Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 
  • Users Online: 284
  • Home
  • Print this page
  • Email this page
Cover page of the Journal of Health Sciences


 
 Table of Contents  
ORIGINAL ARTICLE
Year : 2021  |  Volume : 14  |  Issue : 3  |  Page : 365-369

Factors affecting outcome in neonates with congenital diaphragmatic hernia at tertiary care center


1 Department of Pediatric Surgery, Faculty of Medical Sciences, Khaja Bandanawaz University, Kalaburagi, Karnataka, India
2 Department of Pediatrics, Faculty of Medical Sciences, Khaja Bandanawaz University, Kalaburagi, Karnataka, India

Date of Submission13-Jul-2021
Date of Acceptance26-Jul-2021
Date of Web Publication30-Sep-2021

Correspondence Address:
Dr. Nandkishor Dhanvantrao Shinde
Block C, F-1, Asian Gardenia, Kalaburagi, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kleuhsj.kleuhsj_190_21

Rights and Permissions
  Abstract 

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a major congenital anomaly requiring surgical intervention after birth. Due to improvements in antenatal diagnosis and perinatal management with well advanced neonatal intensive care, there are better overall survival rates in the developed countries. However, the outcome is still poor in developing countries where multiple factors contribute to higher morbidity and mortality.
AIM: The aim of the study is to evaluate various factors affecting the perioperative management and their outcome in neonates with CDH.
MATERIALS AND METHODS: This descriptive observational study was conducted over a period of 4 years. Neonates who underwent surgery for CDH were included. Demographic data, clinical data, gender, birth weight, period of gestation, antenatal diagnosis records, additional congenital anomalies, initial blood gases in the first 24 h of life, stomach or liver herniation in the thorax, postoperative complications, and final outcome were recorded.
RESULTS: During our study period, 36 neonates underwent surgery for CDH. Associated anomalies were detected in 47.2% cases. Persistent pulmonary hypertension was seen in 44.4% cases. Sepsis (55.5%) was the most common complication. Mortality in our study was 64%. Persistent pulmonary hypertension (93.75%) was the most common factor associated with mortality followed by sepsis (80%). Antenatal diagnosis before 20 weeks had the highest mortality (85.7%). Liver or stomach as a thoracic content in diaphragmatic hernia had high mortality.
CONCLUSION: Sepsis, shock, hypothermia, metabolic acidosis, and pneumonitis were the preventable factors affecting outcome in CDH. Improving pediatric surgical facilities and neonatal care can improve survival in developing countries.

Keywords: Anomalies, congenital diaphragmatic hernia, lung hypoplasia, mortality, pulmonary hypertension


How to cite this article:
Shinde ND, Uplaonkar V, Koneru V, Akhtar M. Factors affecting outcome in neonates with congenital diaphragmatic hernia at tertiary care center. Indian J Health Sci Biomed Res 2021;14:365-9

How to cite this URL:
Shinde ND, Uplaonkar V, Koneru V, Akhtar M. Factors affecting outcome in neonates with congenital diaphragmatic hernia at tertiary care center. Indian J Health Sci Biomed Res [serial online] 2021 [cited 2021 Nov 29];14:365-9. Available from: https://www.ijournalhs.org/text.asp?2021/14/3/365/327250


  Introduction Top


Congenital diaphragmatic hernia (CDH) is a significant congenital developmental anomaly caused by the failure of the pleuroperitoneal canal to close, resulting in improper lung development and function. It occurs in about one out of every 2500–3000 births.[1],[2]

Multiple genetic factors, environmental exposures, and nutritional deficiencies are proposed etiological factors for CDH. The condition could be an isolated anomaly or it could be associated with severe congenital anomalies such as cardiac, gastrointestinal, and genitourinary malformations, as well as chromosomal aneuploidy like trisomies.[3],[4]

CDH is most frequent on the left side (85%), with the right side (13%) and bilateral hernias being less common (2%). Posterolateral diaphragmatic hernias also known as Bochdalek hernias are the most common type (70%–75%).[1]

Lung hypoplasia and immaturity associated with persistent pulmonary hypertension have been the possible pathophysiology of CDH.[5] Outcome of neonates with CDH depends upon multiple factors such as prematurity, associated congenital anomalies, severity of pulmonary hypertension, pulmonary hypoplasia, and need for extracorporeal membrane oxygenation (ECMO).[6]

In recent decades, improvement in fetal diagnosis, fetal intervention, and neonatal intensive care advancements like, mechanical ventilation, high frequency ventilation, conventional and cycled continuous positive airway pressure, for the management of CDH neonates has increased survival significantly in developed countries currently up to 75%–80%. However, even after improvement in the management of CDH, the mortality remains high when compared to other congenital anomalies.[7],[8]

Developing countries lack these advanced facilities in most centers, and hence, the outcome is expected to be poor compared to developed countries.

The aim of this study is to evaluate the factors affecting surgical and perioperative management and its outcome in neonates with CDH at a tertiary care institute in a resource-constrained developing country.


  Materials and Methods Top


This descriptive observational study was conducted over a period of 4 years from June 2017 to May 2021. Neonates who underwent surgery for CDH at our institute were included in the study. Neonates who refused surgery, died without surgery, or surgery beyond neonatal period (30 days) were excluded from the study.

All diagnosed neonates with respiratory distress were electively intubated and kept on ventilator (synchronized intermittent-mandatory ventilation was used with peak inspiratory pressure [PIP] of 18–30 cmH2O). Depending on the condition of neonates, inotropic agents (e.g. dobutamine, dopamine 5–10 μg/kg/min) and pulmonary vasodilators were administered. In all neonates, diagnosis was confirmed with postnatal chest X-ray. Two-dimensional echocardiography, ultrasound abdomen and thorough clinical examination were done to rule out associated anomalies. After stabilization of neonates, surgery with abdominal approach with subcostal incision was performed. Content herniated into thorax was noted and reduced into the abdominal cavity, intercostal drain was kept, and diaphragmatic defect was closed. Postoperatively neonates with respiratory distress were kept on supportive ventilation. Chest tube was removed on the 3rd to 5th postoperative day. Follow-up was done weekly for 1 month, then every 3 monthly for 1 year.

Demographic data, clinical data, gender, birth weight, period of gestation, antenatal diagnosis records, type of delivery, additional congenital anomaly, initial blood gases in the first 24 h of life, stomach or liver herniation in the thorax, postoperative complications, and final outcome were recorded.

Categorical variables were summarized as percentages and quantitative variables were summarized as mean with standard deviation, or median with inter-quartile range according to the distribution of the variable. The data were entered and analyzed in Epi Info version 7 software (Centers for Disease Control and Prevention (CDC), Atlanta, Georgia. United States federal agency). Ethical committee clearence taken from Khaja Bandanawaz institute of Medical Sciences, Kalaburagi with reference no. KBNIMS/IEC/2017-18/71.


  Results Top


During the study period, 36 neonates underwent surgery for CDH at our institute. Out of 36 neonates, 25 (69%) were males and 11 (31%) were females with a male-to-female ratio of 2.3:1. Mean birth weight of neonates was 2400 ± 900 g. Mean gestation age was 36 ± 4 weeks with 19 (52.8%) neonates being born full term (≥37 weeks). On antenatal ultrasonography screening, antenatal diagnosis was made in 32 cases (88.8%) at a mean age of 28 ± 6 weeks of gestation. 7 (21.8%) cases detected before 20 weeks of gestation, 14 (43.8%) detected between 21 weeks and 28 weeks, and 11 (34.4%) after 28 weeks of gestation. 29 (80.6%) of them were born through cesarean section (due to maternal indication). There were no familial cases.

28 (77.8%) neonates had respiratory distress at presentation and were kept on elective ventilation preoperatively. Seven (19.4%) neonates presented with shock and 9 (25%) were hypothermic at the time of presentation to the institute.

Average blood gas analysis results in the initial 24 h of life in survived and nonsurvived neonates are shown in [Table 1].
Table 1: Blood gas analysis in the first 24 h of life in neonates with congenital diaphragmatic hernia

Click here to view


Associated anomalies were detected in 17 (47.2%) cases, on echocardiography 8 (22.2%) neonates had congenital heart disease, the most common being ventricular septal defect 3 (37.5%). In rest of the nine cases (25%), there were other anomalies, in which 5 (13.9%) had malrotation of gut, 2 (5.5%) had renal anomalies, 1 had hemivertebra, and 1 had trisomy 21.

All neonates underwent diaphragmatic hernia repair surgery with abdominal approach. 36 (100%) neonates had left-sided diaphragmatic defect in our study. Left lobe of liver was in thorax in 5 (13.8%) cases, stomach was in thorax in 19 (52.8%) cases, spleen was in thorax in 25 (69.4%) cases, and 5 (13.9%) cases had malrotation of gut, which underwent Ladds procedure intraoperatively in the same setting. The average duration of postoperative conventional mechanical ventilation was 80 ± 9 h in survived neonates as compared to 48 ± 8 in nonsurvived neonates. PIP was 23.85 ± 8.62 cm of H2O in those who did not survive compared to 19.54 ± 5.15 cm of H2O in cases who survived. ECMO and nitric oxide (NO) treatment modalities were not use to stabilize neonates before or after surgery due to nonavailability at our institute. Average duration of surgery was 60–90 min in our study. Mean duration of postoperative hospital stay was 17 ± 5 days.

Persistent pulmonary hypertension was seen in 16 (44.4%) cases which increased the mortality further. Sepsis (55.5%) was the most common postoperative complication followed by pneumonitis [Table 2]. We were able to manage these complications conservatively.
Table 2: Postoperative complication

Click here to view


Postoperative mortality in our study was 23 (64%) with survival rate of 36%. Various factors associated with postoperative mortality are described in [Table 3]. Persistent pulmonary hypertension (93.75%) was the most common factor associated with mortality followed by sepsis (80%) [Table 3]. Antenatal diagnosis before 20 weeks had highest mortality (85.7%) compared to delayed diagnosis in our study. Liver or stomach as a thoracic content in diaphragmatic hernia had high mortality [Table 3].
Table 3: Factors associated with postoperative mortality

Click here to view


Survival was 70% in neonates with birth weight more than 2500 g. Postoperative survival rate was 65% in term infants. 57.2% of the neonates with shock at presentation survived to discharge. Similarly, 66.7% neonates with hypothermia survived.


  Discussion Top


CDH contributes 8% of all major congenital anomalies.[9] In majority of cases, CDH is symptomatic at birth and is considered as a surgical emergency. However, poor pulmonary functions lead to a poor outcome, and hence, stabilization of neonates with ventilator support and management of pulmonary hypertension is necessary before surgery.[10] The management of these neonates is a challenge and requires a multidisciplinary approach.[11]

Prenatal diagnosis of CDH is possible, by detecting the abnormal location of the liver, stomach, or other viscera in the fetal thorax. In our study, antenatal diagnosis was made in 88.8% cases; however, prenatal detection rate varies enormously from 10% to 79% in different published studies.[6],[12],[13] Early antenatal diagnosis had high mortality rate and survival was better if diagnosed after 25 weeks.[6],[7],[8],[9],[10],[11],[12],[13] Similarly, in our study, we observed that antenatal diagnosis before 20 weeks had highest mortality of 85.7% compared to delayed diagnosis.

Survival was 70% in neonates with birth weight more than 2500 g and 65% in term infants in our study. However, birth weight, sex, gestational age did not show any significant effect on survival in most of the similar studies.[14],[15] However, in a study by Kadir and Lilja,[16] it is observed that mortality decreased by 7% for each 100 g increase in birth weight.

Respiratory distress (77.8%) was the most common presentation in our study, which is also a common presentation in almost all similar studies.[1],[10],[11]

Neonates requiring low FiO2 and achieving higher PaO2 had better survival and neonates with metabolic acidosis with low PH <7.15, high PaCO2 (>60 mm Hg), low PaO2, and high pulmonary pressure requiring high PIP were associated with higher mortality in our study, similar to other studies.[17],[18],[19]

In our study, associated congenital anomalies were detected in 47.2% cases and congenital heart disease was the most common anomaly in 22.2% neonates. 70.6% mortality was seen in neonates with associated congenital anomalies; other studies also show similar findings with high mortalities in neonates with CDH associated with other congenital anomalies.[1],[7],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20]

In our study, persistent pulmonary hypertension (93.75%) was the most common factor associated with mortality, other studies also show similar finding.[1],[10],[11],[12],[18],[19],[20]

Liver or stomach as a thoracic content in diaphragmatic hernia had high mortality (80%) in our study probably due to distinctive pulmonary hypoplasia, which is also seen in other studies.[1],[21],[22]

In our study, sepsis (55.5%) was the most common postoperative complication followed by pneumonitis (27.8%). Persistent pulmonary hypertension (93.75%) was the most common factor associated with mortality followed by sepsis (80%). 57.2% of the neonates with shock and 66.7% neonates with hypothermia survived; these findings were also seen in other studies.[1],[7],[10],[11],[17],[21],[22] Other factors affecting outcome in our study were early antenatal detection, associated anomalies, herniation of liver or stomach in thorax, metabolic acidosis, and high PIP requirements; similar finding was also seen in other studies.[1],[10],[11],[21],[22] Sepsis, shock, hypothermia, metabolic acidosis, and pneumonitis were the preventable factors affecting outcome in neonates with CDH.

In literature, wide variations in survival rates occur in different subsets of neonates with CDH. Postoperative mortality in our study was 64% which is higher as compared to developed countries (20%–25%) where advanced facilities such as high-frequency jet ventilation and ECMO are available.[7],[8],[23],[24],[25] Morini et al.[26] demonstrated that mortality rates declined from 83.5% to 38.3% when ECMO treatment was applied in patients unresponsive to conventional management.[26] In our study, a higher mortality rate might be due to nonavailability of high-frequency jet ventilation, ECMO, or NO at our institute.


  Conclusion Top


Sepsis, shock, hypothermia, metabolic acidosis, and pneumonitis were the preventable factors affecting outcome in CDH. Persistent pulmonary hypertension, associated anomalies, and lung hypoplasia were uncontrollable factors causing poor outcome. Improving pediatric surgical facilities and neonatal care services can improve survival in developing countries.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Chandrasekharan PK, Rawat M, Madappa R, Rothstein DH, Lakshminrusimha S. Congenital diaphragmatic hernia – A review. Matern Health Neonatol Perinatol 2017;3:6.  Back to cited text no. 1
    
2.
Ameis D, Khoshgoo N, Keijzer R. Abnormal lung development in congenital diaphragmatic hernia. Semin Pediatr Surg 2017;26:123-8.  Back to cited text no. 2
    
3.
Beurskens LW, Tibboel D, Lindemans J, Duvekot JJ, Cohen-Overbeek TE, Veenma DC, et al. Retinol status of newborn infants is associated with congenital diaphragmatic hernia. Pediatrics 2010;126:712-20.  Back to cited text no. 3
    
4.
Wat MJ, Veenma D, Hogue J, Holder AM, Yu Z, Wat JJ, et al. Genomic alterations that contribute to the development of isolated and non-isolated congenital diaphragmatic hernia. J Med Genet 2011;48:299-307.  Back to cited text no. 4
    
5.
Greer JJ. Current concepts on the pathogenesis and etiology of congenital diaphragmatic hernia. Respir Physiol Neurobiol 2013;189:232-40.  Back to cited text no. 5
    
6.
Graham G, Devine PC. Antenatal diagnosis of congenital diaphragmatic hernia. Semin Perinatol 2005;29:69-76.  Back to cited text no. 6
    
7.
Brownlee EM, Howatson AG, Davis CF, Sabharwal AJ. The hidden mortality of congenital diaphragmatic hernia: A 20-year review. J Pediatr Surg 2009;44:317-20.  Back to cited text no. 7
    
8.
Losty PD. Congenital diafragmatic hernia: Where and what is the evidence? Semin Pediatr Surg 2014;23:278-82.  Back to cited text no. 8
    
9.
Doyle NM, Lally KP. The CDH study group and advances in the clinical care of the patient with congenital diaphragmatic hernia. Semin Perinatol 2004;28:174-84.  Back to cited text no. 9
    
10.
Jain A, Singh V, Sharma M. Congenital diaphragmatic hernia our experience – A brief review. Indian J Anaesthesiol 2002;46:426-9.  Back to cited text no. 10
    
11.
McHoney M. Congenital diaphragmatic hernia, management in the newborn. Pediatr Surg Int 2015;31:1005-13.  Back to cited text no. 11
    
12.
Chan DK, Ho LY, Joseph VT. Mortality among infants with high-risk congenital diaphragmatic hernia in Singapore. J Pediatr Surg 1997;32:95-8.  Back to cited text no. 12
    
13.
Metkus AP, Filly RA, Stringer MD, Harrison MR, Adzick NS. Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg 1996;31:148-51.  Back to cited text no. 13
    
14.
Larsen UL, Jepsen S, Strøm T, Qvist N, Toft P. Congenital diaphragmatic hernia presenting with symptoms within the first day of life; outcomes from a non-ECMO centre in Denmark. BMC Pediatr 2020;20:196.  Back to cited text no. 14
    
15.
Hoffman SB, Massaro AN, Gingalewski C, Short BL. Predictors of survival in congenital diaphragmatic hernia patients requiring extracorporeal membrane oxygenation: CNMC 15-year experience. J Perinatol 2010;30:546-52.  Back to cited text no. 15
    
16.
Kadir D, Lilja HE. Risk factors for postoperative mortality in congenital diaphragmatic hernia: A single-centre observational study. Pediatr Surg Int 2017;33:317-23.  Back to cited text no. 16
    
17.
Molugan M, Kamalarathnam CN, Muthukumaran J. Clinical profile of congenital diaphragmatic hernia and their short-term outcome in a tertiary care neonatal unit: A retrospective study. Indian J Child Health 2017 4:435-7.  Back to cited text no. 17
    
18.
Osiovich HC. Improving survival of neonates with isolated congenital diaphragmatic hernia. Indian Pediatr 2004;41:1138-42.  Back to cited text no. 18
    
19.
Snoek KG, Reiss IK, Greenough A, Capolupo I, Urlesberger B, Wessel L, et al. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: The CDH EURO consortium consensus – 2015 update. Neonatology 2016;110:66-74.  Back to cited text no. 19
    
20.
Graziano JN; Congenital Diaphragmatic Hernia Study Group. Cardiac anomalies in patients with congenital diaphragmatic hernia and their prognosis: A report from the congenital diaphragmatic hernia study group. J Pediatr Surg 2005;40:1045-9.  Back to cited text no. 20
    
21.
Duess JW, Zani-Ruttenstock EM, Garriboli M, Puri P, Pierro A, Hoellwarth ME. Outcome of right-sided diaphragmatic hernia repair: A multicentre study. Pediatr Surg Int 2015;31:465-71.  Back to cited text no. 21
    
22.
Bojanić K, Woodbury JM, Cavalcante AN, Grizelj R, Asay GF, Colby CE, et al. Congenital diaphragmatic hernia: Outcomes of neonates treated at Mayo Clinic with and without extracorporeal membrane oxygenation. Paediatr Anaesth 2017;27:314-21.  Back to cited text no. 22
    
23.
Ozdogan T, Durakbasa C, Mutus M, Iscen M. Congenital diaphragmatic hernia: A 4-year experience in a single centre. Afr J Paediatr Surg 2010;7:105-6.  Back to cited text no. 23
[PUBMED]  [Full text]  
24.
Kuluz MA, Smith PB, Mears SP, Benjamin JR, Tracy ET, Williford WL, et al. Preliminary observations of the use of high-frequency jet ventilation as rescue therapy in infants with congenital diaphragmatic hernia. J Pediatr Surg 2010;45:698-702.  Back to cited text no. 24
    
25.
Kattan J, Godoy L, Zavala A, Faunes M, Becker P, Estay A, et al. Improvement of survival ininfants with congenital diaphragmatic hernia in recent years: Effect of ECMO availability and associated factors. Pediatr Surg Int 2010;26:671-6.  Back to cited text no. 25
    
26.
Morini F, Goldman A, Pierro A. Extracorporeal membrane oxygenation in infants with congenital diaphragmatic hernia: A systematic review of the evidence. Eur J Pediatr Surg 2006;16:385-91.  Back to cited text no. 26
    



 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
   Abstract
  Introduction
   Materials and Me...
  Results
  Discussion
  Conclusion
   References
   Article Tables

 Article Access Statistics
    Viewed180    
    Printed10    
    Emailed0    
    PDF Downloaded20    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]