[Show abstract][Hide abstract] ABSTRACT: 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 ( approximately 50%) intermediate risk ( approximately 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.
[Show abstract][Hide abstract] ABSTRACT: 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.
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.
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]).
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.
[Show abstract][Hide abstract] ABSTRACT: Background/purpose: Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal
death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging.
The objective of this study was to create a standardized reporting system for CDH.
Methods: Data were prospectively collected on all live born infants with CDH from 51 centers in 9
countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A–D)
using a standardized system. Other data known to affect outcome were combined to create a usable
staging system. The primary outcome was death or hospital discharge.
Results: A total of 1,975 infants were evaluated. A total of 326 infants were not repaired, and all died.
Of the remaining 1,649, the defect was scored in 1,638 patients. A small defect (A) had a high survival,
while a large defect was much worse. Cardiac defects significantly worsened outcome. We grouped
patients into 6 categories based on defect size with an isolated A defect as stage I. A major cardiac
anomaly (+) placed the patient in the next higher stage. Applying this, patient survival is 99% for stage
I, 96% stage II, 78% stage III, 58% stage IV, 39% stage V, and 0% for non-repair.
Journal of Pediatric Surgery 12/2013; 48(12):2408. · 1.38 Impact Factor
[Show abstract][Hide abstract] 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.
Journal of Pediatric Surgery 06/2013; 48(6):1177-82. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Chylothorax is a known complication in neonates after congenital diaphragmatic hernia (CDH) repair. This report uses a large international registry to evaluate risk factors, treatment, morbidity, and survival associated with chylothorax in a prospective cohort of neonates after CDH repair.
From January 2007 to January 2010, live-born neonates with repaired, unilateral CDHs were evaluated from a prospective database for chylothorax development. Chylothorax was diagnosed based on pleural fluid examination. Study variables included patient characteristics, CDH defect and disease severity characteristics, chylothorax treatment, and survival. In addition, the temporal relationship between timing of CDH repair and extracorporeal membrane oxygenation (ECMO) therapy was evaluated as a risk factor for chylothorax. Univariate and multivariate regression analyses were utilized.
Among the 1383 patients evaluated, chylothorax was diagnosed in 4.6% of the cohort. Patch repair and ECMO were statistically significant risk factors for chylothorax. The odds of developing a chylothorax were significantly increased in patients with CDH repair on ECMO (aOR 2.6; 95% CI: 1.3-4.9) or after ECMO (aOR 3.1; 95% CI: 1.7-5.8). Most chylothoraces (83.1%) were successfully treated without surgery. Chylothorax patients had significant morbidity including increased oxygen use at 30days and longer length of stay. Survival was not significantly affected by chylothorax.
Chylothorax is a known but uncommon complication of neonatal CDH repair. In this very large series of chylothorax in association with CDH, major risk factors appear to be related to increased disease severity with the highest risk in patients repaired on or after ECMO. Chylothoraces usually improve with conservative therapy and lead to significant morbidity but not increased mortality.
Journal of Pediatric Surgery 04/2013; 48(4):724-9. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVE: To evaluate the impact of associated heart defects on outcomes to discharge, and identify factors affecting survival of all infants born with congenital diaphragmatic hernia (CDH) in last decade using Congenital Diaphragmatic Hernia Study Group data. STUDY DESIGN: This was a retrospective review of all infants with CDH enrolled in Congenital Diaphragmatic Hernia Study Group database from January 2000 to December 2010. The study cohort was divided into 3 groups (GRP): GRP 1, CDH with major heart defects; GRP 2, CDH with minor heart defects; and GRP 3, CDH with no reported heart defects. RESULTS: The 4268 enrolled infants included 345 (8%) in GRP 1, 412 (10%) in GRP 2, and 3511 (82%) in GRP 3. Survival was significantly lower in GRP 1 compared with GRP 2 and GRP 3 (36% vs 73%). In GRP 1, the most common defects were left heart obstructive lesions (34%). Survival was lowest in infants with transposition of great arteries (0%) and single ventricle physiology (16%). There was no change in survival rate for any group between 2000-2005 and 2006-2010. In GRP 1, factors that predicted lower survival were birth weight <2.5 kg, associated noncardiac anomalies, single ventricle physiology, no sildenafil therapy, no CDH repair, and no cardiac repair. CONCLUSION: Survival is significantly lower in patients with CDH and major heart defects compared with patients with minor or no heart defects. Outcomes of newborns with CDH and major heart defects have not improved over the last decade.
The Journal of pediatrics 08/2012; · 4.02 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Objective:To analyze operative repair, extracorporeal membrane oxygenation (ECMO) and survival rates based on highest pre-ductal oxygen saturation (Pre-O(2)SAT) in a large infant cohort reported to Congenital Diaphragmatic Hernia Study Group Registry between 2000 and 2010.Study Design:Analyzed data included gestational age, birth weight, defect side and size, repair, ECMO use, survival and highest reported PaO(2) and Pre-O(2)SAT in first 24 h of life. We excluded 614 infants due to severe anomaly. Pre-O(2)SAT data were available for 1672 infants.Result:Among infants with highest Pre-O(2)SAT value <85%, survival (24/105=23%) and repair (55/105=52%) rates were significantly decreased compared with infants with higher values. Survival increased to 44% for infants with highest Pre-O(2)SAT<85% who underwent operative repair. Of these, 83% (20/24) required ECMO support compared with 15% (144/961) of survivors with Pre-O(2)SAT>99% (P<0.001). The lowest reported Pre-O(2)SAT with survival was 32% and for survival without ECMO was 52%.Conclusion:A reported highest Pre-O(2)SAT<85% in the first 24 h of life was not uniformly fatal; but survival of infants with Pre-O(2)SAT<85% was associated with high ECMO use and prolonged hospitalization.Journal of Perinatology advance online publication, 1 March 2012; doi:10.1038/jp.2012.18.
Journal of perinatology: official journal of the California Perinatal Association 03/2012; · 1.59 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Operative approach, including minimally invasive surgery (MIS) in the repair of congenital diaphragmatic hernia (CDH), is variable among institutions. The short-term recurrent hernia rate is not well described. We evaluated the in-hospital recurrence rate of MIS repairs of infants with CDH from the Congenital Diaphragmatic Hernia Registry.
Prospectively collected data from infants with CDH were analyzed from the Congenital Diaphragmatic Hernia Registry from January 1995 to January 2010. Recurrent hernia was defined as reoperations during initial hospitalization. Operative approaches included abdominal, thoracic, laparoscopic, and thoracoscopic techniques.
Five thousand four hundred eighty infants with CDH were identified, of which 4516 (82.4%) were repaired. Operative data were available in 4390 infants. One hundred fifty-one infants (3.4%) underwent MIS repairs with 12 reported recurrences (7.9%) compared with 114 for open techniques (2.7%, P < .05). Minimally invasive surgery demonstrated a significant increased odds for recurrence (odds ratio, 3.59; 95% confidence interval, 1.92-6.71) after adjusting for gestational age, birth weight, patch repair, and extracorporeal membrane oxygenation.
Minimally invasive techniques appear to have a significant higher recurrent hernia rate, with thoracoscopy being the highest. Although adjusted for patch repair, other factors with regard to disease severity may contribute to differences in outcomes among centers. This study is limited to short-term recurrence during initial hospitalization.
Journal of Pediatric Surgery 06/2011; 46(6):1158-64. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Congenital diaphragmatic hernia (CDH) is associated with a mortality rate of 10-35% in live-born infants. Moreover, CDH survivors have a substantial risk of developing long-term pulmonary sequelae, such as bronchopulmonary dysplasia (BPD).
This study aims to evaluate risk factors associated with BPD and mortality in neonates with CDH, with particular focus on the initial ventilation mode.
Eligible for inclusion were live-born infants with CDH born from 2001 through 2006 at the centers participating in the CDH Study Group. BPD (defined as oxygen dependency at day 30) and/or mortality by day 30 served as the primary endpoint.
A total of 2,078 neonates were included in the analysis. At day 30, 56% of the patients had either died or met the criteria for BPD. In infants who survived until day 30, the prevalence of BPD was 41%. The overall mortality rate was 31%. High-frequency oscillatory ventilation as initial ventilation mode, a right-sided defect, a prenatal diagnosis, a lower Apgar score at 5 min, a cardiac anomaly, a chromosomal anomaly and a lower gestational age were all associated with BPD and/or mortality by day 30.
Despite improvements in neonatal care, the rates of BPD and early mortality in newborns with CDH are still considerable. Several important risk factors for a worse outcome are reported in this nonrandomized prospective observational study.
[Show abstract][Hide abstract] ABSTRACT: Congenital diaphragmatic hernia (CDH) remains a significant cause of death in newborns. With advances in neonatal critical care and ventilation strategies, survival in the term infant now exceeds 80% in some centers. Although prematurity is a significant risk factor for morbidity and mortality in most neonatal diseases, its associated risk with infants with CDH has been described poorly. We sought to determine the impact of prematurity on survival using data from the Congenital Diaphragmatic Hernia Registry (CDHR).
Prospectively collected data from live-born infants with CDH were analyzed from the CDHR from January 1995 to July 2009. Preterm infants were defined as <37 weeks estimated gestational age at birth. Univariate and multivariate logistic regression analysis were performed.
During the study period, 5,069 infants with CDH were entered in the registry. Of the 5,022 infants with gestational age data, there were 3,895 term infants (77.6%) and 1,127 preterm infants (22.4%). Overall survival was 68.7%. A higher percentage of term infants were treated with extracorporeal membrane oxygenation (ECMO) (33% term vs 25.6% preterm). Preterm infants had a greater percentage of chromosomal abnormalities (4% term vs 8.1% preterm) and major cardiac anomalies (6.1% term vs 11.8% preterm). Also, a significantly higher percentage of term infants had repair of the hernia (86.3% term vs 69.4% preterm). Survival for infants that underwent repair was high in both groups (84.6% term vs 77.2% preterm). Survival decreased with decreasing gestational age (73.1% term vs 53.5% preterm). The odds ratio (OR) for death among preterm infants adjusted for patch repair, ECMO, chromosomal abnormalities, and major cardiac anomalies was OR 1.68 (95% confidence interval [CI], 1.34-2.11).
Although outcomes for preterm infants are clearly worse than in the term infant, more than 50% of preterm infants still survived. Preterm infants with CDH remain a high-risk group. Although ECMO may be of limited value in the extremely premature infant with CDH, most preterm infants that live to undergo repair will survive. Prematurity should not be an independent factor in the treatment strategies of infants with CDH.
Surgery 08/2010; 148(2):404-10. · 3.37 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Severe congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) is associated with high mortality. Timing of CDH repair relative to ECMO therapy remains controversial. Our hypothesis was that survival would significantly differ between those who underwent repair during ECMO and those who underwent repair after ECMO therapy.
We examined deidentified data from the CDH study group (CDHSG) registry from 1995 to 2005 on patients who underwent repair and ECMO therapy (n = 636). We used Cox regression analysis to assess differences in survival between those who underwent repair during and after ECMO.
Five covariates were significantly associated with mortality as follows: timing of repair relative to ECMO (P = .03), defect side (P = .01), ECMO run length (P < .01), need for patch repair (P = .03), birth weight (P < .01), and Apgar score at 5 minutes (P = .03). Birth year, inborn vs transfer status, diaphragmatic agenesis, age at repair, and presence of cardiac or chromosomal abnormalities were not associated with survival. Repair after ECMO therapy was associated with increased survival relative to repair on ECMO (hazard ratio, 1.407; P = .03).
These data suggest that CDH repair after ECMO therapy is associated with improved survival compared to repair on ECMO, despite controlling for factors associated with the severity of CDH.
Journal of Pediatric Surgery 07/2009; 44(6):1165-71; discussion 1171-2. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The goal of the study was to test the hypothesis that infants with known congenital diaphragmatic hernias born at early term gestation (37-38 weeks) rather than later (39-41 weeks) had greater survival rates and less extracorporeal membrane oxygenation use. Primary outcomes were survival to hospital discharge or transfer and extracorporeal membrane oxygenation use. METHODS; A retrospective cohort study of term infants with prenatal diagnoses of congenital diaphragmatic hernia was performed with the Congenital Diaphragmatic Hernia Study Group Registry of patients with congenital diaphragmatic hernias who were treated between January 1995 and December 2006.
Among 628 term infants at 37 to 41 weeks of gestation who had prenatal diagnoses of congenital diaphragmatic hernia and were free of major associated anomalies, early term birth (37 vs 39-41 weeks) and greater birth weight were associated independently with survival, whereas black race was related inversely to survival. Infants born at early term with birth weights at or above the group mean (3.1 kg) had the greatest survival rate (80%). Among infants born through elective cesarean delivery, infants born at 37 to 38 weeks of gestation, compared with 39 to 41 weeks, had less use of extracorporeal membrane oxygenation (22.0% vs 35.5%) and a trend toward a greater survival rate (75.0% vs 65.8%).
The timing of delivery is an independent, potentially important factor in the consideration of elective delivery for infants diagnosed prenatally as having congenital diaphragmatic hernias. Among fetuses with prenatally diagnosed congenital diaphragmatic hernias and without major associated anomalies, early term delivery may confer advantage.
[Show abstract][Hide abstract] ABSTRACT: Congenital diaphragmatic hernia is a significant cause of neonatal mortality. The objective of this study was to evaluate the clinical factors associated with death in infants with congenital diaphragmatic hernia by using a large multicenter data set.
This was a prospective cohort study of all liveborn infants with congenital diaphragmatic hernia who were cared for at tertiary referral centers belonging to the Congenital Diaphragmatic Hernia Study Group between 1995 and 2004. Factors thought to influence death included birth weight, Apgar scores, size of defect, and associated anomalies. Survival to hospital discharge, duration of mechanical ventilation, and length of hospital stay were evaluated as end points.
A total of 51 centers in 8 countries contributed data on 3062 liveborn infants. The overall survival rate was 69%. Five hundred thirty-eight (18%) patients did not undergo an operation and died. The defect size was the most significant factor that affected outcome; infants with a near absence of the diaphragm had a survival rate of 57% compared with infants having a primary repair with a survival rate of 95%. Infants without agenesis but who required a patch for repair had a survival rate of 79% compared with primary repair.
The size of the diaphragmatic defect seems to be the major factor influencing outcome in infants with congenital diaphragmatic hernia. It is likely that the defect size is a surrogate marker for the degree of pulmonary hypoplasia. Future research efforts should be directed to accurately quantitate the degree of pulmonary hypoplasia or defect size antenatally. Experimental therapies can then be targeted to prospectively identify high-risk patients who are more likely to benefit.
[Show abstract][Hide abstract] ABSTRACT: The objective of this study was to evaluate the impact of newer therapies on the highest risk patients with congenital diaphragmatic hernia (CDH), those with agenesis of the diaphragm.
CDH remains a significant cause of neonatal mortality. Many novel therapeutic interventions have been used in these infants. Those children with large defects or agenesis of the diaphragm have the highest mortality and morbidity.
Twenty centers from 5 countries collected data prospectively on all liveborn infants with CDH over a 10-year period. The treatment and outcomes in these patients were examined. Patients were followed until death or hospital discharge.
A total of 1,569 patients with CDH were seen between January 1995 and December 2004 in 20 centers. A total of 218 patients (14%) had diaphragmatic agenesis and underwent repair. The overall survival for all patients was 68%, while survival was 54% in patients with agenesis. When patients with diaphragmatic agenesis from the first 2 years were compared with similar patients from the last 2 years, there was significantly less use of ECMO (75% vs. 52%) and an increased use of inhaled nitric oxide (iNO) (30% vs. 80%). There was a trend toward improved survival in patients with agenesis from 47% in the first 2 years to 59% in the last 2 years. The survivors with diaphragmatic agenesis had prolonged hospital stays compared with patients without agenesis (median, 68 vs. 30 days). For the last 2 years of the study, 36% of the patients with agenesis were discharged on tube feedings and 22% on oxygen therapy.
There has been a change in the management of infants with CDH with less frequent use of ECMO and a greater use of iNO in high-risk patients with a potential improvement in survival. However, the mortality, hospital length of stay, and morbidity in agenesis patients remain significant.
Annals of Surgery 11/2006; 244(4):505-13. · 6.33 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Prenatal corticosteroids have been used in fetuses with congenital diaphragmatic hernia (CDH). We tested the utility of steroids by 2 methods.
Mothers carrying fetuses with CDH were randomized to 3 weekly doses of betamethasone or placebo starting at 34 weeks. Patients were followed until death or discharge. In a separate cohort study, the CDH Registry was used to compare infants who received prenatal steroids to those who had not.
Thirty-four patients were enrolled at 7 centers, with 32 completing the trial. There were 15 placebo and 17 steroid patients. There was no difference in survival, length of stay, duration of ventilation, or oxygen use at 30 days. For the cohort study, we looked at infants older than 34 weeks who were born after October 2000 when data on prenatal steroids were collected. There were 1093 patients; 390 were evaluable, with 56 receiving steroids. There was no difference in survival, length of stay, ventilator days, or oxygen use at 30 days.
Neither the trial nor the CDH Registry suggest that late prenatal corticosteroids benefit fetuses with CDH. More than 1700 mothers and fetuses would need to be enrolled in a trial to show a 10% improvement in survival. It is unlikely that late steroids offer benefit to most fetuses with CDH.
Journal of Pediatric Surgery 05/2006; 41(4):668-74; discussion 668-74. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We compared the morbidity of patients with meconium aspiration syndrome (MAS) with that in patients with all other respiratory conditions treated with extracorporeal membrane oxygenation (ECMO) (no MAS). If ECMO for MAS was associated with a lower complication rate, then relaxed ECMO entry criteria could be considered. A retrospective review was performed of all patients in the national extracorporeal life support (ELSO) registry from 1989 to 2004. Complications were divided into mechanical, hematologic, neurologic, renal, pulmonary, cardiovascular, infectious, and metabolic categories. MAS and no-MAS patients were divided into veno-venous (VV) or veno-arterial (VA) ECMO categories, based on mode of ECMO used, and number of complications per patient in each category was determined. Statistical significance was determined by Chi-square test. A total of 1587 patients (700 MAS, 887 no MAS) on VV ECMO and 2723 (572 MAS, 2151 no MAS) on VA ECMO were identified with a total of 2415 complications in MAS and 9550 in no-MAS patients. Overall, MAS patients had a significantly lower number of complications per patient in each category versus no-MAS patients. These results indicate that regardless of type of ECMO, there are fewer complications on ECMO in MAS versus no-MAS patients. These data support the consideration of relaxed ECMO entry criteria for MAS.
ASAIO journal (American Society for Artificial Internal Organs: 1992) 01/2006; 53(4):489-91. · 1.39 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Late-presenting congenital diaphragmatic hernia (CDH) is a rare subset of CDH, most of the information derived from small series or case reports. The aim of this study was to document the clinical manifestations of late-presenting CDH using a large multicenter database.
Information about late-presenting CDH (diagnosed at later than 30 days of age) was identified from the database of the CDH Study Group (3098 cases collected during 1995-2004) and reviewed retrospectively.
Seventy-nine cases (2.6%) from 30 centers met the inclusion criteria. Seven cases had a Morgagni hernia. There were 50 males (65%) and 27 females (35%). The mean age at diagnosis was 372 days (32 days to 15 years). Major associated anomalies (10 cardiac and 7 chromosomal abnormalities) were identified in 12 cases (15%). Presenting symptoms were respiratory in 20 (43%), gastrointestinal in 15 (33%), both in 6 (13%), and none (asymptomatic) in 5 (11%). The hernia was left-sided in 53 (69%), right-sided in 21 (27%), and central or bilateral in 3 (4%). Patients with gastrointestinal symptoms invariably had left-sided hernias (n = 19), whereas patients with respiratory symptoms (n = 24) seemed equally likely to have right- or left-sided lesions. A primary repair without patch was done in all cases with 100% survival.
Presenting symptoms of late-onset CDH can be respiratory or gastrointestinal, but presentation with gastrointestinal problems was more common in left-sided hernias, whereas respiratory symptoms predominated in right-sided lesions. The prognosis is excellent once the correct diagnosis is made.
Journal of Pediatric Surgery 01/2006; 40(12):1839-43. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Respiratory failure in neonates with congenital diaphragmatic hernia (CDH) may in part be caused by a primary or secondary surfactant deficiency. Knowledge of the optimal approach to surfactant replacement in neonates with CDH and respiratory failure is limited. The aim of this study was to determine if surfactant replacement on extracorporeal membrane oxygenation (ECMO) results in improved outcomes in neonates > or =35 weeks' gestation with unrepaired CDH.
Using the CDH Study Group Registry, the authors identified 448 neonates with CDH who were > or =35 weeks' gestation, had no major anomalies, were treated with ECMO within the first 7 days of life, and underwent repair on or after ECMO therapy. Patients in 2 groups were compared: group 1 (- Surf, n = 334) consisted of patients who received no surfactant and group 2 (+ Surf, n = 114) consisted of patients who received at least 1 dose of surfactant while on ECMO. An analysis of all patients in both groups was performed. Additionally, subgroup analyses stratified by gestational age were performed for patients 351/7 to 366/7 weeks' gestation and for patients > or =37 weeks' gestation. Primary end-points for the study were survival and length of ECMO run. Secondary end-points were length of intubation, need for supplemental oxygen at 30 days of life, and at discharge to home. Demographic, clinical, and outcome variables were examined using Fisher's Exact tests for categorical variables and using unpaired t tests for continuous variables. Odds ratios were calculated for categorical end-point variables.
Demographic and clinical variables were similar between groups. Analyses of aggregate data showed no significant differences between groups in length of ECMO run, survival, number of days intubated, and percent of patients requiring supplemental oxygen at 30 days or discharge. Subgroup stratification by gestational age did not show significant differences between groups in any of the outcome variables.
The data from this study suggest that surfactant replacement on ECMO for neonates with congenital diaphragmatic hernia does not provide significant benefit in the infant's clinical course with respect to survival, length of ECMO course, length of intubation, or subsequent need for supplemental oxygen.
Journal of Pediatric Surgery 11/2004; 39(11):1632-7. · 1.38 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Use of exogenous surfactant in congenital diaphragmatic hernia (CDH) patients is routine in many centers. The authors sought to determine the impact of surfactant use in the premature infant with CDH.
Data on liveborn infants with CDH from participating institutions were collected prospectively. Surfactant use and timing and outcome data were analyzed retrospectively. The authors evaluated the prenatal diagnosis patients as well. The outcome variable was survival to discharge. Odds ratios with confidence intervals were calculated.
Five hundred ten infants less than 37 weeks' gestation were entered in the CDH registry. Infants with severe anomalies (n = 80) were excluded. Information on surfactant use was available for 424 patients. Infants receiving surfactant (n = 209) had a greater odds of death than infants not receiving surfactant (n = 215, odds ratio, 2.17, 95% CI: 1.5 to 3.2; P <.01). In prenatally diagnosed infants with immediate distress, there was a trend toward worse survival rates among those receiving surfactant at 1 hour (52 patients) versus those that did not (93 patients; odds ratio, 1.93, 95% CI: 0.96 to 3.9; P <.07).
Surfactant, as currently used, is associated with a lower survival rate in preterm infants with CDH. The use of surfactant replacement in premature infants with CDH can be recommended only within the context of a randomized clinical trial.
Journal of Pediatric Surgery 06/2004; 39(6):829-33. · 1.38 Impact Factor