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

Mah, Kandice; Chiu, Pao Chin; Kim, Peter C.W.

doi:10.1159/000315889

Cited by 27 publications

(15 citation statements)

References 75 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: 97%

Congenital diaphragmatic hernia

Kotecha¹,

Barbato²,

Bush³

et al. 2011

Eur Respir J

141

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: 97%

Congenital diaphragmatic hernia

Kotecha¹,

Barbato²,

Bush³

et al. 2011

Eur Respir J

141

122

View full text Add to dashboard Cite

show abstract

“…However, the majority of studies are derived from the experience of tertiary referral institutions, which invariably results in case selection bias and the impact of pregnancy termination, which is difficult to quantify. 15,16 The aim of this study is to review the temporal changes in the perinatal management of CDH and the 1-year survival rates from 1996 to 2015 in the state of Western Australia. We hypothesized that with advancements in the postnatal management of infants with CDH, there have been improved survival outcomes.…”

Section: Most Of These Studies Examined the Various Clinical And Etiomentioning

confidence: 99%

“…These factors include the presence of other major anomalies, chromosomal aberrations, preterm birth, and low birth weight. However, the majority of studies are derived from the experience of tertiary referral institutions, which invariably results in case selection bias and the impact of pregnancy termination, which is difficult to quantify …”

Section: Introductionmentioning

confidence: 99%

Congenital diaphragmatic hernia: Impact of contemporary management strategies on perinatal outcomes

et al. 2018

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Objective This study aims to review temporal changes in perinatal management and 1‐year survival outcomes of cases of congenital diaphragmatic hernia (CDH) from 1996 to 2015 in Western Australia (WA). Method This research is a retrospective study of all cases of CDH in WA from 1996 to 2015 identified from five independent databases within the WA health network. Detailed information pertaining to pregnancy and survival outcomes were obtained from review of maternal and infant medical records. Results There were 215 cases of CDH with 164 diagnosed prenatally. Between 1996 and 2010, a decline in live birth rates for CDH‐affected pregnancies was observed, reaching a nadir of 5.3 per 10 000 births before increasing to a peak of 9.73 per 10 000 births in 2011‐2015. A corresponding decline was seen in the number of pregnancies terminated in the same period from 8.3 to 4.6 per 10 000 births (P = 0.14) and an increase in survival of live births from 38.9% to 81.3% (P = 0.01). Conclusion The improved overall survival rate in infants with CDH over the last 20 years may have resulted in an increased tendency for women to continue their pregnancy with a concomitant decline in termination rates. Information from this study will help in the counselling of women following prenatal detection of CDH.

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“…Congenital diaphragmatic hernia (CDH) is a life‐threatening developmental anomaly, which arises early during human development. Its birth incidence fluctuates worldwide around 1 in 2,000–3,000 new‐borns and it is an important cause of stillbirths and abortions 1–3. CDH patients suffer from severe respiratory distress caused by a classical “trio” of features: a defect in the muscular or tendinous portion of the diaphragm, pulmonary hypoplasia, and pulmonary hypertension.…”

Section: Introductionmentioning

confidence: 99%

Developmental and genetic aspects of congenital diaphragmatic hernia

Veenma

Klein

Tibboel

2012

Pediatric Pulmonology

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Congenital diaphragmatic hernia (CDH) is a frequent occurring cause of neonatal respiratory distress and occurs 1 in every 3,000 liveborns. Ventilatory support and pharmaceutical treatment of the co-occurring lung hypoplasia and pulmonary hypertension are insufficient in, respectively, 20% of isolated cases and 60% of complex ones leading to early perinatal death. The exact cause of CDH remains to be identified in the majority of human CDH patients and prognostic factors predicting treatment refraction are largely unknown. Their identification is hampered by the multifactorial and heterogenic nature of this congenital anomaly. However, application of high-resolution molecular cytogenetic techniques to patients' DNA now enables detection of chromosomal aberrations in 30% of the patients. Furthermore, recent insights in rodent embryogenesis pointed to a specific disruption of the early mesenchymal structures in the primordial diaphragm of CDH-induced offspring. Together, these data allowed for the introduction of new hypotheses on CDH pathogenesis, although many issues remain to be resolved. In this review, we have combined these new insights and remaining questions on diaphragm pathogenesis with a concise overview of the clinical, embryological, and genetic data available.

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

Cited by 27 publications

References 75 publications

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia

Congenital diaphragmatic hernia: Impact of contemporary management strategies on perinatal outcomes

Developmental and genetic aspects of congenital diaphragmatic hernia

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