Anatomical variants of congenital diaphragmatic hernia, their clinical significance and feasibility of prenatal differentiation

Authors

  • G.O. Grebinichenko SI «Institute of Pediatrics, Obstetrics and Gynecology named after academical O.M. Luk'yanova of NAMS of Ukraine», Kyiv, Ukraine https://orcid.org/0000-0003-4391-6724
  • I.Y. Gordienko SI «Institute of Pediatrics, Obstetrics and Gynecology named after academical O.M. Luk'yanova of NAMS of Ukraine», Kyiv, Ukraine https://orcid.org/0000-0001-7594-4880
  • O.K. Sliepov SI «Institute of Pediatrics, Obstetrics and Gynecology named after academical O.M. Luk'yanova of NAMS of Ukraine», Kyiv, Ukraine https://orcid.org/0000-0002-6976-1209
  • A.O. Zhuravel SI «Institute of Pediatrics, Obstetrics and Gynecology named after academical O.M. Luk'yanova of NAMS of Ukraine», Kyiv, Ukraine

DOI:

https://doi.org/10.15574/PP.2020.84.19

Keywords:

congenital diaphragmatic hernia, congenital malformations, prenatal diagnosis

Abstract

Purpose — to present verified typical anatomical variants of isolated congenital diaphragmatic hernia and clinical outcomes in newborns depending on the type of pathology, to compare with data of prenatal examination, and to assess feasibility of prenatal differentiation of congenital diaphragmatic hernia.

Materials and methods. The data of operation protocols and autopsy results of newborn patients with isolated congenital diaphragmatic hernia for the period 2007–2020 were analyzed, and then compared with prenatal exam data and clinical outcomes. Data from different anatomical variants of congenital diaphragmatic hernia were analyzed using descriptive statistics methods.

Results. Anatomical data were evaluated in 67 cases with the following typical variants: left-sided non-communicating defect (20.9%), left-sided communicating with herniation of intestine (19.4%), intestine and stomach (26,9%), intestine, stomach and liver (19.4%, 13/67), right- sided communicating with intestine and liver herniation (10.4%), right- sided non-communicating (1.5%), bilateral communicating defects (1.5%). Mortality at the stage of stabilization in these variants was 0%, 0%, 11.1%, 30.8%, 71.4%, 0% and 100%, postoperative mortality, respectively, 7.1%, 0%, 12.5%, 44.4%, 0%, 0% (excluding bilateral hernia), total mortality 7.1%, 0%, 22.2%, 61.5%, 71.4%, 0%, 100%. Comparison of lung indices in patients with left-sided hernias showed their similarity in groups with non-communicating defects and communicating with herniation of intestine. Significant differences were found in the groups with herniation of the intestine and stomach, and intestines, stomach and liver. The mean liver-to-lung ratio in right-sided communicating defects was 3.7±1.9, in left-sided communicating defects 1.7±0.8 and in non-communicating 0.44±0.25, the difference between all groups was highly significant. Patterns of stomach position in different variants of pathology were determined.

Conclusions. Analysis of postnatally verified cases of diaphragmatic hernia showed marked anatomical variability. The highest mortality and the lowest rate of surgical correction registered was in communicating right-sided defects, and in communicating left-sided with simultaneous herniation of the intestine, stomach and liver. The best outcomes were found in non-communicating defects, or in communicating with herniation of intestine. Prenatal evaluation of stomach position may be the basis to differentiation between clinico-anatomical variants of the pathology.

The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the Institution. The informed consent of the patient was obtained for conducting the studies.

No conflict of interest was declared by the authors.

References

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

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.

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

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

Cohen-Katan S, Newman-Heiman N, Staretz-Chacham O, Cohen Z, Neumann L, Shany E. (2009). Congenital diaphragmatic hernia: short-term out-come. Isr Med Assoc J. 11 (4): 219-224.

Cordier AG, Russo FM, Deprest J, Benachi A. (2020). Prenatal diagnosis, imaging, and prognosis in Congenital Diaphragmatic Hernia. Semin Perinatol. 44 (1): 51163. https://doi.org/10.1053/j.semperi.2019.07.002; PMid:31439324

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.

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. URL: http://rdrt.com.ua/index.php/journal/article/view/242. https://doi.org/10.37336/2707-0700-2019-4-1

Gordienko IY, Grebinichenko GO, Tarapurova OM. (2020). Variants of stomach position in different types of congenital diaphragmatic hernia in the fetus. Radiation Diagnostics, Radiation Therapy. 2: 7-17. https://doi.org/10.37336/2707-0700-2020-2-1

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

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. doi 10.15574/PP.2019.80.10.

Hasegawa T, Kamata S, Imura K, Ishikawa S, Okuyama H, Okada A, Chiba Y. (1990). Use of lung-thorax transverse area ratio in the antenatal evaluation of lung hypoplasia in congenital diaphragmatic hernia. J Clin Ultrasound. 18: 705-709. https://doi.org/10.1002/jcu.1990.18.9.705; PMid:2174921

Ibirogba ER, Novoa Y, Novoa VA, Sutton LF, Neis AE, Marroquin AM, Coleman TM, Praska KA, Freimund TA, Ruka KL, Warzala VL, Sangi-Haghpeykar H, Ruano R. (2019). Standardization and reproducibility of sonographic stomach position grades in fetuses with congenital diaphragmatic hernia. J Clin Ultrasound. 47 (9): 513-517. https://doi.org/10.1002/jcu.22759; PMid:31313328

Jani J, Nicolaides KH, Keller RL, Benachi A, Peralta CF, Favre R, Moreno O, Tibboel D, Lipitz S, Eggink A, Vaast P, Allegaert K, Harrison M, Deprest J, Antenatal-CDH-Registry Group. (2007). Observed to expected lung area to head circumference ratio in the prediction of survival in fetuses with isolated diaphragmatic hernia. Ultrasound Obstet Gynecol. 30 (1): 67-71. https://doi.org/10.1002/uog.4052; PMid:17587219

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

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

Laudy JA, Wladimiroff JW. (2000). The fetal lung. 2: Pulmonary hypoplasia. Ultrasound Obstet Gynecol. 16 (5): 482-494. https://doi.org/10.1046/j.1469-0705.2000.00252.x; PMid:11169336

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

Metkus AP, Filly RA, Stringer MD, Harrison MR, Adzick NS. (1996). Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg. 31 (1): 148-151. https://doi.org/10.1016/S0022-3468(96)90338-3

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

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

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.

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

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

Sarac M, Bakal U, Tartar T, Canpolat S, Kara A, Kazez A. (2018). Bochdalek hernia and intrathoracic ectopic kidney: Presentation of two case reports and review of the literature. Niger J Clin Pract. 21 (5): 681-686. https://doi.org/10.4103/njcp.njcp_217_17; PMid:29735873

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.

Snoek KG, Reiss IK, Greenough A, Capolupo I, Urlesberger B, Wessel L, Storme L, Deprest J, Schaible T, van Heijst A, Tibboel D, CDH EURO Consortium. (2016). Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus - 2015 Update. Neonatology. 110 (1): 66-74. https://doi.org/10.1159/000444210; PMid:27077664

Spaggiari E, Stirnemann J, Bernard JP, De Saint Blanquat L, Beaudoin S, Ville Y. (2013). Prognostic value of a hernia sac in congenital diaphragmatic hernia. Ultrasound Obstet Gynecol. 41 (3): 286-290. https://doi.org/10.1002/uog.11189; PMid:22605546

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

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

Downloads

Published

2020-12-30

Issue

No. 4(84) (2020): Ukrainian journal of Perinatology and Pediatrics

Section

Original articles

License

The policy of the Journal “Ukrainian Journal of Perinatology and Pediatrics” is compatible with the vast majority of funders' of open access and self-archiving policies. The journal provides immediate open access route being convinced that everyone – not only scientists - can benefit from research results, and publishes articles exclusively under open access distribution, with a Creative Commons Attribution-Noncommercial 4.0 international license(СС BY-NC).

Authors transfer the copyright to the Journal “MODERN PEDIATRICS. UKRAINE” when the manuscript is accepted for publication. Authors declare that this manuscript has not been published nor is under simultaneous consideration for publication elsewhere. After publication, the articles become freely available on-line to the public.

Readers have the right to use, distribute, and reproduce articles in any medium, provided the articles and the journal are properly cited.

The use of published materials for commercial purposes is strongly prohibited.