Congenital diaphragmatic hernia – a Belgrade single center experience

Jasna Kalanj; Petar Salevic; Snezana Rsovac; Biljana Medjo; Sanja Sindjic Antunovic; Dusica Simic

doi:10.1515/jpm-2015-0333

Article

Congenital diaphragmatic hernia – a Belgrade single center experience

Jasna Kalanj , Petar Salevic , Snezana Rsovac , Biljana Medjo , Sanja Sindjic Antunovic and Dusica Simic

Published/Copyright: March 17, 2016

Published by

Become an author with De Gruyter Brill

Submit Manuscript Author Information Explore this Subject

From the journal Journal of Perinatal Medicine Volume 44 Issue 8

Abstract

Objective:

Though the outcome for children with congenital diaphragmal hernia (CDH) is improving, management of seriously compromised respiratory and cardiovascular function remains a great challenge. The aim of this study was to review a tertiary center experience in treating children with CDH.

Design:

Retrospective observational study from January 2005 to December 2014.

Setting:

Neonatal Intensive Care Unit (NICU) of University Children Hospital (UCH), Belgrade, Serbia.

Patients:

Children with CDH.

Results:

The CDH was diagnosed prenatally in 23% patients. An overall survival rate was 62%. Among those patients who underwent surgical repair 29 (90%) survived. There was statistically significant difference in survival rate between operated patients and total examined population (P=0.020). Prenatally diagnosed neonates with CDH had significantly lower survival rate comparing to those who were postnatally diagnosed (20% vs. 75%; P=0.002). Fatal outcome was more frequent in patients with small birth weight comparing to those with normal birth weight (67% vs. 30%; P=0.046).

Conclusions:

Our center survival rate for CDH is in accordance with other reported studies. Based on our experience there are potential points for further improvement. First, further increase of prenatal detection, planning for delivery, and coordinated transfer to tertiary institution, in order to avoid transfer of near death patients. Second, preoperative management in the NICU. This could be done by more uniform implementation of current consensual guidelines in monitoring, mechanical ventilation and circulatory support of these delicate patients, together with rationale use of newer therapeutic resources.

Keywords: Congenital diaphragmatic hernia; neonatology; survival; treatment

Corresponding author: Petar Salevic, Pediatric and Neonatal Intensive Care Unit, University Children Hospital, Belgrade, Serbia, Tel.: +381692720957 (private); +381112060641 (work), E-mail: petar.salevic@udk.bg.ac.rs.

Funding statement: This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

References

[1] Mielniczuk M, Kusza K, Brzeziński P, Jakubczyk M, Mielniczuk K, Czerwionka-Szaflarska M. Current guidelines on management of congenital diaphragmatic hernia. Anaesthesiol Intensive Ther. 2012;44:232–7.Search in Google Scholar

[2] Wright J, Forster C, Sharkey D. Management of congenital diaphragmatic hernia. Nottingham Neonatal Service-Clinical Guidelines. Nottingham, UK. 2013. Available at: https://www.google.rs/url?sa=t&rct=j&q=&esrc=s&source=web&cd=1&cad=rja&uact=8&ved=0ahUKEwi7g-_izsjKAhWBWiwKHV8aBwwQFggfMAA&url=http%3A%2F%2Fwww.nuh.nhs.uk%2Fhandlers%2Fdownloads.ashx%3Fid%3D61198&usg=AFQjCNHXOXyjRe5pikSQxwydP2V1EsutmQ&bvm=bv.112766941,d.bGg.Search in Google Scholar

[3] Pober RB, Russell KM, Guernsey Ackerman K. Congenital Diaphragmatic Hernia Overview. GeneReviews^® [Internet]. Initial Posting: February 1, 2006; Last Update: March 16, 2010. Available online at: http://www.ncbi.nlm.nih.gov/books/NBK1359/.Search in Google Scholar

[4] Pober BR. Genetic aspects of human congenital diaphragmatic hernia. Clin Genet. 2008;74:1–15.10.1111/j.1399-0004.2008.01031.xSearch in Google Scholar PubMed PubMed Central

[5] Tovar JA. Congenital diaphragmatic hernia. Orphanet J Rare Dis. 2012;7:1.10.1186/1750-1172-7-1Search in Google Scholar PubMed PubMed Central

[6] Armstrong K, Franklin O, Molloy EJ. Congenital diaphragmatic hernia and congenital heart disease. In: Molloy EJ, editor. Congenital diaphragmatic hernia-prenatal to childhood management and outcomes. Rijeka, Croatia: InTech; 2012. p. 113–24. Available at: http://www.intechopen.com/books/congenital-diaphragmatic-hernia-prenatal-to-childhood-management-and-outcomes/congenital-diaphragmatic-hernia-and-congenital-heart-disease.10.5772/31825Search in Google Scholar

[7] Congenital diaphragmatic hernia. In NCCU Clinical Guidelines – Section 2: Respiratory problems and management. King Edward Memorial/Princess Margaret Hospitals, Perth Western Australia. Date created June 2006. Date last revised May 2015. Available at: http://www.kemh.health.wa.gov.au/services/nccu/guidelines/documents/2/CongenitalDiaphragmaticHernia%28CDH%29.pdf.Search in Google Scholar

[8] Reiss I, Schaible T, van den Hout L, Capolupo I, Allegaert K, van Heijst A, et al. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium consensus. Neonatology 2010;98:354–64.10.1159/000320622Search in Google Scholar PubMed

[9] Wynn J, Krishnan U, Aspelund G, Zhang Y, Duong J, Stolar CJ, et al. Outcomes of Congenital Diaphragmatic Hernia in the modern era of management. J Pediatr. 2013;163:114–9.10.1016/j.jpeds.2012.12.036Search in Google Scholar PubMed PubMed Central

[10] Kotecha S, Barbato A, Bush A, Davenport M, Delacourt C, Deprest J, et al. Congenital diaphragmatic hernia: ERS task force report. Eur Respir J. 2012;39:820–9.10.1183/09031936.00066511Search in Google Scholar PubMed

[11] Tracy ET, Mears SE, Smith PB, Danko, Danko ME, Diesen DL, et al. Protocolized approach to the management of congenital diaphragmatic hernia: benefits of reducing variability in care. J Pediatr Surg. 2010;45:1343–48.10.1016/j.jpedsurg.2010.02.104Search in Google Scholar PubMed PubMed Central

[12] Congenital Diaphragmatic Hernia: Management Guidelines. Duke Children’s Hospital and Health Center. Durham, North Carolina. 2009. Available at: http://www.dukechildrens.org/repository/dukechildrens/2009/11/03/14/08/38/7953/CDH_management_guidelines.pdf.Search in Google Scholar

[13] Snider AR, Serwer GA, Ritter SB. Echocardiography in pediatric heart disease. 2^nd ed. St Louis: Mosby-year book; 1997. p. 164–70.Search in Google Scholar

[14] Storme L, Aubry E, Rakza T, Houeijeh A, Debarge V, Tourneux P, et al. Patophysiology of persistent pulmonary hypertension of the newborn: Impact of the perinatal environment. Arch Cardiovasc Dis. 2013;106:169–77.10.1016/j.acvd.2012.12.005Search in Google Scholar PubMed

[15] Gentili A, Giuntoli L, Bacchi RM, Masciopinto F, Lima M, Baroncini S. Neonatal congenital diaphragmatic hernia: respiratory and blood-gas derived indices in choosing surgical timing. Minerva Anestesiol. 2012;78:1117–25.10.1155/2012/402170Search in Google Scholar

[16] Colvin J, Bower C, Dickinson JE, Sokol J. Outcomes of congenital diaphragmatic hernia: a population-based study in Western Australia. Pediatrics. 2005;116:356–63.10.1542/peds.2004-2845Search in Google Scholar PubMed

[17] Khemakhem R, Haggui B, Rahay H, Nouira F, Charieg A, Ghorbel S, et al. Congenital diaphragmatic hernia in neonate: a retrospective study about 28 observations. Afr J Paediatr Surg. 2012;9:217–22.10.4103/0189-6725.104723Search in Google Scholar PubMed

[18] Cadichon SB. Congenital diaphragmatic hernia. In: Praveen K, Burton BK, editors. Congenital malformations: evidence-based evaluation and management. New York, NY: McGraw-Hill Medical; 2008. p. 151–9.Search in Google Scholar

[19] Lee SYS, Tan KH. Antenatally diagnosed congenital diaphragmatic hernia in Singapore: a five-year series. Singapore Med J. 2013;54:432–6.10.11622/smedj.2013149Search in Google Scholar PubMed

[20] Werlein A, Goebert P, Maier RF. Planning delivery in cases of congenital malformations from the neonatologist’s point of view. Z Geburstshilfe Neonatol. 2014;218:27–33.10.1055/s-0034-1367052Search in Google Scholar

[21] Bialkowski A, Moenkemeyer F, Patel N. Intravenous sildenafil in the Management of Pulmonary Hypertension Associated with Congenital Diaphragmatic Hernia. Eur J Pediatr Surg. 2015;25:171–6.10.1055/s-0033-1357757Search in Google Scholar PubMed

[22] Yamamoto Y, Thebaud B, Vadivel A, Eaton F, Jain V, Hornberger LK. Doppler parameters of fetal lung hypoplasia and impact of Sildenafil. Am J Obstet gynecol. 2014;211:263.e1–8.10.1016/j.ajog.2014.03.013Search in Google Scholar PubMed

[23] Rocha G, Baptista MJ, Correia-Pinto J, Guimaraes H. Congenital diaphragmatic hernia: experience of 14 years. Minerva Pediatr. 2013;65:271–8.Search in Google Scholar

[24] Brindle ME, Cook EF, Tibboel D, Lally PA, Lally KP, Congenital diaphragmatic hernia study group. A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborn. Pediatrics. 2014;134:e413–9.10.1542/peds.2013-3367Search in Google Scholar PubMed

[25] Edmonds L, Boniface C, Alcock G, Stalewski H, Shi E. Congenital diaphragmatic hernia in Northern Queensland. J Paediatr Child Health. 2013;49:475–9.10.1111/jpc.12211Search in Google Scholar PubMed

[26] O’Mahony E, Stewart M, Sampson A, East C, Palma-Dias R. Perinatal outcome of congenital diaphragmatic hernia in an Australian tertiary hospital. Aust N J Z Obstet Gynaecol. 2012;52:189–94.10.1111/j.1479-828X.2011.01381.xSearch in Google Scholar PubMed

The authors stated that there are no conflicts of interest regarding the publication of this article.

Received: 2015-9-27

Accepted: 2016-2-17

Published Online: 2016-3-17

Published in Print: 2016-11-1

You are currently not able to access this content.

Articles in the same Issue

https://doi.org/10.1515/jpm-2015-0333

Keywords for this article

Congenital diaphragmatic hernia; neonatology; survival; treatment