Mortality prediction in congenital diaphragmatic hernia

doi:10.1016/j.jpedsurg.2007.12.012

Journal of Pediatric Surgery

Volume 43, Issue 5, May 2008, Pages 783-787

https://doi.org/10.1016/j.jpedsurg.2007.12.012 Get rights and content

Abstract

Background

A validated risk stratification tool for congenital diaphragmatic hernia (CDH) is required for accurate outcomes analyses. Existing mortality-predictive models include those of the CDH Study Group (CDHSG) based on birth weight and 5-minute Apgar score, the Canadian Neonatal Network (CNN) based on gestational age and admission score in Score for Neonatal Acute Physiology version II, and the Wilford Hall/Santa Rosa clinical prediction formula (WHSR_PF) derived from blood gas measurements. The purpose of this study was to evaluate the calibration and discrimination of these predictive models using the Canadian Pediatric Surgical Network dataset.

Methods

Neonatal risk variables and birth hospital survivorship were collected prospectively in 11 perinatal centers, between May 2005 and October 2006. Actual vs predicted outcomes were analyzed for each equation to measure the calibration and discrimination of each model.

Results

Twenty (21.2%) of 94 infants with CDH died during birth hospitalization. The CDHSG model demonstrated superior discrimination (area under the receiver operator characteristic curve = 0.85; CNN = 0.79; WHSR_PF = 0.63). Model calibration reflected by the Hosmer-Lemeshow P value was poorest with the WHSR_PF = 0.37 and comparable between CDHSG and CNN (0.48 and 0.46, respectively).

Conclusion

Predictive outcome models are essential for risk-adjusted outcome analysis of CDH. The ideal predictive equation should prove robust across CDH datasets.

Section snippets

Materials and methods

The dataset was obtained from the CAPSNET, a multidisciplinary group of 16 Canadian perinatal centers that collect prospective, disease-specific data on both CDH, and gastroschisis (Appendix 1). A perinatal center is defined as one with a level III neonatal intensive care unit (NICU), pediatric anesthesia, and subspecialty surgery (at least general and neurosurgery) capabilities, and a geographically or functionally adjoined maternal-fetal medicine/advanced prenatal diagnosis center. Pediatric

Descriptive characteristics and outcomes of CDH cohort

The total number of NICU admissions during the study period from the 11 CAPSNET contributing sites was 10,094. One hundred thirteen CDH cases were entered into the database, of which 105 were liveborn. Eleven patients had open files (meaning they remained alive in hospital), whereas 94 achieved the outcome criteria of death or discharge from the hospital of birth, and these represent the study cohort. Of these, 62 (66.0%) were diagnosed in the prenatal period. Babies were born at a mean GA of

Discussion

Congenital diaphragmatic hernia presents with a spectrum of disease severity that makes it difficult to accurately predict outcome. Although some neonates are born virtually asymptomatic, others require maximal NICU treatment strategies, including high frequency oscillatory ventilation, inhaled nitric oxide, and ECMO before operative repair [11], [12], [13], [14], [15], [16]. Predicting the specific interventions a patient is likely to require as well as their overall outcome based on early

Acknowledgments

This study was supported by grant FRN no. 69050 from the Canadian Institute of Health Research (Ontario, Canada). We thank all the local CAPSNET data abstractors for their work in data collection and Jennifer Claydon for her assistance in compiling the data.

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Cited by (55)

A novel congenital diaphragmatic hernia prediction model for Chinese subjects: A multicenter cohort investigation
2023, Heliyon
Brindle et al. (2014) and the Congenital Diaphragmatic Hernia Study Group constructed a simplified clinical prediction rule (Brindle score) to stratify infants with congenital diaphragmatic hernia based on disease severity. We aimed to develop a predictive model applicable to Chinese patients with congenital diaphragmatic hernia and externally validate whether the Brindle score is applicable to the Chinese population.
Multiple imputations supplemented the missing data. A least absolute shrinkage and selection operator regression was used to screen the factors influencing adverse outcomes. Internal validation was performed by bootstrap resampling. The C-index, area under the receiver operating characteristic curve, and the Hosmer-Lemeshow test evaluated the predictive power.
A nomogram named “CCDH score” (Chinese Congenital Diaphragmatic Hernia score), including pulmonary hypertension, low 5-min Apgar score (<7), chromosomal anomaly, major cardiac anomalies (MCAs), observed-to-expected lung-to-head ratio, and the percentage of liver herniation, was constructed. The CCDH score revealed good calibration and discriminative abilities, with a C-index of 0.941. In the training and external validation cohorts, the area under the receiver operating characteristic curve of the Brindle score were 0.820 and 0.881, respectively. The Brindle score has fair predictive power in the Chinese population, but the newly established CCDH score seems more suitable for Chinese patients with congenital diaphragmatic hernia.
The CCDH score is the first predictive model constructed based on the characteristics of the Chinese population and can accurately predict the survival outcomes of patients with congenital diaphragmatic hernia.
Sources of regional and center-level variability in survival and cost of care for congenital diaphragmatic hernia (CDH)
2021, Journal of Pediatric Surgery
Enormous variability in management and cost occurs in CDH care. The purpose of this study was to identify regional mortality and cost patterns underlying this variability.
This is a retrospective study of neonatal CDH patients at U.S. hospitals using data from the Pediatric Health Information System (PHIS) database (2015–2018). Patients were risk-stratified using CDH Study Group predicted survival (CDHSG-PS), and mortality and costs were assessed by region (East, West, Mid-West, and South) and center.
Higher mortality and extracorporeal life support (ECLS) rates were found in the Mid-West and South (p < 0.0001). Higher mortality was seen with ECLS among low-volume centers in the South (p = 0.007). When broken down by CHDSG-PS, higher severity patients had higher mortality in the Mid-West and South (p = 0.038). Cost was significantly lower for high severity nonsurvivors than survivors ($244,005 vs $565,487, p = 0.0008). The East spent more on high-severity patients with lower mortality compared to other regions, but also spent 3.5 times more on low severity nonsurvivors than survivors. Costs were higher at high-volume centers for low- and medium-severity patients, but all centers spent the same on high-severity patients.
Center volume, region, and patient severity all contribute to the complex survival and cost disparities that exist in CDH care. Standardization of care may improve survival and reduce cost variability.
Retrospective database study.
Level II
Prediction tools in congenital diaphragmatic hernia
2020, Seminars in Perinatology
Because congenital diaphragmatic hernia (CDH) is characterized by a spectrum of severity, risk stratification is an essential component of care. In both the prenatal and postnatal periods, accurate prediction of outcomes may inform clinical decision-making, care planning, and resource allocation. This review examines the history and utility of the most well-established risk prediction tools currently available, and provides recommendations for their optimal use in the management of CDH patients.
Prognostic value of the oxygenation index to predict survival and timing of surgery in infants with congenital diaphragmatic hernia
2019, Journal of Pediatric Surgery
To compare the mean oxygenation index on day1 (MOId1) with the best OI on day1 (BOId1) in predicting 30-day mortality in congenital diaphragmatic hernia (CDH). In addition, to determine whether serial OIs in the first 48 h after birth and preoperative OI (PreOp-OI), were associated with optimal timing of surgery, ventilation requirement and hospital stay in infants with CDH.
The medical records of infants with antenatally diagnosed CDH during 2009–2015 were retrospectively reviewed. Receiver operator characteristic curves were constructed to identify MOId1 and BOId1 cutoff levels to predict 30-day mortality. In those who underwent surgery, the mean OI on each of the first two days (MOId1, MOId2) and PreOp-OI were correlated using Spearman (rs) with the age at surgery, the duration of ventilation and length of stay. Statistical significance was P < 0.05* and < 0.001**.
Survivors (n = 44) and nonsurvivors (n = 24) were comparable in gestational age, birth weight and defect laterality. Nonsurvivors had higher median BOId1 (15.4 vs 2.9; P < 0.01) and MOId1 (48 vs 7.5; p < 0.01) than survivors. Mortality was best predicted by two cutoffs [BOId1 > 6 (sensitivity 92%, specificity 89%); and MOId1 > 17 (sensitivity 96%, specificity 96%)].
Forty-four infants underwent surgery at a median postnatal age of five (range 2–19) days. MOId1 and MOId2 both correlated significantly with the age at surgery (rs = 0.4**, rs 0.5**) but not ventilation period and length of stay. PreOp-OI correlated significantly with age at surgery, duration of ventilation and length of stay (rs = 0.32*, rs = 0.47**, rs = 0.37*). A PreOp-OI < 3 was predictive of optimal timing for surgery with improved duration of ventilation (8 vs 22, P = 0.001) and length of stay (26 vs 47, P = 0.004). However, 11/44 (25%) patients would not achieve a PreOp-OI < 3 by day 7 + of life and might still require surgery, one of them died.
Both MOId1 and BOId1 are highly predictive of mortality in CDH. Oxygenation indices in the first 48 h poorly predicted the timing of surgery. PreOp-OI < 3 may be a cutoff for optimal timing for surgery in infants for CDH repair.
Prognostic Study.
Level III.
Risk-stratification enables accurate single-center outcomes assessment in congenital diaphragmatic hernia (CDH)
2019, Journal of Pediatric Surgery
Citation Excerpt :
This may simply reflect a center-specific lack of disparities in care or relative uniformity in the quality of regional prenatal and perinatal care. Multivariable analysis showed that ECMO use and blood gas markers of severe pulmonary hypertension and poor gas exchange were strong predictors of mortality, in keeping with multiple previous models examining mortality risk in CDH [6,18–21]. Unsurprisingly then, the ECMO risk model, which incorporates arterial PCO2 levels, was found to perform well at our center.
Management of CDH is highly variable from center to center, as are patient outcomes. The purpose of this study was to examine risk-stratified survival and extracorporeal membrane oxygenation (ECMO) rates at a single center, and to determine whether adverse outcomes are related to patient characteristics or management.
A retrospective single-center review of CDH patients was performed, and outcomes compared to those reported by the CDH Study Group (CDHSG) registry. Patient demographics, disparities, and clinical characteristics were examined to identify unique features of the cohort. A model derived using the registry that estimates probability of ECMO use or death in CDH newborns was used to risk-stratify patients and assess mortality rates. Observed over expected (O/E) ECMO use rates were calculated to measure whether “excess” or “appropriate” ECMO use was occurring.
There were 81 CDH patients treated between 2004–2017, and 5034 in the CDHSG registry. Mortality in ECMO-treated patients was higher than the registry. Socioeconomic variables were not significantly associated with outcomes. The strongest predictors of mortality were ECMO use and early blood gas variables. The risk model accurately predicted ECMO use with a c-statistic of 0.79. Compared with the registry, the disparity in mortality rates was greatest for moderate-risk patients. O/E ECMO use was highest in low and moderate-risk patients.
ECMO use is a more consistent predictor of mortality than CDH severity at a single center, and there is relative overuse of ECMO in lower-risk patients. Risk stratification allows for more accurate institutional assessment of mortality and ECMO use, and other centers could consider such an adjusted analysis to identify opportunities for outcomes improvement.
III.
Prenatal prediction of survival in congenital diaphragmatic hernia: An audit of postnatal outcomes
2019, Journal of Pediatric Surgery
Citation Excerpt :
Predictive outcomes were binned into severe (o/e ≤ 25%), moderate (o/e 26–45%), and mild (o/e > 45%) based upon prediction categories described in the TOTAL Trial [15]. A secondary analysis was performed to compare postnatal predictors of survival, including the CDH Study group (CDHSG) equation to prenatal predictors [16]. The CDHSG equation is a probability equation for survival based upon birth weight and the 5-min Apgar score that was developed and validated with multi-institutional registry data [17,18].
Effective antenatal counseling in congenital diaphragmatic hernia (CDH) relies on proper measurement of prognostic indices. This quality initiative audited the accuracy of prenatal imaging with postnatal outcomes at two tertiary pediatric referral centers.
Prenatal lung–head ratio (LHR) and total fetal lung volume (TFLV) for CDH patients treated between 2006 and 2017 were retrieved. Study inclusion required at least one LHR or TFLV measurement between 24 and 32 weeks gestational age. Postnatal outcomes [mortality, extracorporeal life support (ECLS) need, patch repair, persistent pulmonary hypertension, oxygen requirement at 28 days] were abstracted from the Canadian Pediatric Surgery Network (CAPSNet) database and local chart review. Univariate and descriptive analyses were conducted.
Eighty-two of 121 eligible CDH patients (68%) were included. Overall mortality, ECLS rates, and patch repair were 33%, 12.5%, and 45%, respectively. Lower LHR values correlated with increased rates of each outcome and persisted despite multiple measurements. Values obtained were higher than those in published schemata. LHR values > 45% were most associated with survival, avoidance of ECLS, and primary repair. TFLV values only correlated with mortality and patch repair.
This audit confirms that LHR and TFLV values predict CDH outcomes. However, absolute values obtained require careful interpretation and internal review.
IV.

View all citing articles on Scopus

: Presented at the 39th Annual Meeting of the Canadian Association of Pediatric Surgeons, August 23-26, 2007, St John's Newfoundland, Canada.

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CAPS original papersMortality prediction in congenital diaphragmatic hernia

Abstract

Background

Methods

Results

Conclusion

Section snippets

Materials and methods

Descriptive characteristics and outcomes of CDH cohort

Discussion

Acknowledgments

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr

J Pediatr Surg

J Pediatr

Arch Pediatr

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

CAPS original papers
Mortality prediction in congenital diaphragmatic hernia