Epidemiology and outcome of congenital diaphragmatic hernia: a 9‐year experience

Abstract

The aim of this study was to report the birth prevalence and short-term outcome of congenital diaphragmatic hernia (CDH) in a large geographically defined population, and to assess the feasibility of performing a randomised control trial (RCT) in this population. Data were collected on all cases of CDH reported to the East Midlands and South Yorkshire Congenital Anomalies Register between 1997 and 2005. A total of 194 cases of CDH were identified from 547,025 births; a birth prevalence of 3.5/10,000. Overall 1-year survival was 42%. In total, 69% of cases resulted in a live birth, of these 61% survived to 1 year; 73% were diagnosed antenatally and 22% postnatally, with 1-year survivals 30% and 71%, respectively. A total of 54% were isolated cases and 46% associated with another anomaly, with more live births (80% vs. 56%) and better 1-year survival (62% vs. 19%) with isolated CDH. Overall, only 83 babies were born alive with an isolated CDH: the only group suitable for inclusion in a RCT. In conclusion, given the small numbers of live isolated CDH cases it is impossible that any network alone would be able to perform a valid RCT of treatments, highlighting the need for collaborative international trials to address this complex condition.

Antenatally diagnosed congenital diaphragmatic hernia in Singapore: a five-year series

“…(6,(18)(19)(20)(21) The majority of cases are similarly diagnosed via fetal anomaly imaging, which is conducted from 18 to 20 weeks of gestation. (22) Although left-sided CDH has been found to be most common, (11,19,(22)(23)(24) our study is unique in that right-sided, bilateral and central forms of CDH were not detected. The topmost ultrasonographic findings are stomach bubble in the thoracic cavity, cardiac displacement and polyhydramnios.…”

“…However, this survival rate is higher than that in other similar studies, where the overall survival rates were consistently below 50.0%. (11,22,33,34) CDH is known to be associated with hidden mortality, which is accounted for by TOP, intrauterine fetal deaths and postnatal deaths. (6,20,35) We have explored deaths caused by the former two causes, but further postnatal follow-up may help to better explain the difference in overall survival rates.…”

“…Additionally, due to the observational and retrospective nature of our study, we were not able to influence the care of any of the pregnancies. As expressed by Wright et al, (11) well-organised randomised controlled trials would be the most challenging but most ideal way to reach a consensus to guide the future management of CDH.…”

Predictors of Mortality and Morbidity in Infants with CDH

“…Some studies reported higher mortality in right-sided CDH (Boix-Ochoa et al, 1974;Touloukian & Markowitz, 1984;Skari et al, 2000;Colvin et al, 2005;Jani et al, 2008;Chao et al, 2010;Schaible et al, 2012), others did not find statistical difference in mortality rate when compared with left-sided defects (Ontario Congenital Anomalies Study Group, 2004; J.E. Wright et al, 2010). Whether right-side CDH is associated with higher rates of malformations is also debatable (Skari, et al, 2000;Bedoyan et al, 2004;Hedrick et al, 2004).…”

Congenital Diaphragmatic Hernia Survivors: Outcomes in Infancy, Childhood and Adolescence

“…Given the small numbers of CDH cases occurring at individual centres, cooperation and collaboration between centres and internationally is essential to develop such protocols, and to enhance further research in this field via randomized controlled trials and investigation into long-term outcomes among survivors (van den Hout et al, 2011;Wright et al, 2011).…”