Background Congenital diaphragmatic hernia (CDH) is a frequently lethal birth defect and, despite advances, extracorporeal life support (ie, extracorporeal membrane oxygenation ECMO) is commonly ...required for severely affected patients. Published data suggest that CDH survival after 2 weeks on ECMO is poor. Many centers limit duration of ECMO support. Study Design We conducted a single-institution retrospective review of 19 years of CDH patients treated with ECMO, designed to evaluate which factors affect survival and duration of ECMO and define how long patients should be supported. Results Of two hundred and forty consecutive CDH patients without lethal associated anomalies, 96 were treated with ECMO and 72 (75%) survived. Eighty required a single run of ECMO and 65 survived (81%), 16 required a second ECMO run and 7 survived (44%). Of patients still on ECMO at 2 weeks, 56% survived, at 3 weeks 46% survived, and at 4 weeks, 43% of patients still on ECMO survived to discharge. After 5 weeks of ECMO, survival had dropped to 15%, and after 40 days of ECMO support there were no survivors. Apgar score at 1 minute, Apgar score at 5 minutes, and Congenital Diaphragmatic Hernia Study Group predicted survival all correlated with survival on ECMO, need for second ECMO, and duration of ECMO. Lung-to-head ratio also correlated with duration of ECMO. All survivors were discharged breathing spontaneously with no support other than nasal cannula oxygen if needed. Conclusions In patients with severe CDH, improvement in pulmonary function sufficient to wean from ECMO can take 4 weeks or longer, and might require a second ECMO run. Pulmonary outcomes in these CDH patients can still be excellent, and the assignment of arbitrary ECMO treatment durations <4 weeks should be avoided.
Abstract Purpose The aim of our study was to elucidate if the defect size reflects the magnitude of the developmental defect in patients with CDH. Methods All patients recorded in the CDH Study Group ...registry between January 1, 2007, and December 31, 2010, and with defect classification were included in the study. They were divided according to defect size (A–D) and compared for: gestational age (GA), birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies, cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and hernia sac. Results A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and 182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively, ( p < 0.0001; p for trend < 0.0001). GA, BW, prevalence of associated anomalies, particularly CVM, number of abnormal organ systems, and prevalence of sac were significantly different between the groups, with a significant reduction of GA, BW, and prevalence of sac. There was an increase in prevalence of associated anomalies, liver in the chest, and number of abnormal systems as the defect size increased. Conclusion Defect size is directly correlated with mortality rate, prevalence of other anomalies (particularly CVM), and number of abnormal systems, and inversely with GA, BW, and prevalence of hernia sac. The defect size may be a marker for the magnitude of developmental abnormality, thereby explaining its relationship with the outcome.
Background Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns and, despite improved outcomes with multimodality therapies, optimal timing of repair remains ...undefined. We sought to evaluate the influence of surgical timing on patient outcomes and hypothesized that delayed repair does not improve survival in CDH. Methods Prospectively collected data from 1,385 CDH Registry infants without preoperative extracorporeal membrane oxygen therapy (ECMO) were evaluated. Patients were stratified by timing of repair: Day of life (DOL) 0–3 (group 1), 4–7 (group 2), or >8 (group 3), and the effect of surgical timing on mortality was determined by logistic regression and risk-adjusted for severity of illness. Results The unadjusted odds ratio (OR) for mortality increased significantly with delayed repair (group 2, 1.73 95% CI, 1.00–2.98; group 3, 3.42 95% CI, 1.97–5.96). However, when adjusted for severity of illness, delay in repair did not predict increased mortality (group 2, 1.2 95% CI, 0.7–2.2; group 3, 1.4 95% CI, 0.8–2.6), nor did it portend an increased need for postoperative ECMO (group 2, 1.1 95% CI, 0.5–2.4; group 3, 0.5 95% CI, 0.2–1.4). Conclusion After adjustment for known risk factors, the timing of CDH repair in low-risk infants does not seem to influence mortality. However, specific clinical parameters guiding timing of elective CDH repair remain unknown.
Abstract Purpose To determine developmental outcomes and associated factors in patients with congenital diaphragmatic hernia (CDH) at 2 years of age. Methods This is a multicenter prospective study ...of a CDH birth cohort. Clinical and socioeconomic data were collected. Bayley Scales of Infant Development (BSID-III) and Vineland Adaptive Behavior Scales (VABS-II) were performed at 2 years of age. Results BSID-III and VABS-II assessments were completed on 48 and 49 children, respectively. The BSID-III mean cognitive, language, and motor scores were significantly below the norm mean with average scores of 93 ± 15, 95 ± 16, and 95 ± 11. Ten percent (5/47) scored more than 2 standard deviations below the norm on one or more domains. VABS-II scores were similar to BSID-III scores with mean communication, daily living skills, social, motor, adaptive behavior scores of 97 ± 14, 94 ± 16, 93 ± 13, 97 ± 10, and 94 ± 14. For the BSID-III, supplemental oxygen at 28 days, a prenatal diagnosis, need for extracorporeal membrane oxygenation (ECMO) and exclusive tube feeds at time of discharge were associated with lower scores. At 2 years of age, history of hospital readmission and need for tube feeds were associated with lower scores. Lower socioeconomic status correlated with lower developmental scores when adjusted for significant health factors. Conclusion CDH patients on average have lower developmental scores at 2 years of age compared to the norm. A need for ECMO, oxygen at 28 days of life, ongoing health issues and lower socioeconomic status are factors associated with developmental delays.
Abstract Purpose The aim of the study was to evaluate the relationship between the degree of liver herniation and mortality or need for extracorporeal membrane oxygenation (ECMO) in isolated ...left-sided congenital diaphragmatic hernia (CDH). Methods This is a retrospective review of all fetuses with isolated left-sided Bochdalek-type CDH evaluated at our center with ultrafast fetal magnetic resonance imaging between January 2004 and December 2010. Percentage of liver herniation was defined as the ratio of herniated liver volume to total fetal liver volume. The liver/thoracic volume ratio was defined as the ratio of herniated liver volume to total thoracic volume. Data were analyzed using receiver operating characteristic curves and Fisher's Exact and Mann-Whitney U tests. Results Fifty-three fetuses with isolated left-sided CDH were evaluated. Fetuses with “liver-up” (n = 32) and “liver-down” (n = 21) anatomy had similar rates of mortality (25% vs 14%, P = .49) and ECMO use (41% vs 29%, P = .40). The accuracy of liver-up (a dichotomous variable) to predict mortality or need for ECMO was 49% and 53%, respectively. Percentage of liver herniation greater than 21% was associated with mortality ( P < .001) or need of ECMO ( P < .001), with an accuracy of 87% and 79%, respectively. Liver/thoracic volume ratio of greater than 14% was also associated with mortality or ECMO use ( P < .001 and P = .01, respectively), with an accuracy of 85% and 72%, respectively. Conclusion Increased amounts of liver herniation in fetuses with isolated left-sided CDH are associated with higher rates of mortality or the need for ECMO support. Quantification of liver herniation (a continuous variable) is superior to overall liver herniation as a dichotomous variable (liver-up vs liver-down) in the prediction of perinatal morbidity and mortality.
The mammalian diaphragm muscle is essential for respiration, and thus is one of the most critical skeletal muscles in the human body. Defects in diaphragm development leading to congenital ...diaphragmatic hernias (CDH) are common birth defects and result in severe morbidity or mortality. Given its functional importance and the frequency of congenital defects, an understanding of diaphragm development, both normally and during herniation, is important. We review current knowledge of the embryological origins of the diaphragm, diaphragm development and morphogenesis, as well as the genetic and developmental aetiology of diaphragm birth defects.
The mammalian diaphragm is a critical skeletal muscle essential for respiration. Defects in diaphragm development, leading to congenital diaphragmatic hernias (CDH), are common and often lethal birth defects. Thus, an understanding of diaphragm development normally and during herniation is important. We review the current knowledge of the diaphragm's embryological origins and morphogenesis and the genetic and developmental etiology of CDH.
Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly with a high rate of mortality and morbidity.
Our aim was to determine a possible effect of standardized treatment on outcome in ...infants with CDH.
All prenatally diagnosed patients with unilateral CDH born alive between January 2006 and December 2009 at the Erasmus MC or the University Hospital Mannheim were eligible for inclusion. Patients who underwent a fetal tracheal occlusion were excluded. From November 1, 2007, all CDH patients were treated according to a standardized treatment protocol. Patients were divided into two chronological groups according to their date of birth: without standardized treatment (group 1, Jan 2006-Oct 2007) and with standardized treatment (group 2, Nov 2007-Dec 2009). Outcome measures were mortality by day 28, bronchopulmonary dysplasia (BPD), defined as oxygen dependency at day 28, and need for extracorporeal membrane oxygenation (ECMO) therapy. Uni- and multivariate analyses were performed.
167 patients were included. By day 28, 18% of the infants had died. Of the patients who were alive at day 28, 49% had BPD. An ECMO procedure was performed in 31% of the patients. Overall mortality for all included patients was 22%. In group 1, overall mortality was 33% and in group 2 overall mortality was 12% (p = 0.004). A standardized treatment protocol was independently associated with a reduced risk for mortality by day 28 (OR 0.28, 95% CI 0.11-0.68). Higher observed-to-expected lung-to-head ratios were independently associated with a lower risk for mortality by day 28 (OR 0.97, 95% CI 0.95-0.99), BPD (OR 0.97, 95% CI 0.94-0.98) and need for ECMO (OR 0.98, 95% CI 0.96-0.99). An intrathoracic position of the liver was independently associated with an increased risk for BPD (OR 3.12, 95% CI 1.41-6.90) and need for ECMO therapy (OR 3.25, 95% CI 1.54-6.88).
Survival rates in patients with CDH increased significantly after the implementation of a standardized treatment protocol.
Background
Bochdalek hernias are a very rare form of diaphragmatic hernias. There are no robust studies that reveal the true natural history of this disease process. The aim of this study was to ...summarize clinically relevant data for the purpose of assisting surgeons with the work-up, diagnosis, and treatment of adult patients with Bochdalek hernia.
Methods
A literature search was performed using PubMed, Google scholar, EMBASE and the following keywords: Bochdalek hernia, congenital diaphragmatic hernia, and posterolateral hernia. All case reports and series after 1955 that pertained to adults were included in the review. The following data points were queried: age, sex, presentation, studies utilized during work-up, laterality, surgical approach, hernia sac management, specific minimally invasive surgical techniques, and follow-up.
Results
A total of 124 articles comprising 173 patients met the inclusion criteria. Based on the data provided, several conclusions regarding this disease process can be made. Most patients present with symptoms related to their hernia (86%). Pain is the most common complaint (69%). While laparotomy is the most widely used surgical approach (38%), minimally invasive surgical techniques have gained popularity since their first report in 1995. Laparoscopic repair can be performed with a low complication rate (7%) and short hospital stay (4 days).
Conclusions
Using modern surgical techniques to include laparoscopy, repair can be performed safely, with a short hospital stay, and with minimal morbidity or mortality.
Although etiology and pathology of symptomatic diaphragm paralysis and eventration are distinct, their treatments are the same: to reduce dysfunctional caudal excursion of the diaphragm during ...inspiration by plication. Minimally invasive diaphragm plication techniques have emerged as equally effective and less morbid alternatives to open plication. This review focuses on the etiology, pathophysiology, diagnosis, and treatment of diaphragmatic eventration or paralysis in adults.
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