occlusion (FETO) for the treatment of severe congenital diaphragmatic hernia (CDH).

          Methods

          This was a single‐arm clinical trial of FETO for isolated left‐sided CDH with liver herniation and Kitano Grade 3 stomach position (>50% stomach herniation into the right chest). FETO was performed at 27–29 weeks of gestation for cases with observed/expected lung to head ratio (o/e LHR) <25% and at 30–31 weeks for cases with o/e LHR ≥25%.

          Results

          Eleven cases were enrolled between March 2014 and March 2016, and balloon insertion was successful in all cases. The median o/e LHR at entry was 27% (range, 20–33%). The median gestational age at FETO was 30.9 (range, 27.1–31.7) weeks. There were no severe maternal adverse events. One fetus died unexpectedly at 33 weeks of gestation due to cord strangulation by the detached amniotic membrane. There were 3 cases (27%) of preterm premature rupture of membranes. In all 10 cases, balloon removal at 34–35 weeks of gestation was successful. The median gestational age at delivery was 36.5 (range, 34.2–38.3) weeks. The median duration of occlusion and the median interval between balloon insertion and delivery were 26 days (range: 17–49 days) and 43 days (range, 21–66 days), respectively. Both the survival rate at 90 days of age and the rate of survival to discharge were 45% (5/11).

          Conclusion

          The FETO is feasible without maternal morbidity in Japan and could be offered to women whose fetuses show severe isolated left‐sided CDH to accelerate fetal lung growth.

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          Most cited references37

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          Severe diaphragmatic hernia treated by fetal endoscopic tracheal occlusion.

          To examine operative and perinatal aspects of fetal endoscopic tracheal occlusion (FETO) in congenital diaphragmatic hernia (CDH). This was a multicenter study of singleton pregnancies with CDH treated by FETO. The entry criteria for FETO were severe CDH on the basis of sonographic evidence of intrathoracic herniation of the liver and low lung area to head circumference ratio (LHR) defined as the observed to the expected normal mean for gestation (o/e LHR) equivalent to an LHR of 1 or less. FETO was carried out in 210 cases, including 175 cases with left-sided, 34 right-sided and one with bilateral CDH. In 188 cases the CDH was isolated and in 22 there was an associated defect. FETO was performed at a median gestational age of 27.1 (range, 23.0-33.3) weeks. The first eight cases were done under general anesthesia, but subsequently either regional or local anesthesia was used. The median duration of FETO was 10 (range, 3-93) min. Successful placement of the balloon at the first procedure was achieved in 203 (96.7%) cases. Spontaneous preterm prelabor rupture of membranes (PPROM) occurred in 99 (47.1%) cases at 3-83 (median, 30) days after FETO and within 3 weeks of the procedure in 35 (16.7%) cases. Removal of the balloon was prenatal either by fetoscopy or ultrasound-guided puncture, intrapartum by ex-utero intrapartum treatment, or postnatal either by tracheoscopy or percutaneous puncture. Delivery was at 25.7-41.0 (median, 35.3) weeks and before 34 weeks in 65 (30.9%) cases. In 204 (97.1%) cases the babies were live born and 98 (48.0%) were discharged from the hospital alive. There were 10 deaths directly related to difficulties with removal of the balloon. Significant prediction of survival was provided by the o/e LHR and gestational age at delivery. On the basis of the relationship between survival and o/e LHR in expectantly managed fetuses with CDH, as reported in the antenatal CDH registry, we estimated that in fetuses with left CDH treated with FETO the survival rate increased from 24.1% to 49.1%, and in right CDH survival increased from 0% to 35.3% (P < 0.001). FETO in severe CDH is associated with a high incidence of PPROM and preterm delivery but a substantial improvement in survival.
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            Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia.

            To assess the value of antenatally determined observed to expected fetal lung area to head circumference ratio (LHR) in the prediction of postnatal survival in isolated, congenital diaphragmatic hernia (CDH). Two groups of fetuses were examined. The first group included 650 normal fetuses at 12-32 weeks' gestation, and the data collected were used to establish a normal range of observed to expected LHR with gestational age. The second group included the data of a retrospective multicenter study of 354 fetuses with isolated CDH in which the LHR was measured on one occasion at 18-38 weeks' gestation. The patients were divided into those with left-sided CDH with and without intrathoracic herniation of the liver and right-sided CDH. Regression analysis was used to determine the significant predictors of postnatal survival. In both the normal fetuses and those with CDH the LHR increased but the observed to expected LHR did not change significantly with gestational age. In normal fetuses the mean observed to expected LHR in the left lung was 100% (95% CI, 61-139%) and in the right lung it was 100% (95% CI, 67-133%). In fetuses with CDH the mean observed to expected LHR was 39% (range 7-79%). Regression analysis demonstrated that significant predictors of survival were the observed to expected LHR (odds ratio (OR) 1.09, 95% CI, 1.06-1.12), side of CDH (left side OR 11.14, 95% CI, 3.41-36.39) and gestational age at delivery (OR 1.18, 95% CI, 1.02-1.36). In CDH, the LHR increases while observed to expected LHR is independent of gestational age. In fetuses with both left- and right-sided CDH, measurement of the observed to expected LHR provides a useful prediction of subsequent survival.
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              A randomized trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia.

              Experimental and clinical data suggest that fetal endoscopic tracheal occlusion to induce lung growth may improve the outcome of severe congenital diaphragmatic hernia. We performed a randomized, controlled trial comparing fetal tracheal occlusion with standard postnatal care. Women carrying fetuses that were between 22 and 27 weeks of gestation and that had severe, left-sided congenital diaphragmatic hernia (liver herniation and a lung-to-head ratio below 1.4), with no other detectable anomalies, were randomly assigned to fetal endoscopic tracheal occlusion or standard care. The primary outcome was survival at the age of 90 days; the secondary outcomes were measures of maternal and neonatal morbidity. Of 28 women who met the entry criteria, 24 agreed to randomization. Enrollment was stopped after 24 patients had been enrolled because of the unexpectedly high survival rate with standard care and the conclusion of the data safety monitoring board that further recruitment would not result in significant differences between the groups. Eight of 11 fetuses (73 percent) in the tracheal-occlusion group and 10 of 13 (77 percent) in the group that received standard care survived to 90 days of age (P=1.00). The severity of the congenital diaphragmatic hernia at randomization, as measured by the lung-to-head ratio, was inversely related to survival in both groups. Premature rupture of the membranes and preterm delivery were more common in the group receiving the intervention than in the group receiving standard care (mean [+/-SD] gestational age at delivery, 30.8+/-2.0 weeks vs. 37.0+/-1.5 weeks; P<0.001). The rates of neonatal morbidity did not differ between the groups. Tracheal occlusion did not improve survival or morbidity rates in this cohort of fetuses with congenital diaphragmatic hernia. Copyright 2003 Massachusetts Medical Society
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                Author and article information

                Contributors
                sagou-h@ncchd.go.jp
                Journal
                J Obstet Gynaecol Res
                J Obstet Gynaecol Res
                10.1111/(ISSN)1447-0756
                JOG
                The Journal of Obstetrics and Gynaecology Research
                John Wiley & Sons Australia, Ltd (Kyoto, Japan )
                1341-8076
                1447-0756
                28 September 2020
                December 2020
                : 46
                : 12 ( doiID: 10.1111/jog.v46.12 )
                : 2598-2604
                Affiliations
                [ 1 ] Center for Maternal‐Fetal, Neonatal and Reproductive Medicine National Center for Child Health and Development Tokyo Japan
                [ 2 ] Division of Surgery, Department of Surgical Specialties National Center for Child Health and Development Tokyo Japan
                [ 3 ] Department of Pediatric Surgery Osaka University Graduate School of Medicine Suita Japan
                [ 4 ] Department of Pediatric Surgery Osaka Women's and Children's Hospital Izumi Japan
                [ 5 ] Department of Maternal Fetal Medicine Osaka Women's and Children's Hospital Izumi Japan
                [ 6 ] Department of Obstetrics and Gynecology Nagoya University Graduate School of Medicine Nagoya Japan
                [ 7 ] Division of Neonatology, Center for Maternal‐Neonatal Care Nagoya University Hospital Nagoya Japan
                [ 8 ] Department of Obstetrics and Gynecology Kyushu University School of Medicine Fukuoka Japan
                [ 9 ] Department of Pediatric Surgery Kyushu University School of Medicine Fukuoka Japan
                [ 10 ] Department of Obstetrics and Gynecology Osaka University Graduate School of Medicine Suita Japan
                Author notes
                [*] [* ] Correspondence: Dr Haruhiko Sago, Center for Maternal‐Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2‐10‐1 Okura, Setagaya‐ku, Tokyo, 157‐8535 Japan. Email: sagou-h@ 123456ncchd.go.jp

                Article
                JOG14504
                10.1111/jog.14504
                7756773
                32989906
                80a07ff1-3b51-4e68-b339-2286df65df51
                © 2020 The Authors. Journal of Obstetrics and Gynaecology Research published by John Wiley & Sons Australia, Ltd on behalf of Japan Society of Obstetrics and Gynecology.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                : 28 July 2020
                : 03 September 2020
                : 15 September 2020
                Page count
                Figures: 1, Tables: 3, Pages: 7, Words: 5140
                Funding
                Funded by: Grants from the National Center for Child Health and Development of Japan
                Award ID: 24‐2
                Award ID: 27‐3
                Funded by: National Center for Child Health and Development , open-funder-registry 10.13039/100007786;
                Categories
                Original Article
                Original Articles
                Custom metadata
                2.0
                December 2020
                Converter:WILEY_ML3GV2_TO_JATSPMC version:5.9.6 mode:remove_FC converted:23.12.2020

                adverse events,congenital diaphragmatic hernia,fetal mortality,fetal therapy,fetoscopy

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