Apparent truth about congenital diaphragmatic hernia: a population-based database is needed to establish benchmarking for clinical outcomes for CDH

doi:10.1016/j.jpedsurg.2004.01.032

Journal of Pediatric Surgery

Volume 39, Issue 5, May 2004, Pages 661-665

Presented at the 35th Annual Meeting of the Canadian Association of Paediatric Surgeons, Niagara-on-the-Lake, Ontario, Canada, September 18–21, 2003.

https://doi.org/10.1016/j.jpedsurg.2004.01.032 Get rights and content

Abstract

Background

The authors hypothesize that recent single or multiinstitution-based reports of improved survival of congenital diaphragmatic hernia (CDH) patients are biased by patient selection, practice, and referral patterns. Here the authors report a population-based analysis of the clinical outcomes of CDH in the province of Ontario for 1996.

Methods

A retrospective analysis of cross-sectional data from the Bureau of Vital Statistics of Ontario and all 5 pediatric surgical institutions in Ontario for 1996 was performed.

Results

Twenty-four CDH-associated deaths were registered in Canada in 1996. Fourteen of 24 occurred in Ontario (58.3%). Of 30 institutionally identified CDH in Ontario, 8 patients died (26.7%). CDH-associated infant mortality rate was 6.6 of 100,000 live births in Canada compared with 10 of 100,000 live births for Ontario (Relative risk, 1.4; confidence interval, 0.5, 3.7; P > .01). Neonatal death (<28 postnatal days) accounted for the majority, 13 of 14 (92.8%) of deaths. Six of 14 (42.9%) CDH-associated deaths, however, were not accounted by the institutional-based reporting. In addition, institutional-based survival rates for CDH varied from 62.5% to 100%.

Conclusions

Our results indicate the existing bias associated with institution-based reporting and database of CDH. The “hidden mortality” associated with CDH is still present. A population-based database is needed to establish the benchmarking for management of CDH.

Section snippets

Materials and methods

The Bureau of Vital Statistics has compiled a database of postnatal deaths beyond 20 weeks’ gestation, causes of death, and live births in Canada. Using these statistics, the number of CDH-associated deaths in Canada and Ontario was recorded for the year 1996. Approximately 40% of the national population resides in this geographically defined area with a pediatric population of 2.6 million (based on 1996 Census data). Deaths related to CDH per 100,000 live births were also noted. The Ontario

Patient characteristics

In 1996, a total of 30 cases of CDH were identified in all participating pediatric institutions in Ontario. These cases represent a complete and inclusive number for all CDH in the geographically defined area, the Province of Ontario. To determine whether these patients from the multiinstitutions were comparable with previously reported patients in the literature, we compared our patient characteristics with those in 3 recent large CDH case series (Table 1). 1, 2, 4 Patient age and weight at

Discussion

Over the last decade, various single institutional-based case series have reported a significant improvement in the survival rate of patients with CDH compared with previous periods.1, 2, 4 This improvement has been attributed to various factors, including the use of inhaled nitric oxide, high-frequency oscillatory ventilation, extracorporeal membrane oxygenation, permissive hypercapnea, exogenous surfactant therapy, and corticosteroid therapy.3, 8, 9 Despite the plethora of major pediatric

References (23)

K. Azarow et al.
Congenital diaphragmatic hernia—A tale of two citiesThe Toronto experience
J Pediatr Surg
(1997)
J. Boloker et al.
Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair
J Pediatr Surg
(2002)
J.M. Wilson et al.
Congenital diaphragmatic hernia—A tale of two citiesThe Boston experience
J Pediatr Surg
(1997)
D.O. Fauza et al.
Congenital diaphragmatic hernia and associated anomaliesTheir incidence, identification, and impact on prognosis
J Pediatr Surg
(1994)
N.P. Smith et al.
Congenital diaphragmatic hernia
Pediatr Respir Rev
(2002)
J.C. Langer et al.
Timing of surgery for congenital diaphragmatic herniaIs emergency operation necessary?
J Pediatr Surg
(1988)
B. Frenckner et al.
Improved results in patients who have congenital diaphragmatic hernia using preoperative stabilization, extracorporeal membrane oxygenation, and delayed surgery
J Pediatr Surg
(1997)
C. Reyes et al.
Delayed repair of congenital diaphragmatic hernia with early high-frequency oscillatory ventilation during preoperative stabilization
J Pediatr Surg
(1998)
R.H. Clark et al.
Current surgical management of congenital diaphragmatic herniaA report from the Congenital Diaphragmatic Hernia Study Group
J Pediatr Surg
(1998)
M.R. Harrison et al.
Congenital diaphragmatic herniaThe hidden mortality
J Pediatr Surg
(1978)

H. Sakai et al.

Effect of surgical repair on respiratory mechanics in congenital diaphragmatic hernia

J Pediatr Surg

(1987)

Cited by (44)

A single-center observational study on congenital diaphragmatic hernia: Outcome, predictors of mortality and experience from a tertiary perinatal center in Singapore
2020, Pediatrics and Neonatology
Citation Excerpt :
Our study indicated a survival rate of 78.9% without ECMO in infants with CDH at our institution. Our survival is comparable with other international centers and higher than the mean overall survival reported from the CDH Study Group Registry (67%).22 Our results demonstrated a good outcome despite the lack of ECMO resources.
Congenital diaphragmatic hernia (CDH) is a common birth defect associated with significant mortality and morbidity. There is limited outcome data on CDH in the Southeast Asian region. Rapid accessibility to our CDH Perinatal Center, as a consequence of the small geographic size of our country and efficient land transportation system, has largely eliminated deaths of live outborn babies prior arrival at our center. We selected a study period when extracorporeal membrane oxygenation (ECMO) support was not available at our institution. The data will therefore be relevant in developing management guidelines and antenatal counselling for perinatal centers in this region managing CDH with limited resources, without ECMO facilities.
A retrospective study of antenatally or postnatally diagnosed CDH infants born between January 2002 and June 2005 was performed. We selected this study period as ECMO support was not available over this period. We studied the demographics, clinical characteristics, postnatal predictors of mortality and outcomes of CDH infants in a single tertiary institution.
A total of 24 patients with CDH were identified. Seventy-nine percent of liveborns with CDH survived to hospital discharge. Antenatal detection rate was 83.3%. Significant postnatal predictors of mortality were preoperative pneumothorax (p = 0.035), high CRIB score (p = 0.007), low one- and five-minute Apgar score (p = 0.011, p = 0.026 respectively) and high pCO2 on initial arterial blood gas (p = 0.007). At one-year follow-up, three patients had delayed gross motor milestones which resolved subsequently. Re-admissions were required for recurrent bronchiolitis (33%) and oesophageal reflux which resolved in all cases. Two (13.3%) infants had surgical complications and needed re-admission for probable adhesive intestinal obstruction; one required adhesiolysis and the other was managed conservatively with good outcome.
A single-center CDH outcome in Singapore, without ECMO use, was good. This is a cohort now with long-term survival outcome which will be valuable to the neonatology community.
Addressing the hidden mortality in CDH: A population-based study
2017, Journal of Pediatric Surgery
Improvements in the clinical management of CDH have led to overall improved reported result from single institutions. However, population-based studies have highlighted a hidden mortality.
To explore the incidence in Sweden and to address the hidden mortality for CDH during a 27-year period in a population-based setting.
This is a population based cohort study that includes all patients diagnosed with CDH that were registered in the National Patient Register, the Medical Birth Register, the Register of Congenital Malformations and the Register for Causes of Death between 1987 and 2013.
The mortality rates were calculated based on the number deaths divided by the number of live born cases. The hidden mortality was defined as the number of CDH cases that were not born (because of TOP or IUFD), cases of neonatal demise during birth or demise the same day of birth in patients who were in peripheral institutions and who never reached tertiary centers.
In total, 861 CDH patients were born in Sweden between 1987 and 2013, which corresponds to an incidence of 3.0 born CDH per 10,000 live births. When adding the cases of TOP and IUFD, the total incidence of CDH in Sweden was 3.5/10,000 live born. The mortality rate between 1987 and 2013 was 36%: 44% during the first time period 1987–1999 and 27% in the later period 2000–2013. The hidden mortality in the second period was 30%, resulting in a total mortality rate of 45%.
The incidence of CDH during a 27-year period remained unchanged in the population. However, we observed a decrease in the prevalence because of the increasing numbers of TOP. A significant hidden mortality exists, with overall mortality rate of 45% for CDH in this population.
II (cohort).
Congenital diaphragmatic hernia: Not quite there yet
2013, Journal of Pediatrics
Management of the Infant with Congenital Diaphragmatic Hernia
2012, The Newborn Lung: Neonatology Questions and Controversies Expert Consult
Management of the Infant with Congenital Diaphragmatic Hernia
2012, The Newborn Lung
Survival disparities in newborns with congenital diaphragmatic hernia: a national perspective
2010, Journal of Pediatric Surgery
Citation Excerpt :
More than two thirds of all births with CDH occurred at NIACH, but most of these patients were transferred to other hospitals. Nevertheless, 32% of all deaths occurred at NIACH consistent with population-based studies [4,18-21]. In addition, there was no significant difference in mortality rates between hospital types once transfers were excluded.
The aim of the study was to examine national outcomes for congenital diaphragmatic hernia (CDH).
We analyzed the Kids' Inpatient Database for patients admitted at less than 8 days of age.
Overall, 2774 hospitalizations were identified. Most patients were white and had private insurance. Most patients were treated at urban (96%), teaching (75%), and not identified as children's hospital (NIACH) (50%). Birth was the most common admission source at NIACH (91%) and children's unit in general hospital (CUGH) (59%), compared to hospital transfer at children's general hospital (CGH) (81%). Most CDH were repaired through the abdomen (81%), and 25% required extracorporeal membrane oxygenation (ECMO). Most NIACH patients were transferred to another hospital, whereas most at CGH and CUGH were discharged home. Survival to discharge was 66% after excluding hospital transfers. Univariate analysis revealed higher survival for males, birth weight (BW) of 3 kg or more, whites, patients with private insurance, and those in the highest median household income quartile. Survival was 86% after CDH repair but 46% for ECMO. Multivariate analysis identified black race (hazard ratio [HR], 1.536; P = .03) and other race (HR, 1.515; P = .03) as independent predictors of mortality.
Hospital survival for CDH is related to sex, BW, race, and socioeconomic status. Blacks and other non-Hispanic minorities have higher mortality rates.

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^∗: Leslie Scott, London Health Sciences Center, London Ontario; Douglas Mah, Brian Cameron, McMaster University, Hamilton, Ontario; Noelle Grace, North York General Hospital, Toronto, Ontario; Juan Bass, Children’s Hospital of Eastern Ontario, Ottawa, Ontario; Dan Panaeru, Queen’s University, Kingston, Ontario, and Peter Masiokos, Desmond Bohn, Paul Wales, and Peter C. W. Kim, Hospital for Sick Children, Toronto, Ontario, Canada.

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Apparent truth about congenital diaphragmatic hernia: a population-based database is needed to establish benchmarking for clinical outcomes for CDH

Abstract

Background

Methods

Results

Conclusions

Section snippets

Materials and methods

Patient characteristics

Discussion

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