Right-sided congenital diaphragmatic hernia – the experience of the neonatal surgery center

Authors

  • O. Sliеpov Center for neonatal surgery for malformations and their rehabilitation SI «Institute of Pediatrics, Obstetrics and Gynecology named after academician O.M. Lukyanovа NAMS of Ukraine», Ukraine
  • O. Ponomarenko Center for neonatal surgery for malformations and their rehabilitation SI «Institute of Pediatrics, Obstetrics and Gynecology named after academician O.M. Lukyanovа NAMS of Ukraine», Ukraine
  • M. Migur Center for neonatal surgery for malformations and their rehabilitation SI «Institute of Pediatrics, Obstetrics and Gynecology named after academician O.M. Lukyanovа NAMS of Ukraine», Ukraine
  • L. Sliеpova Center for neonatal surgery for malformations and their rehabilitation SI «Institute of Pediatrics, Obstetrics and Gynecology named after academician O.M. Lukyanovа NAMS of Ukraine», Ukraine
  • O. Gladyshko Center for neonatal surgery for malformations and their rehabilitation SI «Institute of Pediatrics, Obstetrics and Gynecology named after academician O.M. Lukyanovа NAMS of Ukraine», Ukraine
  • G. Golopapa Center for neonatal surgery for malformations and their rehabilitation SI «Institute of Pediatrics, Obstetrics and Gynecology named after academician O.M. Lukyanovа NAMS of Ukraine», Ukraine

DOI:

https://doi.org/10.15574/PS.2020.69.13

Keywords:

congenital diaphragmatic hernia, right-sided, herniation of the liver, critical hypoplasia of the lungs, surgical correction, newborn baby

Abstract

Congenital right-sided diaphragmatic hernias are a special form of diaphragmatic hernias. A relatively low frequency and features of anatomy, diagnosis clinical course, and survival characterize them.

Objective: to analyze the results of treatment of right-sided diaphragmatic hernias in newborns.

Materials and methods: a retrospective analysis of the medical records and autopsy protocols of 22 newborns with right-sided diaphragm defect, who were in the clinics of the State Institution «IPAG named after academician O.M. Lukyanova of the National Academy of Medical Sciences of Ukraine», was carried out for the last 37 years.

Results: in 3 cases stillbirth was stated, in all live-born children (n=19) with right-sided СDH the defect was symptomatic. Moreover, in 84.2% (n=16) of them, symptoms of the disease occurred immediately after birth, in 10.5% (n=2) – from the first to 6 hours of life, in 5.3% (n=1) – after 24 hours, from birth. Signs of pulmonary hypertension were determined based on the difference between pre- and postductal peripheral blood saturation. Thus, in 62.5% (n=5) of operated children and in 63.6% (n=7) who died at the stages of stabilization, the difference in pre- and postductal saturation was more than 10%, whichindicated the presence of 100% pulmonary hypertension, and blood shunting from right to left, through fetal communications. The time of preoperative stabilization ranged from 1 to 23 days, on average 7.25 days. 8 children were operated. The surgical approach was right-sided subcostal laparotomy (n=5) or right-sided thoracotomy (n=2). In one case, there was a combined approach: right-sided laparotomy + right-sided thoracotomy. 5 children survived, 3 died.

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 participating institution.

 

References

Abdelmohs1. Abramov A, Fan W, Hernan R, Farkouh-Karoleski C, Chung WK, Duron V. (2020). Comparative outcomes of right versus left congenital diaphragmatic hernia: A multicenter analysis. J Pediatr Surg. 55(1): 33-38. https://doi.org/10.1016/j.jpedsurg.2019.09.046; PMid:31677822

Berdan EA, Saltzman DA. (2012). Right‐versus left‐sided congenital diaphragmatic hernia-can we trust the data? Pediatr Crit Care Med. 13: 103-104. https://doi.org/10.1097/PCC.0b013e31823886ee; PMid:22222649

Brindle ME, Cook EF, Tibboel D, Lally PA, Lally KP. (2014). A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns Pediatrics. 134: 413-419. https://doi.org/10.1542/peds.2013-3367; PMid:25022745

Brownlee EM, Howatson AG, Davis CF et al. (2009). The hidden mortality of congenital diaphragmatic hernia: a 20-year review. Journal of pediatric surgery. 44: 317-320. https://doi.org/10.1016/j.jpedsurg.2008.10.076; PMid:19231525

Bryner BS, Kim AC, Khouri JS et al. (2009). Right-sided congenital diaphragmatic hernia: high utilization of extracorporeal membrane oxygenation and high survival. Journal of pediatric surgery. 44: 883-887. https://doi.org/10.1016/j.jpedsurg.2009.01.037; PMid:19433162

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? Journal of pediatric surgery. 53: 113-117. https://doi.org/10.1016/j.jpedsurg.2017.10.027; PMid:29122292

Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S. (2017). Congenital Diaphragmatic hernia - a review. Matern Health Neonatol Perinatol. 11;3:6. https://doi.org/10.1186/s40748-017-0045-1; PMid:28331629 PMCid:PMC5356475

Colvin J, Bower C, Dickinson JE, Sokol J. (2005). Outcomes of congenital diaphragmatic hernia: a population-based study in Western Australia Pediatrics. 116: 63-356. https://doi.org/10.1542/peds.2004-2845; PMid:16140678

DeKoninck P, Gomez O, Sandaite I et al. (2015). Right-sided congenital diaphragmatic hernia in a decade of fetal surgery. BJOG: An International Journal of Obstetrics & Gynaecology. 122: 940-946. https://doi.org/10.1111/1471-0528.13065; PMid:25227954

Done E, Gucciardo L,van Mieghem T, Jani J, Cannie M, van Schinbroeck D, Devlieger R, de Catte L, Kloritsch P, Mayer S, Beck V, Debcer A, Gratacos E, Micolaides K, Deprest J. (2008). Prenatal diagnosis, prediction of outcome and in utero therapy of isolated congenital diaphragmatic hernia. Prenatal. Diagn. 28: 581-591. https://doi.org/10.1002/pd.2033; PMid:18634116

Duess JW, Zani-Ruttenstock EM, Garriboli M et al. (2015). Outcome of right-sided diaphragmatic hernia repair: a multicentre study. Pediatric surgery international. 31: 465-471. https://doi.org/10.1007/s00383-015-3695-y; PMid:25801417

Loo CK, Pearen MA, Pereira TN et al. (2018). Lung and liver growth and retinoic acid status in human fetuses with congenital diaphragmatic hernia. Early human development. 116: 17-23. https://doi.org/10.1016/j.earlhumdev.2017.10.005; PMid:29096166

Lorenzo D. (2013). Botto. International Clearinghouse for Birth Defects Surveillance and Research annual report 2012 Rome. International Clearinghouse for Birth Defects Surveillance and Research. Italy. 250.

Mayorga-Buiza MJ, Mata J, Santigosa Garcia М, Antinolo G, Lopez Ontanilla A, Marenco М. (2014). Ex utero intrapartum treatment procedure for scheduled repair of moderate-severe isolated congenital diaphragmatic hernia: our experience. European Journal of Anaesthesiology (EJA). 31: 187. https://doi.org/10.1097/00003643-201406001-00532

O'Mahony E, Stewart M, Sampson A, East C, Palma-Dias R. (2012). Perinatal outcome of congenital diaphragmatic hernia in an Australian tertiary hospital Aust New Zeal. J. Obstet. Gynaecol. 52: 94-189. https://doi.org/10.1111/j.1479-828X.2011.01381.x; PMid:22145569

Pramod S, Puligandla Julia Grabowski, Mary Austin. (2015). Management of congenital diaphragmatic hernia: A systematic review from the APSA outcomes and evidence based practice committee. Journal of Pediatric Surgery. 50 (11): 1958-1970. https://doi.org/10.1016/j.jpedsurg.2015.09.010; PMid:26463502

Praveen Kumar Chandrasekharan, Munmun Rawat, Rajeshwari Madappa, David H. Rothstein, Satyan Lakshminrusimha. (2017). Congenital Diaphragmatic hernia - a review. Matern Health Neonatol Perinatol. 3: 6. https://doi.org/10.1186/s40748-017-0045-1; PMid:28331629 PMCid:PMC5356475

Samangaya RA, Choudhri S, Murphy F, Zaidi T, Gillham JC, Morabito A. (2012). Outcomes of congenital diaphragmatic hernia: a 12-year experience Prenat Diagn. 32: 523-529. https://doi.org/10.1002/pd.3841; PMid:22499217

Santos LR, Maksoud-Filho JG, Tannuri U, Andrade WC, Maksoud JG. (2003). Prognostic factors and survival in neonates with congenital diaphragmatic hernia. J. Pediatr. 79: 81-86. https://doi.org/10.2223/JPED.942; PMid:12973514

Schaible T, Kohl T, Reinshagen K, Brade J, Neff KW, Stressig R et al. (2012). Right - versus left‐sided congenital diaphragmatic hernia. Pediatr Crit Care Med. 13: 66-71. https://doi.org/10.1097/PCC.0b013e3182192aa9; PMid:21478793

Silva RI do Carmo, Peixoto-Filho FM, Bueno A, Fonseca M, Saint Clair dos Santos Gomes Junior. (2019). Prognostic factors of death in children during the first year of life due to congenital diaphragmatic hernia: analysis of a hospital cohort from 2005 to 2015. Jornal de Pediatria. 96(5): 569-575. https://doi.org/10.1016/j.jped.2019.03.005; PMid:31029681

Skari Hans, Bjornland Kristin, Frenckner Bjorn, Sandgren Katarina, Wester Tomas, Emblem Ragnhild. (2002). Congenital diaphragmatic hernia in Scandinavia from 1995 to 1998: Predictors of mortality. J Pediatr Surg. 37(9): 1269-1275. https://doi.org/10.1053/jpsu.2002.34980; PMid:12194115

Skari Hans, Bjornland Kristin, Haugen Guttorm, Egeland Thore, Emblem Ragnhild. (2000). Congenital diaphragmatic hernia: A meta-analysis of mortality factors. Journal of Pediatric Surgery. 35(8): 1187-1197. https://doi.org/10.1053/jpsu.2000.8725; PMid:10945692

Slavotinek AM, Warmerdam B, Lin AE et al. (2007). Populationbased analysis of left-and right-sided diaphragmatic hernias demonstrates different frequencies of selected additional anomalies. American Journal of Medical Genetics Part A. 143: 3127-3136. https://doi.org/10.1002/ajmg.a.32100; PMid:18008313

Slepov OK. (2010). Clinical and morphological classification pulmonary hypoplasia of congenital diaphragmatic hernia in newborns. Pediatrics, obstetrics and gynecology. 4: 132.

Slepov OK, Veselsky VL, Ponomarenko OP et al. (2012). Modern approaches treatment of congenital diaphragmatic hernias in newborns. Collection of scientific works «Topical issues of treatment of children with surgical pathology»: 77-78.

Slepov OK, Ponomarenko OP, Soroka VP et al. (2010). Anatomical features and influence on the survival of newborns with congenital diaphragmatic hernias. Pediatric surgery. 3(28): 45-50.

Snoek KG, Reiss IK, Greenough A, Capolupo I, Urlesberger B, Wessel L et al. (2016). Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium Consensus - 2015 update Neonatology. 110: 66-74. https://doi.org/10.1159/000444210; PMid:27077664

Spaggiari E, Stirnemann JJ, Sonigo P, Khen-Dunlop N, Saint Blanquat L, Ville Y. (2015). Prenatal prediction of pulmonary arterial hypertension in congenital diaphragmatic hernia Ultrasound Obstet Gynecol. 45: 572-577. https://doi.org/10.1002/uog.13450; PMid:24976012

Published

2020-12-30

Issue

Section

Original articles. Neonatal surgery