Abstract
Purpose
The features of right-sided congenital diaphragmatic hernias (RCDHs) are quite different from those of left-sided CDHs (LCDHs). We have summarized the features of RCDHs experienced in our institution.
Methods
This retrospective study analyzed the cases of patients with CDH registered at our institution between 2011 and 2020. Defects on each side were compared based on prenatal diagnosis, medical treatment, type of surgery, and outcomes.
Results
A total of 101 patients underwent surgery at our institution during the neonatal period, and 11 had RCDHs. RCDHs and LCDHs were significantly different in terms of extracorporeal membrane oxygenation (36% vs. 6%, p = 0.002), patch repair (81% vs. 28%, p < 0.001), recurrence rate (36% vs. 11%, p = 0.022), and length of hospital stay (117 days vs. 51 days, p = 0.012). The severity of the fetal diagnosis did not reflect postnatal severity. All patients with RCDH survived to discharge, and there was no significant difference in survival rate between the right and left sides.
Conclusion
Neonates with RCDH required more intensive treatments; however, the survival rate was comparable between RCDH and LCDH. RCDH was significantly different from LCDH and an optimal treatment strategy for RCDHs should be established.
Similar content being viewed by others
References
Nagata K, Usui N, Kanamori Y, Takahashi S, Hayakawa M, Okuyama H, Inamura N, Fujino Y, Taguchi T (2013) The current profile and outcome of congenital diaphragmatic hernia: a nationwide survey in Japan. J Pediatr Surg 48:738–744. https://doi.org/10.1016/j.jpedsurg.2012.12.017
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 29:276–283. https://doi.org/10.1002/uog.3863
Boloker J, Bateman DA, Wung JT, Stolar CJ (2002) Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair. J Pediatr Surg 37:357–366. https://doi.org/10.1053/jpsu.2002.30834
Javid PJ, Jaksic T, Skarsgard ED, Lee S, Network CN (2004) Survival rate in congenital diaphragmatic hernia: the experience of the Canadian Neonatal Network. J Pediatr Surg 39:657–660. https://doi.org/10.1016/j.jpedsurg.2004.01.022
Congenital Diaphragmatic Hernia Study Group, Lally KP, Lally PA, Lasky RE, Tibboel D, Jaksic T, Wilson JM, Frenckner B, Van Meurs KP, Bohn DJ, Davis CF, Hirschl RB (2007) Defect size determines survival in infants with congenital diaphragmatic hernia. Pediatrics 120:e651–e657. https://doi.org/10.1542/peds.2006-3040
Migliazza L, Bellan C, Alberti D, Auriemma A, Burgio G, Locatelli G, Colombo A (2007) Retrospective study of 111 cases of congenital diaphragmatic hernia treated with early high-frequency oscillatory ventilation and presurgical stabilization. J Pediatr Surg 42:1526–1532. https://doi.org/10.1016/j.jpedsurg.2007.04.015
Lazar DA, Cass DL, Rodriguez MA, Hassan SF, Cassady CI, Johnson YR, Johnson KE, Johnson A, Moise KJ, Belleza-Bascon B, Olutoye OO (2011) Impact of prenatal evaluation and protocol-based perinatal management on congenital diaphragmatic hernia outcomes. J Pediatr Surg 46:808–813. https://doi.org/10.1016/j.jpedsurg.2011.02.009
Beaumier CK, Beres AL, Puligandla PS, Skarsgard ED, Network CPS (2015) Clinical characteristics and outcomes of patients with right congenital diaphragmatic hernia: a population-based study. J Pediatr Surg 50:731–733. https://doi.org/10.1016/j.jpedsurg.2015.02.027
Skari H, Bjornland K, Haugen G, Egeland T, Emblem R (2000) Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 35:1187–1197. https://doi.org/10.1053/jpsu.2000.8725
Fisher JC, Jefferson RA, Arkovitz MS, Stolar CJ (2008) Redefining outcomes in right congenital diaphragmatic hernia. J Pediatr Surg 43:373–379. https://doi.org/10.1016/j.jpedsurg.2007.10.049
Daher P, Zeidan S, Azar E, Khoury M, Melki I, Mikhael R (2003) Right congenital diaphragmatic hernia a well-known pathology? Pediatr Surg Int 19:293–295. https://doi.org/10.1007/s00383-002-0845-9
Midrio P, Gobbi D, Baldo V, Gamba P (2007) Right congenital diaphragmatic hernia: an 18-year experience. J Pediatr Surg 42:517–521. https://doi.org/10.1016/j.jpedsurg.2006.10.044
Hedrick HL, Crombleholme TM, Flake AW, Nance ML, von Allmen D, Howell LJ, Johnson MP, Wilson RD, Adzick NS (2004) Right congenital diaphragmatic hernia: prenatal assessment and outcome. J Pediatr Surg 39:319–323. https://doi.org/10.1016/j.jpedsurg.2003.11.006
Colvin J, Bower C, Dickinson JE, Sokol J (2005) Outcomes of congenital diaphragmatic hernia: a population-based study in Western Australia. Pediatrics 116:e356–e363. https://doi.org/10.1542/peds.2004-2845
Wilson JM, Lund DP, Lillehei CW, Vacanti JP (1997) Congenital diaphragmatic hernia–a tale of two cities: the Boston experience. J Pediatr Surg 32:401–405. https://doi.org/10.1016/s0022-3468(97)90590-x
Kim AG, Norwitz G, Karmakar M, Ladino-Torres M, Berman DR, Kreutzman J, Treadwell MC, Mychaliska GB, Perrone EE (2020) Discordant prenatal ultrasound and fetal MRI in CDH: wherein lies the truth? J Pediatr Surg 55:1879–1884. https://doi.org/10.1016/j.jpedsurg.2019.11.007
Schaible T, Kohl T, Reinshagen K, Brade J, Neff KW, Stressig R, Büsing KA (2012) Right- versus left-sided congenital diaphragmatic hernia: postnatal outcome at a specialized tertiary care center. Pediatr Crit Care Med 13:66–71. https://doi.org/10.1097/PCC.0b013e3182192aa9
Jani JC, Benachi A, Nicolaides KH, Allegaert K, Gratacós E, Mazkereth R, Matis J, Tibboel D, Van Heijst A, Storme L, Rousseau V, Greenough A, Deprest JA, Antenatal-CDH-Registry group (2009) Prenatal prediction of neonatal morbidity in survivors with congenital diaphragmatic hernia: a multicenter study. Ultrasound Obstet Gynecol 33:64–69. https://doi.org/10.1002/uog.6141
Tsao K, Lally KP (2008) The Congenital Diaphragmatic Hernia Study Group: a voluntary international registry. Semin Pediatr Surg 17:90–97. https://doi.org/10.1053/j.sempedsurg.2008.02.004
Usui N, Nagata K, Hayakawa M, Okuyama H, Kanamori Y, Takahashi S, Inamura N, Taguchi T (2014) Pneumothoraces as a fatal complication of congenital diaphragmatic hernia in the era of gentle ventilation. Eur J Pediatr Surg 24:31–38. https://doi.org/10.1055/s-0033-1357753
Jaffray B, MacKinlay GA (1996) Real and apparent mortality from congenital diaphragmatic hernia. Br J Surg 83:79–82. https://doi.org/10.1002/bjs.1800830125
Touloukian RJ, Markowitz RI (1984) A preoperative x-ray scoring system for risk assessment of newborns with congenital diaphragmatic hernia. J Pediatr Surg 19:252–257. https://doi.org/10.1016/s0022-3468(84)80180-3
Gibson C, Fonkalsrud EW (1983) Iatrogenic pneumothorax and mortality in congenital diaphragmatic hernia. J Pediatr Surg 18:555–559. https://doi.org/10.1016/s0022-3468(83)80358-3
Harrison MR, Bjordal RI, Langmark F, Knutrud O (1978) Congenital diaphragmatic hernia: the hidden mortality. J Pediatr Surg 13:227–230. https://doi.org/10.1016/s0022-3468(78)80391-1
Azarow K, Messineo A, Pearl R, Filler R, Barker G, Bohn D (1997) Congenital diaphragmatic hernia—A tale of two cities: the Toronto experience. J Pediatr Surg 32:395–400. https://doi.org/10.1016/s0022-3468(97)90589-3
Laudy JA, Van Gucht M, Van Dooren MF, Wladimiroff JW, Tibboel D (2003) Congenital diaphragmatic hernia: an evaluation of the prognostic value of the lung-to-head ratio and other prenatal parameters. Prenat Diagn 23:634–639. https://doi.org/10.1002/pd.654
Kitano Y, Nakagawa S, Kuroda T, Honna T, Itoh Y, Nakamura T, Morikawa N, Shimizu N, Kashima K, Hayashi S, Sago H (2005) Liver position in fetal congenital diaphragmatic hernia retains a prognostic value in the era of lung-protective strategy. J Pediatr Surg 40:1827–1832. https://doi.org/10.1016/j.jpedsurg.2005.08.020
Muratore CS, Kharasch V, Lund DP, Sheils C, Friedman S, Brown C, Utter S, Jaksic T, Wilson JM (2001) Pulmonary morbidity in 100 survivors of congenital diaphragmatic hernia monitored in a multidisciplinary clinic. J Pediatr Surg 36:133–140. https://doi.org/10.1053/jpsu.2001.20031
Byrne F, Keller R, Meadows J, Miniati D, Brook MM, Silverman NH, Moon-Grady AJ (2015) Severe left diaphragmatic hernia limits size of fetal left heart more than does right diaphragmatic hernia. Ultrasound Obstet Gynecol 46:688–694. https://doi.org/10.1002/uog.14790
Rasanen J, Wood DC, Weiner S, Ludomirski A, Huhta JC (1996) Role of the pulmonary circulation in the distribution of human fetal cardiac output during the second half of pregnancy. Circulation 94:1068–1073. https://doi.org/10.1161/01.cir.94.5.1068
Friedman WF (1972) The intrinsic physiologic properties of the developing heart. Prog Cardiovasc Dis 15:87–111. https://doi.org/10.1016/0033-0620(72)90006-0
Momma K, Ando M, Mori Y, Ito T (1992) Hypoplasia of the lung and heart in fetal rats with diaphragmatic hernia. Fetal Diagn Ther 7:46–52. https://doi.org/10.1159/000263650
Peralta CF, Cavoretto P, Csapo B, Vandecruys H, Nicolaides KH (2005) Assessment of lung area in normal fetuses at 12–32 weeks. Ultrasound Obstet Gynecol 26:718–724. https://doi.org/10.1002/uog.2651
Cannie M, Jani J, Meersschaert J, Allegaert K, Done’ E, Marchal G, Deprest J, Dymarkowski S (2008) Prenatal prediction of survival in isolated diaphragmatic hernia using observed to expected total fetal lung volume determined by magnetic resonance imaging based on either gestational age or fetal body volume. Ultrasound Obstet Gynecol 32:633–639. https://doi.org/10.1002/uog.6139
Acknowledgements
None
Funding
The authors did not receive support from any organization for the submitted work.
Author information
Authors and Affiliations
Contributions
MO: participated in the planning, conducting of the study, interpretation of data, collection and writing of manuscript. HU and HA: contributed to interpretation and critically reviewed the manuscript. All the authors approved the final submitted draft of this manuscript.
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that there are no conflicts of interest regarding the publication of this paper.
Ethics approval
This study was performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.
Consent to participate/publish
Informed consent was obtained from legal guardians.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Okamoto, M., Amano, H., Uchida, H. et al. Clinical characteristics and outcomes of the right congenital diaphragmatic hernia compared to the left: a 10-year single-center experience. Pediatr Surg Int 37, 1675–1681 (2021). https://doi.org/10.1007/s00383-021-04999-8
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-021-04999-8