Absolute vs relative improvements in congenital diaphragmatic hernia survival: what happened to “hidden mortality”

Mah, Kandice; Zamakhshary, Mohammed; Mah, Doug; Cameron, Brian H.; Bass, Juan; Bohn, Desmond; Scott, Leslie A.; Himidan, Sharifa; Walker, Mark; Kim, Peter C.W.

doi:10.1016/j.jpedsurg.2009.01.046

Cited by 67 publications

(50 citation statements)

References 22 publications

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“…However, survival estimates derived from institutional or unit-based data lend themselves to multiple biases. A recent careful evaluation of survival variables comparing all institution and population data from the province of Ontario, Canada, demonstrated a clear discrepancy in institution-based reporting [86,87]. The number of deaths reported by the institutions was found to be 32% less than that of the population data.…”

Section: Long-term Prognosis Of Cdh Infancymentioning

confidence: 96%

Congenital diaphragmatic hernia

Kotecha¹,

Barbato²,

Bush³

et al. 2011

Eur Respir J

140

122

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Infants with congenital diaphragmatic hernia (CDH) have significant mortality and long-term morbidity. Only 60-70% survive and usually those in high-volume centres. The current Task Force, therefore, has convened experts to evaluate the current literature and make recommendations on both the antenatal and post-natal management of CDH. The incidence of CDH varies from 1.7 to 5.7 per 10,000 live-born infants depending on the study population. Antenatal ultrasound scanning is routine and increasingly complemented by the use of magnetic resonance imaging. For isolated CDH, antenatal interventions should be considered, but the techniques need vigorous evaluation. After birth, management protocols are often used and have improved outcome in nonrandomised studies, but immediate intubation at birth and gentle ventilation are important. Pulmonary hypertension is common and its optimal management is crucial as its severity predicts the outcome. Usually, surgery is delayed to allow optimal medical stabilisation. The role of minimal invasive post-natal surgery remains to be further defined. There are differences in opinion about whether extracorporeal membrane oxygenation improves outcome. Survivors of CDH can have a high incidence of comorbidities; thus, multidisciplinary follow-up is recommended. Multicentre international trials are necessary to optimise the antenatal and post-natal management of CDH patients.

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Section: Long-term Prognosis Of Cdh Infancymentioning

confidence: 96%

Congenital diaphragmatic hernia

Kotecha¹,

Barbato²,

Bush³

et al. 2011

Eur Respir J

140

122

View full text Add to dashboard Cite

show abstract

“…1 The overall survival of infants with this condition is highly variable, [2][3][4][5][6] as is their care, although the standardization of postnatal care has evolved over the years. 7 This is illustrated by the dramatic differences in survival between infants undergoing patch repair compared with those amenable to primary repair.…”

mentioning

confidence: 99%

A Clinical Prediction Rule for the Severity of Congenital Diaphragmatic Hernias in Newborns

et al. 2014

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BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. METHODS: Data were obtained on 2202 infants from the Congenital Diaphragmatic Hernia Study Group database from January 2007 to October 2011. Using binary baseline predictors generated from birth weight, 5-minute Apgar score, congenital heart anomalies, and chromosome anomalies, as well as echocardiographic evidence of pulmonary hypertension, a clinical prediction rule was developed on a randomly selected subset of the data by using a backward selection algorithm. An integer-based clinical prediction rule was created. The performance of the model was validated by using the remaining data in terms of calibration and discrimination. RESULTS: The final model included the following predictors: very low birth weight, absent or low 5-minute Apgar score, presence of chromosomal or major cardiac anomaly, and suprasystemic pulmonary hypertension. This model discriminated between a population at high risk of death (∼50%) intermediate risk (∼20%), or low risk (<10%). The model performed well, with a C statistic of 0.806 in the derivation set and 0.769 in the validation set and good calibration (Hosmer-Lemeshow test, P = .2). CONCLUSIONS: A simple, generalizable scoring system was developed for CDH that can be calculated rapidly at the bedside. Using this model, intermediate- and high-risk infants could be selected for transfer to high-volume centers while infants at highest risk could be considered for advanced medical therapies.

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“…This is reflected by the lower survival rates of 30-70% [16,19,[27][28][29][30][31] reported by multi-center studies compared to single institution reports of 80-88% [18,32,33] . Yet, multi-center studies are still hindered by the many biases that impede institution-based research, as shown in the higher survival rates in multi-center studies [16,19,[27][28][29][30][31] compared to population-based reports [20,28,[34][35][36] .…”

Section: Institution-based Researchmentioning

confidence: 99%

“…It is evident in published research that population studies have consistently quoted lower survival outcomes than institution-based studies [16][17][18][19] due to the presence of a 'hidden mortality' [20] . This apparent discrepancy raises the question of whether CDH outcomes are ade- quately represented by institutional data or if population data should be used to provide a more comprehensive and accurate depiction of CDH endpoints.…”

Section: Introductionmentioning

confidence: 99%

Are We Making a Real Difference? Update on ‘Hidden Mortality’ in the Management of Congenital Diaphragmatic Hernia

Mah

Chiu

Kim³

2010

Fetal Diagn Ther

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Reports of improved survival rates for cases of congenital diaphragmatic hernia (CDH) patients have prevailed in the literature over the past 10 years. These improvements have been attributed to advances in medical management in the postnatal period. However, further inquiries into the true survival of CDH patients through population-based studies have revealed that the reported increase in survival outcomes, which are often single institution-based reports, are confounded by case selection bias which fails to consider those CDH patients who do not reach the referral centers. This apparent discrepancy between population-based and institution-based statistics raises the question of ‘hidden mortality’ and the role it plays in both research and clinical medicine. In this review we will examine the reported survival outcomes of CDH from both institution- and population-based perspectives and explore the presence and implications of hidden mortality on research methodology and clinical practice.

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Absolute vs relative improvements in congenital diaphragmatic hernia survival: what happened to “hidden mortality”

Cited by 67 publications

References 22 publications

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia

A Clinical Prediction Rule for the Severity of Congenital Diaphragmatic Hernias in Newborns

Are We Making a Real Difference? Update on ‘Hidden Mortality’ in the Management of Congenital Diaphragmatic Hernia

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