HEALTH OF WOMAN. 2020.8(154): 47–53; doi 10.15574/HW.2020.154.47
Grebinichenko G. O., Gordienko I. Yu., Sliepov O. K.
SI «Institute of pediatrics, obstetrics and ginecology named academic Elena. M. Lukyanova NAMS of Ukraine»

The objective: to characterize pregnancy course and clinical outcomes for newborns in cases of congenital diaphragmatic hernia, and to compare these data with the results of prenatal examinations
Materials and methods. Results of complex prenatal examination of 259 fetuses as patients with diaphragmatic hernia, which were referred to the department of Fetal medicine in 2007–2020, were analyzed. Data on pregnancy course and clinical outcome for infants were collected in 144 cases: from medical records of the Institute (n=77), and by direct survey, if delivery and specialized care took place in other institutions (n=67). The results were compared depending on the clinical outcome and the place of care.
Results. Among 144 cases with known outcome, 140 (97.2%) were singleton and 4 (2.8%) were twin pregnancies with only one affected fetus. Termination of pregnancy for fetal anomalies before to 22 weeks was performed in 16.7% (44/144), 83.3% of cases (120/144) ended in childbirth. Before 22 weeks for prenatal examination were referred 30.6% (44/144) of women, of them 54.5% (24/44) have chosen termination in presence of unfavorable prognosis, and in the remaining 45.5% (20/44) cases pregnancy was prolonged. Of the 120 labors, 92.5% were term and 7.5% preterm. Antenatal death after 22 weeks occurred in 4.2% (5/120) cases.
Among liveborn infants before surgical correction in the clinics of the Institute have died 25% (18/72), and in other institutions 37.2% (16/43), р>0.05. Postoperative mortality was 13% (7/54) in the Institute and 42.3% (11/26) in other institutions, р=0.0032. The rate of associated pathology was significantly higher among children treated in other institutions (25.6% vs. 5.6%), but the proportion of operated patients did not differ significantly (60.5% in other institutions and 75% in the Institute). Comparison of data of prenatal examination showed significantly higher rate of associated pathology (26.3% vs. 3.2%), polyhydramnios (50.9% vs. 20.6%), right-sided hernia (21.2% vs. 6.3%) and liver herniation in left-sided hernia (81.8% vs. 28.8%) in the group with negative outcome.
Conclusions. The majority of pregnancies with diaphragmatic hernia in the fetus ended in childbirth, with high mortality rate. Strategy of complex prenatal examination in cases of congenital diaphragmatic hernia, prognosis evaluation by multidisciplinary council with individualized pregnancy/labor management planning, and delayed surgical correction allow to optimize specialized care and to avoid ineffective surgical interventions in extremely severe clinical and anatomical variants of pathology.
Keywords: congenital diaphragmatic hernia, prenatal diagnosis, chromosomal anomalies, congenital malformations, pregnancy, labor.

REFERENCES

1. Antypkin YuH, Sliepov OK, Veselskyi VL, Gordienko IYu, Hrasiukova NI, Avramenko TV, Soroka VP, Sliepova LF, Ponomarenko OP. 2014. Suchasni orhanizatsiino-metodychni pidkhody do perynatalnoi diahnostyky ta khirurhichnoho likuvannia pryrodzhenykh vitalnykh vad rozvytku u novonarodzhenykh ditei v umovakh perynatalnoho tsentru. Zhurnal Natsionalnoi akademii medychnykh nauk Ukrainy 20(2):189–199.

2. Ackerman KG, Vargas SO, Wilson JA, Jennings RW, Kozakewich HP, Pober BR. 2012. Congenital diaphragmatic defects: proposal for a new classification based on observations in 234 patients. Pediatr Dev Pathol. 15(4):265-274. https://doi.org/10.2350/11-05-1041-OA.1; PMid:22257294 PMCid:PMC3761363

3. Barbosa BML, Rodrigues AS, Carvalho MHB, Bittar RE, Francisco RPV, Bernardes LS. 2018. Spontaneous prematurity in fetuses with congenital diaphragmatic hernia: A retrospective cohort study about prenatal predictive factors. BMC Pregnancy and Childbirth. 18(1):27. https://doi.org/10.1186/s12884-017-1652-6; PMid:29329576 PMCid:PMC5767016

4. Basta AM, Lusk LA, Keller RL, Filly RA. 2016. Fetal Stomach Position Predicts Neonatal Outcomes in Isolated Left-Sided Congenital Diaphragmatic Hernia. Fetal Diagn Ther. 39(4):248-255. https://doi.org/10.1159/000440649; PMid:26562540 PMCid:PMC4866917

5. Bhat YR, Kumar V, Rao A. 2008. Congenital diaphragmatic hernia in a developing country. Singapore Medical Journal 49(9):715-718.

6. Burgos CM, Frenckner B, Luco M, Harting MT, Lally PA, Lally KP; Congenital Diaphragmatic Hernia Study Group. 2018. Right versus left congenital diaphragmatic hernia – What's the difference? J Pediatr Surg. 53:113-117. https://doi.org/10.1016/j.jpedsurg.2017.10.027; PMid:29122292

7. Cannie M, Jani J, Chaffiotte C, Vaast P, Deruelle P, Houfflin-Debarge V, Dymarkowski S, Deprest J. 2008. Quantification of intrathoracic liver herniation by magnetic resonance imaging and prediction of postnatal survival in fetuses with congenital diaphragmatic hernia. Ultrasound Obstet Gynecol. 32(5):627-632. https://doi.org/10.1002/uog.6146; PMid:18792415

8. Colvin J, Bower C, Dickinson JE, Sokol J. 2005. Outcomes of Congenital Diaphragmatic Hernia: A Population-Based Study in Western Australia. Pediatrics 116(3):e356-e363. https://doi.org/10.1542/peds.2004-2845; PMid:16140678

9. Cordier AG, Jani JC, Cannie MM, Rodу C, Fabietti I, Persico N, Saada J, Carreras E, Senat MV, Benachi A. 2015. Stomach position in prediction of survival in left-sided congenital diaphragmatic hernia with or without fetoscopic endoluminal tracheal occlusion. Ultrasound Obstet Gynecol. 46(2):155-161. https://doi.org/10.1002/uog.14759; PMid:25487417

10. Demidov VN, Mashynets NV, Podurovskaya YL, Burov AA. 2014. Vrozhdennaya diaphragmalnaya gryzha ploda – vozmozhnosti ultrazvukovoy diagnostiki i prognosirovaniya postnetalnoho iskhoda. Akusherstvo i ginekologiya. 4:38-45.

11. Gallot D, Boda C, Ughetto S, Perthus I, Robert-Gnansia E, Francannet C, Laurichesse-Delmas H, Jani J, Coste K, Deprest J, Labbe A, Sapin V, Lemery D. 2007. Prenatal detection and outcome of congenital diaphragmatic hernia: a French registry-based study. Ultrasound Obstet Gynecol. 29(3):276-283. https://doi.org/10.1002/uog.3863; PMid:17177265

12. Gordienko IY, Grebinichenko GO, Slepov OK, Veselskiy VL, Tarapurova OM, Nidelchuk OV, Nosko AO. 2013. New lung-to-femur index in prenatal diagnosis of fetal lung hypoplasia. Health of woman 9:143-146.

13. Gordienko IY, Grebinichenko GO, Tarapurova OM, Velychko AV. 2019. Variants of prenatal ultrasound imaging of congenital diaphragmatic hernia in the fetus. Radiation Diagnostics, Radiation Therapy 4:12-21. http://rdrt.com.ua/index.php/journal/article/view/242. https://doi.org/10.37336/2707-0700-2019-4-1

14. Grebinichenko GO, Gordienko IY, Tarapurova OM, Sliepov OK. 2019. Two-dimensional ultrasound examination for assessment of the degree of liver herniation into the chest in fetuses with congenital diaphragmatic hernia. Ukrainian Journal of Perinatology and Pediatrics. 4(80):10-15. https://doi.org/10.15574/PP.2019.80.10

15. Grebinichenko GO, Gordienko IY, Tarapurova OM, Slepov OK, Veselskiy VL, Nidelchuk OV, Nosko AO, Velychko AV. 2014. An assessment of the degree of fetal lung hypoplasia with two-dimensional ultrasound. Perinatologiya i pediatriya 3:21-25. https://doi.org/10.15574/PP.2014.59.21

16. Kalanj J, Salevic P, Rsovac S, Medjo B, Antunovic SS, Simic D. 2016. Congenital diaphragmatic hernia – a Belgrade single center experience. J Perinat Med. 44(8):913-918. https://doi.org/10.1515/jpm-2015-0333; PMid:26985684

17. Kitano Y, Okuyama H, Saito M, Usui N, Morikawa N, Masumoto K, Takayasu H, Nakamura T, Ishikawa H, Kawataki M, Hayashi S, Inamura N, Nose K, Sago H. 2011. Re-evaluation of stomach position as a simple prognostic factor in fetal left congenital diaphragmatic hernia: a multicenter survey in Japan. Ultrasound Obstet Gynecol. 37(3):277-282. https://doi.org/10.1002/uog.8892; PMid:21337653

18. Kitagawa M, Hislop A, Boyden EA, Reid L. 1971. Lung hypoplasia in congenital diaphragmatic hernia. A quantitative study of airway, artery, and alveolar development. Br J Surg. 58(5):342-346. https://doi.org/10.1002/bjs.1800580507; PMid:5574718

19. Lam JC, Claydon J, Mitton CR, Skarsgard ED. 2006. A risk-adjusted study of outcome and resource utilization for congenital diaphragmatic hernia. J Pediatr Surg. 41(5):883-887. https://doi.org/10.1016/j.jpedsurg.2006.01.025; PMid:16677875

20. Masahata K, Usui N, Shimizu Y, Takeuchi, M, Sasahara J, Mochizuki N, Tachibana K, Abe T, Yamamichi T, Soh H. 2020. Clinical outcomes and protocol for the management of isolated congenital diaphragmatic hernia based on our prenatal risk stratification system. Journal of pediatric surgery 55(8):1528-1534. https://doi.org/10.1016/j.jpedsurg.2019.10.020; PMid:31864663

21. Mullassery D, Ba'ath ME, Jesudason EC, Losty PD. 2010. Value of liver herniation in prediction of outcome in fetal congenital diaphragmatic hernia: a systematic review and meta-analysis. Ultrasound Obstet Gynecol. 35(5):609-614. https://doi.org/10.1002/uog.7586; PMid:20178116

22. Putnam LR, Harting MT, Tsao K, Morini F, Yoder BA, Luco M, Lally PA, Lally KP. 2016. Congenital diaphragmatic hernia study group. Congenital diaphragmatic hernia defect size and infant morbidity at discharge. Pediatrics. 138(5):e20162043. https://doi.org/10.1542/peds.2016-2043; PMid:27940787

23. Raval MV, Wang X, Reynolds M, Fischer AC. 2011. Costs of congenital diaphragmatic hernia repair in the United States-extracorporeal membrane oxygenation foots the bill. J Pediatr Surg. 46(4):617-624. https://doi.org/10.1016/j.jpedsurg.2010.09.047; PMid:21496527

24. Razumovskiy AYu, Mokrushina OG, Beliayeva ID, Levitskaya MV, Shumikhin VS, Afukov II, Smirnova SV. 2012. Sravnitelniy analiz lecheniya novorozhdennykh s vrozhdennoy diafragmalnoy gryzhey posle plastiki diafragmy otkrytym i endoskopicheskim sposobami. Dersaya hirurgiya. (3):4-8.

25. Report from the Congenital Diaphragmatic Hernia Study Group. Report on Patients Born 1/1/2007 – 9/5/2019. Retrieved from Congenital Diaphragmatic Hernia Study Group, University of Texas Health Science Center at Houston.: https://med.uth.edu/pediatricsurgery/wp-content/uploads/sites/3/2019/09/CDHSG-Report-2007-2019.pdf

26. Ruano R, Takashi E, Da Silva VV, Campos JADB, Tannuri U, Zugaib M. 2012. Prediction and probability of neonatal outcome in isolated congenital diaphragmatic hernia using multiple ultrasound parameters. Ultrasound Obstet Gynecol. 39(1):42-49. https://doi.org/10.1002/uog.10095; PMid:21898639

27. Sananes N, Britto I, Akinkuotu AC, Olutoye OO, Cass DL, Sangi-Haghpeykar H, Lee TC, Cassady CI, Mehollin-Ray A, Welty S, Fernandes C, Belfort MA, Lee W, Ruano R. 2016. Improving the Prediction of Neonatal Outcomes in Isolated Left-Sided Congenital Diaphragmatic Hernia by Direct and Indirect Sonographic Assessment of Liver Herniation. J Ultrasound Med. 35(7):1437-1443. https://doi.org/10.7863/ultra.15.07020; PMid:27208195

28. Shanmugam H, Brunelli L, Botto LD, Krikov S, Feldkamp ML. 2017. Epidemiology and prognosis of congenital diaphragmatic hernia: a population-based cohort study in Utah. Birth Defects Research 109(18):1451-1459. https://doi.org/10.1002/bdr2.1106; PMid:28925604

29. Slepov OK, Ponomarenko OP, Soroka VP, Slepova LF, Khristenko VV, Gordienko IY, Tarapurova OM, Lutsenko SV, Dzham OP, Zhuravel AO. 2011. Prychiny pryrodnoji smertnosti novonarodzhenykh z pryrodzhenoiu diafragmalnoiu gtyzheiu. Perinatologiya i pediatriya 3:25-27.

30. The Canadian Congenital Diaphragmatic Hernia Collaborative; Puligandla PS, Skarsgard ED, Offringa M, Adatia I, Baird R, Bailey M, Brindle M, Chiu P, Cogswell A, Dakshinamurti S, Flageole H, Keijzer R, McMillan D, Oluyomi-Obi T, Pennaforte T, Perreault T, Piedboeuf B, Riley SP, Ryan G, Synnes A, Traynor M. 2018. Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline. CMAJ. 190(4):E103-E112. https://doi.org/10.1503/cmaj.170206; PMid:29378870 PMCid:PMC5790558

31. Tudorache S, Chiuţu LC, Iliescu DG, Georgescu R, Stoica GA, Simionescu CE, Georgescu EF, Nemeş RN. 2014. Prenatal diagnosis and perinatal outcome in congenital diaphragmatic hernia. Single tertiary center report. Rom J Morphol Embryol. 55(3):823-833.

32. Werneck Britto IS, Olutoye OO, Cass DL, Zamora IJ, Lee TC, Cassady CI, Mehollin-Ray A, Welty S, Fernandes C, Belfort MA, Lee W, Ruano R. 2015. Quantification of liver herniation in fetuses with isolated congenital diaphragmatic hernia using two-dimensional ultrasonography. Ultrasound Obstet Gynecol. 46:150-154. https://doi.org/10.1002/uog.14718; PMid:25366655